Sarcoma treatment: survival prognosis. Relapses of tumors and tumor-like lesions of bones and soft tissues Symptoms of the development of rhabdomyosarcoma

Sarcoma is a name that unites oncological tumors of a large group. Different types of connective tissue under certain conditions begin to undergo histological and morphological changes. Then the primary connective cells begin to grow rapidly, especially in children. A tumor develops from such a cell: benign or malignant with elements of muscles, tendons, vessels.

The cells of the connective tissue divide uncontrollably, the tumor grows and without clear boundaries enters the territory of healthy tissue. Malignant become - 15% of neoplasms, the cells of which carry blood throughout the body. As a result of metastasis, secondary growing oncological processes are formed, therefore it is believed that sarcoma is a disease that is characterized by frequent relapses. In terms of deaths, it ranks second among all oncological formations.

Is sarcoma cancer or not?

Some of the signs of sarcoma are the same as those of cancer. For example, it also grows infiltratively, destroys adjacent tissues, relapses after surgery, metastases early and spreads in the tissues of organs.

How cancer differs from sarcoma:

the cancer tumor looks like a lumpy conglomerate, growing rapidly without symptoms in the early stages. Sarcoma is pinkish, resembling fish meat;
a cancerous tumor affects epithelial tissue, sarcoma - muscle connective;
the development of cancer occurs gradually in any particular organ in people after 40 years. Sarcoma is a disease of young people and children, it instantly affects their bodies, but is not tied to any one organ;
cancer is easier to diagnose, which increases the rate of cure. Sarcoma is more often found at 3-4 stages, so its mortality rate is 50% higher.

Is sarcoma contagious?

No, she is not contagious. An infectious disease develops from a real substrate that carries the infection by airborne droplets or through blood. Then a disease, such as the flu, can develop in the body of the new host. Sarcoma can be contracted as a result of changes in the genetic code, chromosomal changes. Therefore, sarcoma patients often have close relatives who have already been treated for any of its 100 types.

Sarcoma in HIV is a multiple hemorrhagic sarcomatosis called "angiosarcoma" or "". It is recognized by ulceration of the skin and mucous membranes. A person gets sick as a result of herpes infection of the eighth type through the lymph, blood, secretion of the secretion of the skin and saliva of the patient, as well as through sexual contact. Even with antiviral therapy, Kaposi's tumor often recurs.

The development of sarcoma against the background of HIV is possible with a sharp decrease in immunity. At the same time, AIDS or a disease such as lymphosarcoma, leukemia, lymphogranulomatosis or myeloma can be detected in patients.

The causes of sarcomas

Despite the variety of species, sarcoma rarely falls ill, only in the amount of 1% of all cancers. The causes of sarcoma are manifold. Among the established reasons are: exposure to ultraviolet (ionizing) radiation, radiation. And also viruses and chemicals, precursors of disease, benign neoplasms, turning into oncological ones, become risk factors.

The causes of Ewing's sarcoma may lie in the rate of bone growth and hormonal levels. Risk factors such as smoking, working in chemical plants, contact with chemicals are also important.

Most often, this type of oncology is diagnosed due to the following risk factors:

hereditary predisposition and genetic syndromes: Werner, Gardner, multiple pigmentary skin cancer from basal cells, neurofibromatosis or retinoblastoma;
herpes virus;
chronic lymphostasis of the legs, the relapse of which occurred after radial mastectomy;
injuries, wounds with suppuration, impacts of cutting and stabbing objects (glass fragments, metal, chips, etc.);
immunosuppressive and polychemotherapy (in 10%);
organ transplant operations (in 75% of cases).

Informative video

General symptoms and signs of the disease

The signs of sarcoma appear depending on its location in the vital organs. The biological characteristics of the root cause of the cell and the tumor itself affect the nature of the symptomatology. The early signs of sarcoma are the noticeable size of the mass as it grows rapidly. Pain in joints and bones appears early (especially at night), which is not relieved by analgesics.

For example, due to the growth of rhabdominosarcoma, the oncological process spreads to the tissues of healthy organs and manifests itself in different pain symptoms and hematogenous metastasis. If the sarcoma develops slowly, the symptoms of the disease may not appear for several years.

Symptoms of lymphoid sarcoma are reduced to the formation of oval or round nodes and small swelling in the lymph node. But even with a size of 2-30 cm, a person may not feel pain at all.

In other types of tumors with rapid growth and progression, the following may appear: fever, veins under the skin, and cyanotic ulceration on them. Palpation of the formation reveals that it is limited in mobility. The first signs of sarcoma are sometimes characterized by deformity of the joints of the extremities.

Liposarcomas, along with other types, can be of a primary-multiple nature with sequential or simultaneous manifestation in different areas of the body. This significantly complicates the search for the primary tumor that gives metastases.

Symptoms of a sarcoma located in soft tissues are expressed in painful sensations on palpation. Such a tumor has no outlines, and it quickly penetrates into nearby tissues.

With pulmonary oncology, the patient suffers from shortness of breath, which causes oxygen starvation of the brain, pneumonia, pleurisy, dysphagia may begin, and the right heart may increase.

The cells of the nerve membranes are reborn into neurofibrosarcoma, the cells and fibers of the connective tissue - in. Spindle cell sarcoma, consisting of large cells, affects the mucous membranes. Mesothelioma grows from the mesothelium of the pleura, peritoneum and pericardium.

Types of sarcomas at the site of localization

The types of sarcoma are distinguished depending on the location.

AND Of 100 types, sarcomas most often develop in the area:

peritoneum and retroperitoneal space;
neck, head and bones;
mammary glands and uterus;
stomach and intestines (stromal tumors);
adipose and soft tissues of the limbs and trunk, including desmoid fibromatosis.

Especially often new ones are diagnosed in adipose and soft tissues:

developing from adipose tissue;
what refers to fibroblastic / myofibroblastic formations;
fibrohistiocytic tumors of soft tissues: plexiform and giant cell;
- from smooth muscle tissue;
glomus tumor (pericytic or perivascular);
from the musculature of the skeleton;
and epithelioid hemangioepithelioma, which refer to soft tissue vascular formations;
mesenchymal chondrosarcoma, extraskeletal osteosarcoma - osteochondral tumors;
malignant gastrointestinal tract (gastrointestinal stromal tumor);
oncology of the nerve trunk: peripheral nerve trunk, newt tumor, granulocytic, ectomesenchymoma;
sarcomas of unclear differentiation: synovial, epithelioid, alveolar, clear cell, Ewing, desmoplastic round cell, intimate, PEComu;
undifferentiated / unclassified sarcoma: spindle cell, pleomorphic, round cell, epithelioid.

From bone oncology according to the WHO classification (ICD-10), the following tumors are often found:

cartilage tissue - chondrosarcoma: central, primary or secondary, peripheral (periosteal), clear cell, dedifferentiated and mesenchymal;
bone tissue - osteosarcoma, an ordinary tumor: chondroblastic, fibroblastic, osteoblastic, as well as telangiectatic, small cell, central low grade, secondary and paraostal, periosteal and superficial high grade;
fibroids - fibrosarcoma;
fibrohistiocytic formations - malignant fibrous histiocytoma;
/ PNEO;
hematopoietic tissue - plasmacytoma (myeloma), malignant lymphoma;
giant cell: malignant giant cell;
oncology of the chord - "dedifferentiated" (sarcomatoid);
vascular tumors - angiosarcoma;
smooth muscle tumors - leiomyosarcoma;
tumors from adipose tissue - liposarcoma.

The maturity of all types of sarcomas can be low, medium and highly differentiated. The lower the differentiation, the more aggressive the sarcoma. Treatment and prognosis of survival depend on maturity and stage of education.

Stages and degrees of the malignant process

There are three grades of sarcoma malignancy:

Poorly differentiated degree, in which the tumor consists of more mature cells and the process of their division is slow. It is dominated by the stroma - normal connective tissue with a small percentage of oncoelements. Education rarely metastasizes and recurs a little, but can grow to a large size.
A highly differentiated degree in which tumor cells divide rapidly and uncontrollably. With rapid growth, a sarcoma develops a dense vascular network with a large number of high malignant tumor cells, and metastases spread early. Surgical treatment of a high grade lesion may be ineffective.
Moderately differentiated degree, in which the tumor has an intermediate development, and with adequate treatment, a positive prognosis is possible.

The stages of a sarcoma do not depend on its histological type, but on its location. More determines the stage according to the state of the organ where the tumor began to develop.

The initial stage of sarcoma is characterized by its small size. It does not extend beyond those organs or segments where it originally appeared. There are no violations of the working functions of organs, compression, metastasis. There is practically no pain. If a highly differentiated stage 1 sarcoma is detected, positive results are achieved with complex treatment.

Signs of the initial stage of sarcoma, depending on the location in a particular organ, for example, are as follows:

in the oral cavity and on the tongue - a small node up to 1 cm in size and with clear boundaries appears in the submucous layer or mucous membrane;
on the lips - the knot is felt in the submucosal layer or inside the lip tissue;
in the cellular spaces and soft tissues of the neck - the size of the node reaches 2 cm, it is located in the fascia that limit its location, and does not go beyond them;
in the area of the larynx - the mucous membrane or other layers of the larynx limit the node, up to 1 cm in size. It is located in the fascial case, does not go beyond it and does not disturb phonation and respiration;
in the thyroid gland - a node, up to 1 cm in size, located inside its tissues, the germination of the capsule does not occur;
in the mammary gland - a node up to 2-3 cm grows in a lobule and does not go beyond its limits;
in the region of the esophagus - an oncous node up to 1-2 cm is located in its wall, without disturbing the passage of food;
in the lung - it is manifested by the defeat of one of the segments of the bronchi, without going beyond its limits and without disrupting the working function of the lung;
in the testicle - a small node develops without involving the tunica albuginea in the process;
in the soft tissues of the extremities - the tumor reaches 5 cm, but is located within the sheaths of the fascia.

Stage 2 sarcoma is located inside the organ, all layers grow, disrupts the functional work of the organ with an increase in size, but there is no metastasis.

The oncological process manifests itself as follows:

in the oral cavity and on the tongue - a noticeable growth in the thickness of the tissues, the germination of all membranes, mucous membranes and fascia;
on the lips - by the germination of the skin and mucous membranes;
in the cellular spaces and soft tissues of the neck - up to 3-5 cm in height, going beyond the fascia;
in the larynx area - the growth of the node is more than 1 cm, the germination of all layers, which disrupts phonation and respiration;
in the thyroid gland - the growth of the node is more than 2 cm and the involvement of the capsule in the oncological process;
in the mammary gland - the growth of the node up to 5 cm and the germination of several segments;
in the esophagus - by the germination of the entire thickness of the wall, including the mucous and serous layers, the involvement of the fascia, severe dysphagia (difficulty swallowing);
in the lungs - by compression of the bronchi or by spreading to the nearest pulmonary segments;
in the testicle - by germination of the tunica albuginea;
in the soft tissues of the extremities - by the germination of the fascia that limit the anatomical segment: muscle, cellular space.

In the second stage, when the tumor is removed, the excision area is expanded, so relapses are not frequent.

Stage 3 sarcoma is characterized by the invasion of fascia and nearby organs. Sarcoma metastasizes to regional lymph nodes.

The third stage is manifested:

large size, severe pain syndrome, violation of normal anatomical relationships and chewing in the mouth and tongue, metastases in the LN under the jaw and on the neck;
large size, deforming the lip, spreading along the mucous membranes and metastases in the LU under the jaw and on the neck;
dysfunction of organs located along the neck: innervation and blood supply, swallowing and respiratory functions are disturbed with soft tissue sarcoma of the neck and cell spaces. With growth, the tumor reaches the vessels, nerves and nearby organs, metastases - the LU of the neck and sternum;
a sharp violation of breathing and distortion of the voice, invasion into organs, nerves, fascia and vessels in the neighborhood, metastasis from laryngeal cancer to the superficial and deep lymphatic cervical collectors;
in the mammary gland - large in size, which deforms the mammary gland and metastasis in the LU under the armpits or above the collarbone;
in the esophagus - huge sizes, reaching the fiber of the mediastinum and disrupting the food passage, metastases in the mediastinal lining;
in the lungs - by squeezing the bronchi with large sizes, metastases in the mediastinal and peribronchial lymph nodes;
in the testicle - deformation of the scrotum and germination of its layers, metastasis in the groin LU;
in the soft tissues of the arms and legs - tumor foci 10 centimeters in size. As well as dysfunction of the limbs and tissue deformation, metastases in the regional LN.

At the third stage, extended surgical interventions are performed, despite this, the frequency of sarcoma relapses increases, the treatment results are ineffective.

Stage 4 sarcoma is very difficult, the prognosis after its treatment is the most unfavorable due to its gigantic size, sharp squeezing of the surrounding tissues and germination in them, the formation of a continuous tumor conglomerate, which is prone to bleeding. Often there is a relapse of sarcoma of soft tissues and other organs after surgery or even complex treatment.

Metastasis reaches the regional LN, liver, lungs, and bone marrow. It initiates a secondary oncological process - the growth of a new sarcoma.

Metastases in sarcoma

The ways of sarcoma metastasis can be lymphogenous, hematogenous and mixed. From the pelvic organs, intestines, stomach and esophagus, larynx along the lymphogenous pathway, metastases of sarcomas reach the lungs, liver, bones of the skeleton and other organs.

Through the hematogenous pathway (through venous and arterial vessels), tumor cells or metastases also spread to healthy tissues. But sarcomas, for example, of the mammary and thyroid glands, pulmonary, bronchial, spread from the ovaries by lymphogenous and hematogenous pathways.

It is impossible to predict the organ where the elements of the microvasculature will accumulate and the growth of a new tumor will begin. Dust metastases of sarcoma of the stomach and pelvic organs spread along the peritoneum and thoracic region with hemorrhagic effusion - ascites.

The oncological process on the lower lip, tip of the tongue and in the oral cavity metastasizes more to the lymph nodes of the chin and under the jaw. Formations at the root of the tongue, at the bottom of the oral cavity, in the pharynx, larynx, thyroid gland metastasize in the LU of the vessels and nerves of the neck.

From the mammary gland, the onco cells spread to the clavicle region, to the LU on the outside of the sternocleidomastoid muscle. From the peritoneum, they get to the inner side of the sternocleidomastoid muscle and can be located behind or between its legs.

Most of all, metastasis occurs in adults, lymphosarcoma, liposarcoma, fibrous histiocytoma even with a size of up to 1 cm due to the accumulation of calcium in the tumor focus, intense blood flow and active growth of cancer cells. These formations lack a capsule that could limit their growth and reproduction.

The course of the oncological process is not complicated, and its treatment in connection with metastases to regional lymph nodes will not be so global. In case of distant metastases in internal organs, on the contrary, the tumor grows to a large size, there may be several of them. Treatment becomes more complicated, complex therapy is used: surgery, chemistry and radiation. As a rule, single metastases are removed. Excision of multiple metastases is not performed, it will not be effective. Primary foci differ from metastases in a large number of vessels, cell mitosis. There are more necrosing areas in metastases. Sometimes they are found earlier than the primary focus.

The consequences of sarcoma are as follows:

surrounding organs are compressed;
obstruction or perforation may occur in the intestine, peritonitis - inflammation of the abdominal sheets;
elephantiasis occurs against the background of impaired lymph outflow when the LN is compressed;
limbs are deformed, and movement is limited in the presence of large tumors in the area of bones and muscles;
internal hemorrhages occur during the decay of cancer.

Diagnosis of sarcomas

Diagnosis of sarcoma begins in the doctor's office, where it is determined by external diagnostic signs: exhaustion, jaundice, pale skin color and discoloration over the tumor, cyanotic lip shade, facial swelling, overflow of veins on the surface of the head, plaques and nodules with cutaneous sarcoma.

Diagnosis of sarcoma of high malignancy is carried out according to the pronounced symptoms of intoxication of the body: decreased appetite, weakness, increased body temperature and sweating at night. Cases of oncology in the family are taken into account.

When conducting laboratory tests, examine:

biopsy by a histological method under a microscope. In the presence of tortuous thin-walled capillaries, multidirectional bundles of atypical cancer cells, altered large-nucleated cells with a thin membrane, a large amount of substance between cells containing cartilaginous or hyaline connective tissue substances, histology diagnoses sarcoma. At the same time, the nodes lack normal cells characteristic of organ tissue.
abnormalities in the chromosomes of cancer cells by the cytogenetic method.
a blood test for specific tumor markers is absent, so there is no way to unambiguously determine its type.
general blood test: with sarcoma, it will show the following deviations:

hemoglobin and erythrocyte levels (less than 100 g / l) will significantly decrease, which indicates anemia;
the level of leukocytes will slightly increase (above 9.0x109 / l);
the number of platelets will decrease (less than 150․109 / l);
ESR will increase (above 15 mm / hour).

biochemical blood test, it determines an increased level of lactate dehydrogenase. If the concentration of the enzyme is higher than 250 U / L, then we can talk about the aggressiveness of the disease.

Diagnosis of sarcoma is complemented by a chest x-ray. The method can detect a tumor and its metastases in the sternum and bones.

Radiographic signs of sarcoma are as follows:

the tumor is round or irregular;
the size of the formation in the mediastinum is from 2-3 mm to 10 cm or more;
the structure of the sarcoma will be heterogeneous.

X-ray is necessary to detect pathology in the lymph nodes: one or more. In this case, the LU on the radiograph will be darkened.

If a sarcoma is diagnosed on an ultrasound scan, then it will be characteristic, for example:

heterogeneous structure, uneven scalloped edges and lesion of the LN - with lymphosarcoma in the peritoneal region;
the absence of a capsule, squeezing and pushing the surrounding tissues, foci of necrosis inside the tumor - with sarcoma in the organs and soft tissues of the abdominal cavity. In the uterus and kidneys (inside) or in the muscles, knots will be noticeable;
formations of various sizes without borders and with foci of decay inside them - with skin sarcoma;
multiple formations, heterogeneous structure and metastases of the primary tumor - with fatty sarcoma;
heterogeneous structure and cysts inside, filled with mucus or blood, indistinct edges, effusion in the cavity of the bag of joints - with joint sarcoma.

Tumor markers for sarcoma are determined in each specific organ, as in cancer. For example, in case of ovarian cancer, in case of breast sarcoma, gastrointestinal tract - CA 19-9 or, lungs - ProGRP (gastrin precursor, releasing, peptide), etc.

Computed tomography is performed with the introduction of an X-ray contrast agent to determine the localization, boundaries of the tumor and its forms, damage to the surrounding tissues, blood vessels, lymph nodes and their fusion into conglomerates.

Magnetic resonance imaging is performed to identify the exact size, metastases, destruction of the skin, bones, tissues, periosteum fibrillation, thickening of the joints and others.

The diagnosis is confirmed by a biopsy and determines the malignancy by histological examination:

bundles of tangled cells of a fusiform appearance;
hemorrhagic exudate - fluid coming out of the walls of blood vessels;
hemosiderin - a pigment formed during the breakdown of hemoglobin;
giant atypical cells;
mucus and blood in the sample and otherwise.

Lumbar (spinal) puncture analyzes will indicate sarcoma, where there may be traces of blood and many atypical cells of different sizes and shapes.

Do not remove:

after 75 years;
with severe diseases of the heart, kidneys and liver;
with a large tumor in vital organs that cannot be removed.

The following therapeutic tactics are also used:

With low and moderately differentiated sarcomas at stages 1-2, operations and regional lymph node dissection are performed. After - polychemotherapy (1-2 courses) or external radiation therapy for sarcoma.
With highly differentiated sarcomas at stages 1-2, surgical treatment and extended lymph node dissection are performed. it is performed before and after the operation, and is added with complex treatment.
At the third stage of the oncological process, combined treatment is carried out: before the operation - radiation and chemotherapy to reduce the size of the tumor. During the operation, all germinating tissues, collectors of regional lymph drainage are removed. They restore important damaged structures: nerves and blood vessels.
Amputation is often required for sarcoma, especially for osteosarcoma. Bone site resection is performed for poorly differentiated superficial osteosarcomas in older people. Next, prosthetics are performed.
At the 4th stage, symptomatic treatment is used: correction of anemia, detoxification and anesthetic therapy. For a comprehensive full-fledged treatment at the last stage, access to oncology is required in order to remove it, small size, location in the surface layers of tissues, single metastases.

Of the modern methods, external radiation therapy with linear accelerators is used according to special programs that plan the irradiation fields and calculate the power and dose of exposure to the oncological process zone. Radiotherapy is carried out under full computerized control and automatic verification of the correctness of the settings set on the accelerator control panel in order to exclude human error. used for sarcomas of different localization. It accurately irradiates the tumor with a high dose of radiation without damaging healthy tissue. The source is embedded in it by remote control. In some cases, brachytherapy can replace surgery and external radiation.

Traditional medicine for sarcoma

Treatment of sarcoma with folk remedies is included in complex therapy. Each type of sarcoma has its own medicinal herb, mushrooms, resins, food. Diet in the oncological process is of great importance, since fortified foods with the presence of micro- and macroelements increase immunity, gives strength to fight cancer cells, and prevents metastasis.

For malignant sarcomas, treatment is carried out:

infusions;
alcohol tinctures;
decoctions;
poultices.

Herbs are used:

henbane is black;
spotted hemlock;
bullock;
cocklebur;
white water lily;
grape-leaved clematis;
poppy samosei;
sun-glazed milk,
red fly agaric;
norichnik knotty;
mistletoe white;
incense pickle;
evading peony;
European wormwood;
ordinary hops;
common blackhead;
celandine is great;
sowing saffron;
ash high.

In case of chronic sarcoma ulcers on the skin and mucous membranes, they treat with avran medicinal, cutaneous sarcoma - with wolfberry, sweet clover, cocklebur, kirkazon and grape-leaved clematis, milkweed-sungazer and sweet-and-bitter nightshade, common tansy and common dodder and hops.

With an oncological process in the organs, drugs will be needed:

in the stomach - from wolfberry, crow, common dope and cocklebur, evading peony, large celandine and bitter wormwood;
in the duodenum - from aconites, marsh belozor;
in the esophagus - from spotted hemlock;
in the spleen - from wormwood;
in the prostate gland - from spotted hemlock;
in the mammary gland - from spotted hemlock, levkoin jaundice and common hops;
in the uterus - from the evading peony, wormwood, Lobel's hellebore and sowing saffron;
in the lungs - from the magnificent colchicum and cocklebur.

Osteosarcoma is treated with tincture: chopped St. John's wort (50 g) is poured with grape vodka (0.5 l) and insisted for two weeks with daily shaking of the container. Take 30 drops 3-4 times before meals.

For sarcoma, folk remedies are used according to the method of M.A. Ilves (from the book "The Red Book of the White Land"):

To increase immunity: mix in equal weight fractions: tartar (flowers or leaves), calendula flowers, tricolor and field violet, cocklebur, chamomile flowers and veronica, celandine and sandy immortelle flowers, mistletoe and young burdock root. Brew 2 tbsp. l. collecting 0.5-1 liters of boiling water and insist for 1 hour. Drink during the day.
Divide the herbs from the list into 2 groups (5 and 6 items) and drink for 8 days each collection.

Important! Collected plants such as celandine, violet, cocklebur and mistletoe are poisonous. Therefore, the dose cannot be exceeded.

To eliminate cancer cells, the treatment of sarcoma with folk remedies includes the following Ilves recipes:

grind celandine in a meat grinder and squeeze out the juice, mix with vodka in equal parts (preserve) and store at room temperature. Drink 3 times a day for 1 tsp. with water (1 glass);
grind 100 g of marin root (peony evading) and pour vodka (1 l) or alcohol (75%), leave for 3 weeks. Take 0.5-1 tsp. 3 times with water;
chop the white mistletoe, place in a jar (1 l) by 1/3, pour vodka to the top and let it brew for 30 days. Separate the thick and squeeze, drink 1 tsp. 3 times with water;
chop the meadowsweet root - 100 g and pour vodka - 1 liter. Insist 3 weeks. Drink 2-4 tsp. 3 times a day with water.

The first three tinctures should be alternated after 1-2 weeks. Meadowsweet tincture is used as a spare. All tinctures are taken for the last time before evening dinner. Course - 3 months, in between the monthly course (2 weeks) - drink meadowsweet. At the end of the 3-month course, drink a meadowsweet or one of the tinctures once a day for another 30 days.

Nutrition for sarcoma

The diet for sarcoma should consist of the following foods: vegetables, herbs, fruits, fermented milk, rich in bifido and lacto bacteria, boiled (steam, stew) meat, cereals as a source of complex carbohydrates, nuts, seeds, dried fruits, bran and sprouted cereals , wholemeal bread, cold-pressed vegetable oils.

To block metastases, the diet includes:

oily sea fish: saury, mackerel, herring, sardine, salmon, trout, cod;
green and yellow vegetables: zucchini, cabbage, asparagus, green peas, carrots and pumpkin;
garlic.

You should not eat confectionery, as they stimulate the division of cancer cells, as sources of glucose. Also products with the presence of tannin: persimmon, coffee, tea, bird cherry. Tannin, as a hemostatic agent, promotes thrombus formation. Smoked meats are excluded as sources of carcinogens. You can not drink alcohol, beer, the yeast of which feeds the cancer cells with simple carbohydrates. Sour berries are excluded: lemons, lingonberries and cranberries, since cancer cells actively develop in an acidic environment.

Life prognosis for sarcoma

Five-year survival rate for sarcoma of soft tissues and extremities can reach 75%, up to 60% - with an oncological process on the body.
In fact, even the most experienced doctor does not know how long they live with sarcoma. According to studies, the life expectancy in sarcoma is influenced by the forms and types, stages of the oncological process, the general condition of the patient. With adequate treatment, a positive prognosis is possible for the most hopeless cases.

Disease prevention

Primary prevention of sarcoma includes active identification of patients at increased risk of developing the disease, including those infected with herpes simplex virus VIII (HHV-8). It is especially necessary to monitor patients receiving. Prevention should eliminate and treat conditions and diseases that cause sarcoma.

Secondary prophylaxis is carried out in patients in remission to prevent recurrence of sarcoma and complications after a course of treatment. As a preventive measure, instead of tea, you should drink brewed herbs according to the Ilves method (item 1) for 3 months, take a break for 5-10 days and repeat the reception. You can add sugar or honey to tea.

Soft tissue sarcomas it is a group of malignant neoplasms developing in muscles, adipose, connective or fibrous tissues. Tumors can develop anywhere, but a favorite site of localization is the lower extremities, especially the thighs.

Soft tissue sarcomas look like white-gray nodules. They can have both bumpy and almost smooth surfaces. The consistency of the tumor can be soft (liposarcoma) or dense (fibrosarcoma). Lacking a capsule, a soft tissue sarcoma tends, as it grows, to provoke a thickening of the surrounding tissues and the formation of a so-called false capsule, which gives the pathological focus a clear outline. Most often, tumors are located singly, but there are exceptions. They metastasize in a hematogenous way (through the bloodstream).

Symptoms of the development of soft tissue sarcomas

Pathology is characterized by the appearance of a painless nodule or slight swelling. The neoplasm often has clear contours, but if the malignant focus lies deep, then the edges of the neoplasm may be uneven and difficult to determine. The skin over the tumor practically does not differ from healthy tissue; a local increase in temperature can be observed.

With an increase in the growth of a neoplasm, an expanded venous network and ulcerative areas often appear. During movement, uncomfortable sensations may occur, although there is no pronounced impairment of limb mobility in this disease.

Treatment of soft tissue sarcomas

In the treatment of soft tissue sarcomas, complex techniques are used, including surgery, chemotherapy and radiation exposure. The tumor is excised with a large capture of healthy tissue. When it grows into bone tissue, amputation of the limb and the entire area of the affected tissue is often performed. Radiation therapy is used to reduce the size of the tumor before surgery.

After tumor resection, the course of radiation treatment is repeated, which can significantly reduce the risk of recurrence of the disease. Adjuvant chemotherapy is widespread among modern methods of treatment. It promotes stable remission of the malignant process and prevents the spread of metastases.

An integrated approach to the treatment of soft tissue sarcomas can improve the quality of life of patients and save their lives. With timely treatment, a large percentage of people achieve complete recovery and return to their usual way of life. For a successful outcome of the disease, it is very important to select competent specialists with successful experience in the treatment of such diseases.

Malignant fibrous histiocytoma

Malignant fibrous histiocytoma is one of the most common sarcomas. It can be localized in various internal organs, but especially often develops in the retroperitoneal space and extremities. The tumor looks like a node with fuzzy edges and small hemorrhages. Sometimes the tumor also develops in the bones.

Malignant histiocytoma is considered the most common form of soft tissue tumors. It accounts for more than 40% of these malignant neoplasms. Almost always, the tumor is localized in the middle or deep muscle layers. It affects people 40-65 years old. Fibroxanthosarcoma is characterized by a dense consistency, slow growth, prone to unexpected leaps.

Signs of tumor development

If a malignant fibrous histiocytoma is localized in the bone tissue, then a painful attack will be a sign of the disease. With the growth of the tumor, the load on the bone increases and it tends to break even with a slight fall. On palpation, the tumor is painful.

With histiocytoma of the abdominal cavity, the tumor can press on nearby organs, provoking a malfunction of the bladder, uterus and rectum. The clinical picture of the disease can be different, since there are many varieties of malignant fibrous histiocytomas.

Methods for diagnosing a tumor

To detect malignant histiocytoma, the following diagnostic methods are used:

ultrasound procedure;
radiography;
magnetic resonance imaging, computed tomography;
puncture, biopsy;
histological examination.

In case of unexpected pain, swelling, impaired mobility, it is necessary to undergo a comprehensive examination. The sooner a tumor is diagnosed, the easier it is to treat it using low-traumatic surgical interventions. The level of development of modern medicine allows for minimally traumatic operations, while maintaining the functional abilities of the body and a high quality of life.

Treatment options for MFH

Patients with malignant fibrous histiocytoma are shown complex treatment, including both surgical excision of the tumor, and radiation and chemotherapeutic treatment. Radiation exposure is used before and after surgery. Chemotherapy is indicated for large tumors.

In modern oncological centers, chemotherapy drugs of a new generation have begun to be used, which do not have such pronounced side effects. But, unfortunately, when a malignant fibrous histiocytoma metastasizes, it can be extremely difficult to help a person and remove all existing metastases.

The main therapeutic measure in the presence of this tumor is its complete excision. During the operation, in addition to the pathological focus, the surgeon also removes healthy tissue. It is often necessary to resort to radical excision, excising more than half of the muscle layer. Amputations of the affected limb are also not uncommon.

Malignant fibrous histiocytoma often recurs after surgical treatment. More than 25% of patients after tumor removal are faced with isolated cases of relapses, 10% - with multiple foci of the tumor-like process. In more than 90% of operated patients, local relapse develops within the first five years after treatment.

Malignant fibrous histiocytoma begins to metastasize within the first year after detection. If you take all measures to successfully remove it, then you can hope for a successful treatment outcome. In 80%, the tumor metastases to the lungs, much less often to the retroperitoneal space, bones and lymphatic vessels.

Forecast

The duration of patients with fibrous histiocytoma is influenced by many factors: the size of the tumor, the stage of its development at the time of treatment, the severity of metastasis, and the degree of malignancy. It is very important to pay attention to the signs of the disease in time and start treatment of the tumor in the appropriate medical institution as soon as possible.

On average, more than 70% of patients with favorable disease and treatment rates live for more than 7-10 years. In the presence of severe metastasis, multiple cases of relapse, the period is halved. The effectiveness of the treatment carried out largely depends on the experience of the surgeon and his competence. Malignant tumors are operated only by trained specialists who are familiar with the peculiarities of the course and localization of a particular malignant neoplasm.

Liposarcoma

Liposarcoma is a tumor that arises from the cells of adipose tissue. In terms of the frequency of detection, this tumor ranks second among all soft tissue neoplasms. A favorite place for localization of liposarcoma is the lower extremities, especially the thigh area. Similar tumors are quite common in the retroperitoneal space. The average age of patients with liposarcomas is 50-60 years. Pathology more often affects men, extremely rarely - children. This type of sarcoma is characterized by slow growth. Tumors rarely metastasize, mainly to the lungs, bone marrow or liver.

Liposarcoma symptoms

Liposarcoma is often localized in the deep layers of the skin, up to the periarticular tissues. During palpation, the tumor is usually painless. Due to this feature, the tumor is not diagnosed immediately, but only when pronounced signs of the disease appear:

an increase in the size of the limb or the area in which the liposarcoma is located;
the appearance of functional disorders of internal organs when the tumor is localized in the retroperitoneal space.

Diagnostics of the liposarcoma

At the initial stage of development, liposarcoma must be differentiated from lipoma. For a full examination, the clinic must have modern equipment and oncologists who often have to deal with sarcomas and successfully diagnose them.

Among the common diagnostic methods, the following can be distinguished:

ultrasound procedure;
skeleton scintigraphy.

On palpation, liposarcoma has an elastic consistency, a limited surface, and a lobular structure. An experienced specialist can easily determine the type of tumor by probing.

Liposarcomas are of several types: embryonic lipoma, lipoblastic, myxomatous, vyssokodifferentiated liposarcoma. Myxomatous tumor is characterized by accelerated growth. It grows rapidly in size within a short period of time. Highly differentiated liposarcomas grow very slowly and are usually diagnosed when they are large. They are often discovered quite by accident, during a preventive examination.

Liposarcoma Treatment

Like any type of sarcoma, liposarcoma is treated mainly with surgery. During the operation, not only the tumor itself is subject to removal, but also part of the surrounding tissues. If liposarcoma is localized deep in the tissues of the lower extremities, has a large size, then sometimes it is necessary to carry out partial bone resection followed by transplantation.

After surgical excision of the pathological area, radiotherapy is performed. In modern clinics, it is possible to apply precise radiation treatment, affecting a specific area or organ. With the help of such tactics, it is possible to minimize the negative effects of ionizing radiation on healthy tissues. If liposarcoma is not subject to surgery or has managed to give multiple metastases, then chemotherapy treatment is indicated. Chemotherapy drugs have a partial effect on malignant liposarcoma cells that have spread throughout the body. This technique is also used if surgical intervention is contraindicated for the patient.

Patients with liposarcomas require a carefully planned complex treatment. Medical tactics are chosen taking into account the size of the tumor, its localization, the degree of malignancy and the general well-being of the patient. Differential diagnosis and timely therapeutic measures play a huge role in a favorable outcome of the disease. The earlier and more accurately the diagnosis is made, the better the prescribed treatment will be.

Forecast

The prognosis depends on the histological type of tumor and the degree of its malignancy. If the patient undergoes an operation with complete removal of abnormal cells, then the risk of relapse will be minimal. With the help of diagnostics, all possible metastases should be identified. Overall, the recurrence rate among operated patients is 35%. Full recovery is observed in 30% of cases. Five-year survival rate after the course of treatment is more than 55%.

Leimiosarcoma

Leimiosarcoma it is an oncological neoplasm that develops from smooth muscle fibers, a type of malignant leiomyoma. The tumor can be localized in the uterus, on the lower limbs, neck or head, less often in internal organs. The average age of the sick is 40-65 years.

Leimiosarcoma is characterized by a high degree of malignancy and rapid growth. The tumor is prone to early metastasis. The disease is extremely malignant and is quite rare.

Signs of leiomyosarcoma

The main sign of pathology is the detection of a tumor with progressive growth on examination or by examination. As the leiomyosarcoma develops, the following symptoms may occur:

the appearance of a pathological neoplasm on the scalp, neck or lower extremities in the form of a plaque with uneven edges - elastic and dense, lying in the deep layers of the skin;
the tendency of the neoplasm to become covered with ulcers and bleed;
characteristic color of the skin over the tumor: light yellow, red or blue;
soreness of the tumor;
malfunctions in the work of internal organs (with the growth of a tumor and its pressure on adjacent tissues).

Diagnosis of leiomyosarcoma

Timely diagnostics increases the chances of a full recovery and prevents tumor metastasis. The following methods are used to detect the disease:

biopsy of tumor tissues: if leiomyosarcoma is localized in the internal organs, then puncture or endoscopic methods are used to collect material;
histological examination of the collected material during the biopsy;
ultrasound diagnostics;
X-ray examination;
magnetic resonance imaging, computed tomography.

Diagnostics of malignant neoplasms should be carried out as early as possible, in specialized diagnostic centers. Deciphering the results obtained should be dealt with by experienced specialists familiar with the peculiarities of the development of leiomyosarcoma and their display on the images.

Treatment options for leiomyosarcoma

Treatment of leiomyosarcoma should be comprehensive, taking into account the stage of the disease, the degree of malignancy and possible risks to the patient's life. Tumors of this type are subject to mandatory surgical excision.

If the leiomyosarcoma is located on the surface of the skin, then it is removed with a slight capture of healthy tissue. For subcutaneous tumors, the surgeon must eliminate the neoplasm along with its capsule and adjacent tissues. If metastasis is detected in the lungs, liver, bone marrow or other organs, the metastases are removed together with the tumor.

If the leiomyosarcoma is impressive in size and has a high degree of malignancy, then radiation therapy is mandatory. In some cases, complete amputation of the limb is indicated, which allows the patient to survive and avoid frequent relapses. According to statistics, in 80% of cases after radical removal of the tumor, relapses occur after 2-6 months.

Chemotherapy drugs are used for the spread of metastases to internal organs and tissues. Cytostatics are often used not only after surgery, but also before surgery, in order to reduce tumor growth and partially eliminate metastases. Due to the high risk of malignancy, patients with leiomyosarcomas are subject to complex treatment that combines traditional medical procedures.

Forecast

Despite a poor prognosis, patients with leiomyosarcoma have a good chance of recovery. It is very important to start treatment immediately after detecting a tumor, preferably in a specialized oncological clinic with modern technical equipment. Professional surgeons-oncologists and radiotherapists will select an effective therapy regimen in accordance with the degree of the disease and the state of the patient's body. With the observance of medical recommendations and the use of improved methods of treatment, it is possible to avoid recurrence of the disease and the spread of metastases.

Synovial sarcoma

Synovial sarcoma (malignant synovioma) is one of the most common malignant neoplasms of soft tissues that can grow into bone tissue. Most often it develops from the synovial membrane of the articular structures of the lower extremities. The disease affects people of working age, usually up to 50-60 years. The tumor does not metastasize immediately, but as the process progresses, usually to the bones and lungs. In 40% of cases, synovial sarcoma affects the ankle and knee joints.

There is no exact reason for the development of a malignant synovioma. It is believed that there are certain risk factors that can lead to the onset of pathology, including changes at the genetic level, and radiation exposure, and carcinogenic effects on the human body. Sometimes synoviomas occur against the background of a post-traumatic condition.

Signs of synovial sarcoma

On palpation of the tumor, it has a medium density consistency. With calcification, the tumor becomes harder on the neoplasm. The tumor itself, as a rule, has slit-like spaces and cysts, as well as necrotic areas with signs of hemorrhage.

Cystic cavities often contain characteristic contents that resemble synovial fluid. With the growth of a malignant synovioma, joint pain occurs, a restriction in the mobility of the joint affected by the malignant process. With metastasis, signs of malaise may appear, a local increase in the size of the lymphatic vessels, a decrease in appetite and body weight.

Diagnosis of synovial sarcoma

Malignant synovioma requires a comprehensive diagnosis, including the following methods:

X-ray research methods;
computed, magnetic resonance imaging;
angiography;
histological examination;
biopsy of the neoplasm;
radioisotope scanning;
puncture of the lymph nodes to confirm metastasis;
scintigraphy;
ultrasound procedure.

Effective diagnosis of synovial sarcoma involves a combination of advanced research methods. The tumor must be differentiated from fibrosarcoma, mesothelioma and epithelioid sarcoma.

Modern cancer centers are very well equipped today. Often sarcomas are detected during preventive examinations. In any case, the treatment of synovial sarcoma does not require delay and should begin immediately after a tumor is detected.

Treatment options for malignant synovioma

Malignant synovium is treated with combined methods, including:

surgical excision of the pathological focus;
radiation treatment;
chemotherapy.

During the operation, not only malignant tissues are removed, but also healthy ones within 4 centimeters. If the synovioma has an impressive size, it is possible to carry out a complete amputation of the limb or resection of the entire joint with further endoprosthetics.

Before and after surgery, radiation is indicated. Radiation therapy helps to stop or reduce tumor growth, as well as prevent the development of relapses in the postoperative period. This technique is carried out in courses, the treatment regimen is selected individually. Chemotherapy is not always carried out, but in the case of severe metastasis and a high degree of tumor malignancy. Reception of modern cytostatics allows you to negatively affect micrometastases and significantly improve the quality of life of patients with sarcomas.

Forecast

The most malignant is the monophasic synovioma. It often metastasizes to the lungs, tends to recur even after complete excision of the pathological focus in 20% of cases in all patients who have completed the full course of treatment. The survival rate is low. But with timely treatment, it is possible to avoid the spread of metastases and achieve a stable remission. The biphasic form of synovial sarcoma has a more favorable course. About 50% of patients with such a tumor recover completely and do not experience any restrictions in later life.

Rhabdomyosarcoma

Rhabdomyosarcoma- a rare type of malignant tumor that grows from striated muscles. The pathological process is characterized by uncontrolled growth and division of skeletal muscle cells, as a result of which they become similar to rhabdomyoblasts - rudimentary muscle embryonic cells. The tumor mainly affects pediatric patients, up to 10 years of age. Boys get sick more often than girls.

The reasons for the development of rhabdomyosarcoma

Until now, scientists have not identified the exact reasons for the development of rhabdomyosarcomas. But long-term observations of patients with this group of sarcomas revealed a number of predisposing factors:

increased radiation exposure to the child during intrauterine development or after birth;
trauma, damage;
gene mutations;
intoxication, the negative impact of toxic and toxic substances.

In patients with such tumors, malformations of the genitourinary system, central nervous system and digestive tract are often diagnosed. Rhabdomyosarcoma often develops against the background of pathologies that provide a predisposition to the development of malignant tumors: Li-Flaumeni, Rubinstein-Teibi, Wiedemann-Beckwith syndromes.

Diagnosis of rhabdomyosarcoma

To diagnose patients with suspected rhabdomyosarcoma, the following procedures are performed:

ultrasound diagnostics;
X-ray examination;
positron emission tomography;
tumor biopsy;
excretory urography;
scintigraphy;
ENT diagnostics;
cytological examination of cerebrospinal fluid;
computer, magnetic resonance imaging.

The research method is chosen depending on the location of the tumor and the course of the disease. Often it is necessary to carry out complex diagnostics, sometimes the tumor tissue can be removed by means of minimally invasive endoscopic procedures. Diagnosis of rhabdomyosarcoma must be carried out in specialized oncological centers, with the appropriate technical equipment and a staff of professional doctors.

Symptoms of the development of rhabdomyosarcoma

Rhabdosarcoma rises above the surface of the skin, has a dense texture. Metastasizes most often to the lungs and bones. Symptoms of the tumor depend on the site of its localization. Rhabdomyosarcoma of the neck sometimes occurs with partial or complete disappearance of the voice.

If the tumor is localized in the nasal cavity, then it leads to chronic sinusitis, nosebleeds. With the germination of a neoplasm in the pelvis, a violation of urination may occur, up to acute urinary retention, spotting from the genital tract, pain during urination and difficulty in the act of defecation, in the vagina, clusters of tumors may be found in the vagina. A neoplasm in the face and neck area is often accompanied by paralysis and respiratory failure.

Treatment methods for rhabdomyosarcoma

Treatment for rhabdomyosarcoma should be comprehensive. It includes: radical removal of the tumor, chemotherapy techniques, radiation exposure. Modern cytostatics (cyclophosphamide, vincristine, etoposide, dactinomycin) can significantly reduce the risk of complications even during a long course of chemotherapy.

In the presence of a large tumor, radiation therapy with local irradiation of the malignant zone is performed before surgery. Pathological foci are excised within healthy tissue, sometimes together with regional lymph nodes. If the tumor affects the soft tissues of the extremities and grows into the bone tissue, then, as a rule, amputation is required.

Localization of rhabdomyosarcoma in the genitals often requires their complete removal. This reduces the risk of recurrence of the pathology in the future. Radical treatment of this type of sarcoma involves the surgical removal of metastases in places where it is possible to do this. The most unfavorable course of the disease is tumor metastasis to the skeletal system.

Forecast

The prognosis of the survival of patients with rhabdomyosarcoma depends on the degree of malignancy of the process and the stage of tumor development. At the last stage of the course of the disease, the prognosis is poor. The initial stages of the tumor have a high survival rate and complete recovery. The younger the patient, the more successful the treatment of this disease is.

Russian Cancer Research Center. N.N. Blokhin Russian Academy of Medical Sciences, Moscow

Abstract. Sarcoma is a soft tissue disease characterized by a highly malignant active course. For patients in this group, combination therapy is recommended for initial treatment. We research and develop modern drugs for the treatment of soft tissue sarcomas.

Key words: epidemiology, localization, morphology, sarcoma, diagnosis, staging, treatment.

I. Epidemiology

Soft tissue sarcomas are tumors of the extra-skeletal connective tissue of the human body, i.e. ligaments, tendons, muscles and adipose tissue originating from the primitive mesoderm. This group also includes tumors from Schwann cells of the primitive ectoderm and endothelial cells lining the vessels and mesothelium. This heterogeneous group of tumors is united due to the similarity of the morphological picture, mechanisms of occurrence and clinical manifestations. Visceral sarcomas are extremely rare in the breast, kidney, prostate, lungs and heart and are more sensitive to treatment regimens used to treat sarcomas, rather than epithelial tumors of the same organs. The nomenclature classification reflects the microscopic picture and the degree of differentiation, while in some cases it may indicate an organ affiliation - leiomyosarcoma has the characteristics of smooth muscle tissue, microscopically and most often occurs in organs with the largest number of smooth muscle fibers (uterus, gastrointestinal tract), while synovial sarcoma does not arise from the synovium.

1. Morbidity

Sarcomas of bones and soft tissues are a relatively rare group of tumors. In Russia, about 10,000 new cases are registered annually, which is 1% of all malignant neoplasms. The incidence is 30 cases per 1,000,000 population, 80% are soft tissue sarcomas. In childhood, the incidence is higher and amounts to 6.5%, ranking 5th in morbidity and mortality.

2. Etiology

Genetic predisposition plays a role in the following cases:

Nonvoid basal cell syndrome (Gorlin's syndrome) is an autosomal dominant disease characterized by cutaneous manifestations in the form of multiple basal cell carcinomas, epidermal cysts, depressions of the skin on the palms and feet, as well as cysts of the lower and upper jaws, ribs, vertebrae , short metacarpal bones, ovarian fibromas and hypertelorism. The most common cases are medulloblastoma and fibrosarcoma of the jaw;

Neurofibromatosis (von Recklinghausen's disease) is an autosomal dominant disease characterized by the presence of multiple neurofibromas, axillary freckles and giant nevi, as well as bilateral acoustic neuromas, meningeomas, and fibrous bone dysplasia. The most common occurrences are neurofibrosarcoma (10-15%), malignant neurilemoma (5%), pheochromocytoma, astrocytoma and glioma;

Tuberous sclerosis (Borneville disease) is an autosomal dominant disorder with cutaneous manifestations in the form of hypopigmented masses, adenomas of the sebaceous glands, inguinal fibroids, also characterized by manifestations of epilepsy, mental retardation, hamartomas of the brain, kidneys, liver, adrenal glands, pancreas and heart (rhabdomyoma of the heart is detected in most patients), astrocytomas and glioblastomas most often occur;

Gardner's syndrome is an autosomal dominant disease manifested by skin changes in the form of dermoid or epidermoid cysts, sebaceous cysts, lipomas, fibroids and desmoids, as well as colon polyps, multiple osteomas, including the bones of the skull and jaw. Colon adenocarcinoma is very common;

Werner's syndrome (progeria) is an autosomal recessive disorder characterized by premature aging with skin changes like scleroderma, baldness, trophic ulcers of the extremities. The most common are sarcomas and meningeomas (10%).

Patients with secondary lymphostasis after mastectomy with lymphadenectomy significantly increase the risk of developing angiosarcomas (Steward-Treves syndrome).

Injury. There is no etiological relationship between trauma and sarcomas. In most patients, the trauma draws attention to the growing tumor and is a coincidence.

Ancerogens. An increase in the number of cases of angiosarcomas in patients who worked with vinyl chloride and arsenic was noted. Studies have not shown the dependence of the incidence of sarcomas on carcinogens such as chlorophenols and phenoxyacetic acids.

Radiation. Radio-induced sarcomas are rare and can occur in tissues that are exposed to ionizing radiation. Osteosarcoma and malignant fibrous histiocytoma are the most common histological subtypes. These tumors usually appear 6-30 years or more after the irradiation (median 10 years) and are extremely rare in the early stages (2-4 years). The total dose of radiation, the fractionation regime and the type of radiation affect the incidence. Alkylating agents (cyclophosphamide, etc.) in combination with radiation therapy also increase the risk of secondary malignant neoplasms.

Immunosuppression The most common example is Kaposi's sarcoma in patients with AIDS, CLL and autoimmune hemolytic anemia, as well as in patients after organ transplantation.

Viral etiology. Hepres virus type 8 (HHV8) is detected in AIDS patients; HHV8 DNA was detected in the skin lesions of homosexual men, not infected with HIV, in classical and endemic (African) forms of Kaposi's sarcoma.

II. Localization of soft tissue sarcomas

1. Soft tissue sarcomas of the extremities account for 60% of the total and occur on the lower and upper extremities in a ratio of 3: 1. About 75% of sarcomas (including bone sarcomas) occur in the knee joint.

2. Sarcomas of the head and neck occur rarely, with a frequency of no more than 10%.

3. Trunk and retroperitoneal space - 30%, with 40% being retroperitoneal tumors.

III. Morphology

1. Transformation and dedifferentiation of a benign soft tissue tumor into a malignant one is rare. Differences in the frequency of occurrence of different histological subtypes of soft tissue sarcomas are due to different conclusions of pathomorphologists, and not the variable frequency of occurrence of different subtypes.

2. The biology of each tumor subtype can vary from benign without metastatic potential, more aggressive with locally invasive growth, to malignant with high metastatic potential. For each histological subtype of sarcomas, the tendency to metastasize directly depends on the size and degree of malignancy of the tumor. Thus, highly malignant tumors larger than 5 cm are considered to be tumors with a very high potential for metastasis and vice versa.

3. The main characteristics of malignancy are: frequency of mitosis, morphological characteristics of the cell nucleus, cellularity. Cellular anaplasia, or polymorphism, and the presence of necrosis are the most important factors in determining the grade of malignancy. Determining the grade of malignancy is a subjective procedure, therefore some pathologists prefer to classify sarcomas into 2 types: high grade or low grade. In different classifications, the 3rd or 4th degree is used.

4. Cytogenetics: Chromosomal changes have been reported in many sarcomas. At present, their identification is used only for a more thorough diagnosis of one or another histological subtype. These data have not yet received clinical application.

IV. Locally aggressive soft tissue tumors

1. Nodular fasciitis - pseudosarcomatous, or proliferative, fasciitis is treated with simple excision. The morphologically differential diagnosis is with fibrosarcoma. This tumor, as a rule, does not exceed 5 cm in diameter, is usually asymptomatic, from the moment of appearance it grows very quickly to the specified size, then growth slows down, and a plateau sets in.

2. Atypical lipomatous tumor - a synonym for liposarcoma of the 1st degree of malignancy. Does not have metastatic potential, but requires extensive excision due to the high risk of local recurrence. Usually occurs in the abdominal cavity or retroperitoneal space, can become large and difficult to remove due to proximity to internal organs. This tumor can dedifferentiate into malignant fibrous histiocytoma (dedifferentiated liposarcoma).

3. Desmoid is a low-grade tumor characterized by invasive growth. Synonyms: aggressive fibromatosis or musculo-aponeurotic fibromatosis. Requires wide excision, as there is a high incidence of local recurrence with a positive / borderline resection margin. Radiation therapy helps to achieve better local control, is used in the primary treatment of recurrent tumors or as an adjuvant after surgical excision. In the treatment of patients with relapses in the irradiated area or requiring extensive resections, or with unresectable tumors, systemic chemotherapy is possible. The use of tamoxifen gives 15-20% of objective answers, doxorubicin in combination with dacarbazine - more than 60%. There is evidence of the effectiveness of weekly administration of methotrexate in low doses. The responses are usually slow and delayed.

4. A giant cell tumor of the tendons and synovial membranes occurs on the hand and requires routine excision. If large joints are involved, then total synoviumectomy can be used. Sometimes these tumors cause erosion of bone structures and may look like primary bone tumors on radiographs.

V. Common soft tissue sarcomas

1. Malignant fibrous histiocytoma (MFH) is the most common soft tissue sarcoma. It occurs in the 50-70 age group. Morphologically, it is characterized by high cleavage and pleomorphism, and has a very aggressive course. The myxoid variant (currently myxofibrosarcoma) flows less aggressively.

2. Rhabdomyosarcoma - there are 3 types: pleomorphic, alveolar and embryonic. Fetal is the most common histological subtype in children. It is a systemic disease, and after the diagnosis is made, treatment begins with systemic chemotherapy, followed by surgery or radiation therapy to achieve local control, followed by postoperative chemotherapy. The pleomorphic variant usually occurs in adulthood, has a poor prognosis and extremely low cure rates.

3. Liposarcoma - myxoid liposarcoma is an analogue of liposarcoma of the 2nd degree of malignancy, characterized by a sluggish course and can metastasize into soft and adipose tissues of various localizations and the abdominal cavity. Pleomorphic liposarcoma is a grade 3 (G3) tumor that usually occurs on the extremities and metastases to the lungs.

4. Leiomyosarcoma arises from smooth muscle cells, can be localized in any part of the body, originating from smooth muscle cells of the vascular wall. Most often occurs in the uterus or gastrointestinal tract. Leiomyosarcomas of the gastrointestinal tract rarely respond to chemotherapy, while leiomyosarcomas of the uterus are sensitive to ifosfamide with doxorubicin and the combination of gemzar with taxotere. Leiomyosarcomas of the skin and subcutaneous fat are relatively benign tumors, do not metastasize and are treated only by surgery.

5. Synovial sarcoma. Histologically, 2 types are distinguished - monophasic and biphasic. Usually occurs on the limbs, but it can also be on the trunk, abdominal wall, or internal organs. It is characterized by aggressive growth and good sensitivity to chemotherapy. In 1/3 of cases, calcifications are found on radiographs.

6. Neurofibrosarcoma - a malignant tumor of the membranes of the peripheral nerves, or malignant schwannoma. Often occurs in patients with Recklinghausen's disease. In 50% it occurs in patients with neurofibromatosis.

7. Angiosarcoma is a tumor of vascular origin. (Lymph) angiosarcomas are rare, often secondary to mastectomy due to chronic lymphostasis. (Heme) angiosarcomas can occur anywhere in the body, but are most common in the skin and superficial soft tissues of the head and neck.

8. Hemangiopericytoma is extremely rare, characterized by sluggish growth and local recurrence. Histologically, it resembles synovial sarcoma.

9. Alveolar sarcoma of soft tissues. The cellular nature of the origin is unknown. In adulthood, the tumor is most often detected in the thickness of the thigh muscles, in childhood, as a rule, in the region of the head and neck.

10. Epithelioid sarcoma is more common in the form of tumor formation in the distal extremities, based on the aponeurotic structures. There is a high incidence of metastasis to the skin, SFA, adipose tissue, bones and lymph nodes. Local recurrence usually occurs above the site of the previous operation.

Vi. diagnostics

1. Most patients complain of asymptomatic tumor formation. Symptoms appear due to compression of vital structures, so a small mass on the hand can cause pain or impairment of motor activity, and a large mass on the back does not cause any symptoms. Symptoms may appear due to compression or traction of the nerve trunks. About 20-25% already come with a disseminated process - metastases to the lungs, bones and liver (according to the frequency of occurrence).

2. The technique of performing a biopsy of the tumor is extremely important, the fundamental point is the choice of the biopsy site. The biopsy should be performed at a site that will subsequently enter the area of tumor excision according to the rules of ablasty. Currently, open biopsy of a tumor is widely used, which is associated with the possibility of obtaining a larger amount of tumor material for a qualitative morphological study.

3. The plan of examination of patients with soft tissue sarcomas should include:

Computed tomography (for tumors of the abdominal cavity and retroperitoneal space);

Magnetic resonance imaging (for tumors of the extremities, trunk, head and neck region);

Computed tomography of the lungs.

Vii. Staging

TNM classification is based on the size and depth of the tumor relative to the superficial fascia for sarcomas of the extremities (a - superficially located, b - growing into the fascia and all tumors of the abdominal cavity, pelvic cavity, chest and retroperitoneal space).

AJCC staging system, 2002, 6th revision

G - grade of malignancy:

G1 - highly differentiated;

G2 - moderately differentiated;

G3 - poorly differentiated;

G4 - dedifferentiated (only for 4-stage system);

N - regional lymph nodes;

N0 - no histologically verified affected lymph nodes;

N1 - metastases to regional lymph nodes;

T - primary tumor;

T1a, superficial tumor;

T1b - deep tumor;

T2 - tumor more than 5 cm in diameter;

T2a, superficial tumor;

T2b - deep tumor;

M - distant metastases;

M0 - no distant metastases;

M1 - there are distant metastases.

Grouping by stage:

T1a, b N0 M0, G1-2 (G1 in 3-stage system);

T2a, b N0 M0, G1-2 (G1 3-stage).

Stage II.

T1a, 1b N0 M0, G3-4 (G2 in 3-stage system); T2a N0 M0, G3-4 (G2 in 3-stage system).

Stage III.

T2b N0 M0, G3-4 (G2 in 3-stage system).

Stage IV.

Any T N1 M0, any G. Any T N0 M1, any G.

In the TNM system, a 4-step scale of malignancy is presented, however, after 2002, the use of a 3-step system was adopted, in which G3 and G4 are combined together, which does not change the essence and is easier to use.

Determining the degree of malignancy of sarcomas is very important for the choice of tactics for further treatment. There are 2 identification systems - the NCI system (US National Cancer Institute) and the FNCLCC system (French Federation Nationale des Centers de Lutte Contre le Cancer). According to the NCI system, the histological subtype, the number of cells in the field of view, pleomorphism, the number of mitoses and the severity of foci of necrosis are assessed.

Distinguish:

Grade 1 - 1st degree of malignancy (highly differentiated - the best prognosis, rarely metastasizes, practically insensitive to chemotherapy).

Grade 2 - 2nd degree of malignancy (moderately differentiated).

Grade 3 - 3rd degree of malignancy (low-differentiated, poor prognosis, metastases very often, most are chemically sensitive).

In Europe, the most commonly used system is FNCLCC (French Federation Nationale des Centers de Lutte Contre le Cancer), which is also a 3-step system, and the total score for tumor differentiation, mitotic index and the number of necrosis is assessed. In fact, the degrees of both scales are the same.

VIII. Treatment

1. Surgery

Soft tissue sarcomas develop in a capsule that pushes the surrounding tissue apart as the tumor grows. This membrane is not true, as it is infiltrated by tumor cells and is called a pseudocapsule. During surgery, it is necessary to remove the tumor according to oncological principles together with the pseudocapsule, without opening it, otherwise the risk of recurrence increases sharply. Careful hemostasis is also extremely important, the spread of tumor cells within the boundaries of the postoperative hematoma occurs quickly, and the likelihood of recurrence is very high. In such cases, postoperative radiation therapy is mandatory. Removal of the tumor should be performed as a single block (en bloc) with negative resection margins. To provide better local control in high-grade sarcomas in the postoperative period, radiation therapy can be performed with the location of tumors on the limbs and trunk. It is difficult to achieve absolutely negative resection margins when performing surgical interventions for retroperitoneal sarcomas. A large area of the resected tumor bed can potentially contain tumor cells; however, the use of postoperative radiation therapy in a cytotoxic dose may be impossible due to the low tolerance of internal organs such as the liver, kidneys and gastrointestinal tract. The routine use of postoperative radiation therapy for primary retroperitoneal sarcomas is not recommended. 2. Radiation therapy and preoperative radiation therapy have shown advantages in the form of a possible reduction in tumor size and improved operating conditions, a smaller radiation field (tumor + resection margins compared to the removed tumor bed + resection margins) and a lower radiation dose (usually 50-54 Gr). At the same time, the main negative point is the high percentage of postoperative complications of an infectious nature.

Postoperative radiation therapy has shown advantages in the absence of postoperative complications associated with wound healing; the entire tumor sample is available for examination by a pathomorphologist and assessment of the true size and extent of the primary tumor. Among the negative aspects, it is necessary to note the large dose and the field of radiation.

Trachytherapy can be performed perioperatively, takes less time and does not exceed postoperative radiation therapy in terms of effectiveness (with the exception of low-grade tumors).

And intraoperative radiation therapy can be used in the treatment of deep-seated and retroperitoneal tumors when the risk of complications from conventional radiation therapy is very high.

3. Chemotherapy

The study of the value of adjuvant chemotherapy with doxorubicin in soft tissue sarcomas has yielded conflicting results. A 2008 meta-analysis based on data from 1568 patients from 14 clinical trials showed an absolute advantage of adjuvant therapy in 6% of cases for the local relapse-free interval and in 10% for the occurrence of distant metastases. By 10 years of observation, there was no effect on overall survival. The rather low efficacy rates may be due to the fact that the histological subtype of the tumor was not taken into account when calculating the results (the study included patients with GIST, alveolar and clear cell sarcomas that are not sensitive to standard chemotherapy, as well as retroperitoneal sarcomas). It is generally accepted that patients with a high degree of malignancy (Gr. 3 and 4) and tumors larger than 5 cm can participate in clinical trials to study new regimens of adjuvant chemotherapy. The results of one of similar studies conducted in Italy using epidoxorubicin (pharmacorubicin) at a dose of 60 mg / m2 on days 1 and 2, ifosfamide - 1.8 g / m2 on days 1-5 with filgrastim - 300 μg / day on days 8-15, 5 courses every 3 weeks demonstrated a significant increase in relapse-free (median 48 months and 16 months; p = 0.04) and total (median 75 months and 46 months; p = 0 , 03) survival rate. Radical surgery is often impossible in patients with retroperitoneal sarcomas. However, randomized trials show no improvement in outcomes with preoperative neoadjuvant or postoperative adjuvant chemotherapy in this subgroup of patients. In some situations, radiation therapy or chemotherapy may be used before surgery to shrink the tumor and increase the possibility of radical breast-conserving resection. Radiation therapy may be continued after the operation. For tumors more than 5 cm after radical surgery, radiation therapy is performed.

Simultaneous chemotherapy with doxorubicin and external radiation therapy as a neoadjuvant method with subsequent surgery and continuation of radiation is being studied. The results of neoadjuvant chemotherapy in combination with regional hyperthermia in patients with retroperitoneal and visceral sarcomas showed an improvement in disease-free and overall survival rates for patients who responded to treatment. In patients with stage IV of the process, the surgical method can also be used in the case of resectable lung metastases. In some patients, surgical removal of metastases provides long-term relapse-free survival and even cure. This is most often the case with isolated pulmonary metastases.

In one study, out of 719 patients with metastases of soft tissue sarcomas to the lungs, 213 (30%) were potentially resectable; in 161 (22%), radical resection of lung metastases could be performed.

The following provisions are taken into account to assess the feasibility of resection in metastatic disease:

1. There are no extrathoracic manifestations, pleural effusion and metastases to the lymph nodes of the root and mediastinum.

2. The primary tumor is healed or can be healed.

3. There are no contraindications for thoracotomy and resection of metastases.

4. Radical resection appears to be possible. Many centers use thoracotomy with resection of metastases, others use video thoracoscopy (VATS).

In the published results of resection of metastases of soft tissue sarcomas (SMT) in the lungs, the 3-year survival rate was 46-54%, and the 5-year survival rate was 37-40%. Prognostic factors are a long relapse-free interval (> 2.5 years), a microscopic absence of tumor cells at the resection margins and a histologically low grade of malignancy of the primary tumor (1st and 2nd), as well as size (<2 см) и количество метастазов. При благоприятных факторах прогноза 5-летняя выживаемость составила 60%. Описаны повторные метастазэктомии при рецидиве метастазирования в легкие .

The question of the advisability of pre- or postoperative chemotherapy remains open. Perhaps preoperative chemotherapy is preferable to determine its effectiveness in a particular patient and develop more rational further tactics. Liver metastases can also be resected, but the world experience is small, although there is an impression of an increase in survival in case of radicalism of such interventions. Liver resections and high-frequency ablation have been described. Chemotherapy for soft tissue sarcomas in recent years has begun to undergo significant changes: approaches to the choice of drug combinations depending on the structure of sarcomas are changing, new drugs are emerging, and targeted therapy is gaining clinical prospects. For patients with a widespread process, systemic therapy is still palliative, but it can prolong survival, improve overall health and quality of life. The choice of therapy should be individualized and based on a number of factors, primarily the morphological structure of the tumor and its biological characteristics, as well as the condition and preferences of the patient. The issues of treatment of gastrointestinal tumors (GIST) and rhabdomyosarcomas, which more often affect children, are considered separately. Currently, different morphological types of soft tissue sarcomas require different therapy.

Linovial sarcoma and myxoid liposarcoma are the most sensitive to chemotherapy: myxoid liposarcoma to doxorubicin-containing regimens, synovial sarcoma to alkylating agents such as ifosfamide.

Leiomyosarcomas of the uterus, endometrial stromal sarcomas, myxofibrosarcomas, de-differentiated liposarcomas, malignant tumors of the peripheral nerve sheath have individual variability in sensitivity to chemotherapy. Objective effects in these tumors are also possible with the use of anthracycline / ifosfamide and gemcitabine / docetaxel-containing regimens. The activity of gemcitabine with docetaxel was initially noted in leiomyosarcomas of the uterus and gastrointestinal tract. It was 53%. In the future, the effectiveness of the combination in one study - 43%, in the other - 18% was obtained in retroperitoneal leiomyosarcomas, leiomyosarcomas of the extremities, osteosarcomas, malignant fibrous histiocytomas, Ewing's sarcoma, malignant tumors of the peripheral nerve sheath. Comparison of the activity of the combination of gemcitabine with docetaxel and monochemotherapy with gemcitabine showed the advantage of the combination.

Dixoid liposarcomas have been found to be particularly sensitive to the new drug trabectidine (yondelis), a unique agent from a marine product that acts on nuclear DNA, damaging repair mechanisms.

Angiosarcomas, especially in the head region, are sensitive to taxanes. Liposomal doxorubicin (doxil in the US, kelix in Europe) is also effective for angiosarcomas.

For other types of sarcomas that are insensitive to common cytostatic agents, the study of the molecular mechanisms of pathogenesis may open up new therapeutic strategies, as, for example, demonstrated for advanced gastrointestinal tumors (GIST) using imatinib mesylate (glivec) and sunitinib (sutent ). There is also evidence of the effectiveness of imatinib mesylate (glivec) in patients with dermatofibroma prominence and desmoid tumors. There have been reports of the activity of sorafenib (nexavar), a multitarget tyrosine kinase inhibitor that blocks RAF kinase and blocks the intracellular portion of VEGF receptors, in angiosarcomas and some subtypes of leiomyosarcomas. Stabilization of the process with prolonged survival may be the main goal when using targeted drugs. The first drug active in soft tissue sarcomas in the 1970s was doxorubicin, the effectiveness of which depends on the dose (> 60 mg / m2 or 70 mg / m2) and ranges from 10 to 25%. The less cardiotoxic anthracyclines epidoxorubicin (pharmacorubicin) and liposomal doxorubicin (doxil, kelix) were found to be equally effective to doxorubicin in a few comparative randomized trials. Liposomal doxorubicin is active in angiosarcomas.

The alkylating drug ifosfamide is the second effective drug for soft tissue sarcomas, causing 7-41% of objective effects in patients previously treated with doxorubicin. Doses and regimens of ifosfamide vary widely, but it is desirable to keep in mind the need for a dose sufficient for the effect of 6 g / m2. Some authors note that its efficacy is dose dependent and it is necessary to use> 10 g / m2. Direct comparison of one doxorubicin - 75 mg / m2 for 3 weeks and two regimens of Ifosfamide - 3 g / m2 4 hours daily for 3 days or 9 g / m2 as a 72-hour infusion in patients with metastases of soft tissue sarcomas showed the same results in effectiveness, but greater toxicity of ifosfamide regimens. It should be remembered that ifosfamide is always used with a uroprotective agent.

Docetaxel (Taxotere) is relatively inactive for soft tissue sarcomas, with the exception of angiosarcoma. Paclitaxel is also used to treat angiosarcomas, especially of the scalp. There is a report that the weekly regimen is more effective.

Other drugs with> 20% efficacy in soft tissue sarcomas are vinorelbine, standard doses of methotrexate, temozolomide (especially in leiomyosarcomas), cisplatin, carboplatin, trabectidine. The efficacy of gemcitabine was also noted in one of the studies for leiomyosarcomas of non-gastrointestinal origin in 4 out of 10 patients. In other studies, the effectiveness is less. The efficacy of the combination of gemcitabine (fixed infusion rate) and docetaxel or vinorelbine has been emphasized by many authors both for uterine and gastrointestinal leiomyosarcomas and for the treatment of other types of soft tissue sarcomas. Topotecan also has activity in leiomyosarcomas (non-uterine origin).

Many drug combinations have been studied for soft tissue sarcomas:

oxorubicin + ifosfamide + mesna.

M AID (mesna, doxorubicin, ifosfamide, dakar-bazin).

emcitabine + docetaxel or vinorelbine.

Alternating courses of ifosfamide with etoposide and vincristine with doxorubicin and cyclophosphamide (VAC / IE).

phosphamide, etoposide and cisplatin.

CYVADIC (cyclophosphamide, vincristine, doxorubicin, dacarbazine).

AS (mitomycin, doxorubicin, cisplatin). oxorubicin + dacarbazine (AD). phosphamide + liposomal doxorubicin.

The effectiveness of these regimens is 16-46% with PR in 5-10% of patients and duration of relapse-free survival in 1/3 of patients with PR.

Comparison of combination regimens with doxorubicin monotherapy showed an increase in the frequency of objective effects when using the combinations without affecting survival. PR did not exceed 10%.

Long-term drug infusions are less toxic. In any case, treatment must be individualized. In cases where it is necessary to quickly affect the symptoms of the disease and cause tumor shrinkage, for example, in a locally advanced process (neoadjuvant chemotherapy) for the purpose of further surgery, combined regimens are preferable.

IV. New approaches

Trabectidin (EJ-743, ecteinascidin, yondelis), a new alkaloid from the marine product Esteinascidia turbinate, has been shown to be quite effective in soft tissue sarcomas, especially in myxoid liposarcomas and leiomyosarcomas. The mechanism of action is damage to nuclear DNA by disrupting the repair mechanism. In a phase II study, trabectidine was effective in 17% of patients, with stabilization in 24%. Median survival was 15.8 months, and 72% of patients were alive during the first year of follow-up. Side effects were: stage IV neutropenia. - 33%, the rise of transaminases III-IV stage. - 33%, III degree nausea. - 14%, fatigue III-IV Art. - eleven%. High efficacy of tarbectidine was observed in myxoid liposarcomas. It was 51% of complete and partial regressions. 88% of patients were followed up without progression for 6 months.

Sorafenib (Nexavar) is a multitarget tyrosine kinase inhibitor.

Phase II of the study demonstrated objective effects in leiomyosarcomas (5%) and angiosarcomas (15%). Moreover, 74% of patients with angiosarcomas and 54% of patients with leiomyosarcomas did not progress within 12 weeks.

Sunitinib (sutent) is a multitarget tyrosine kinase inhibitor. Some activity has been noted against such chemoresistant sarcomas as clear cell and alveolar soft tissue sarcoma.

Bevacizumab (Avastin) is a monoclonal antibody that inhibits vascular endothelial growth factor (VEGF). The possibility of objective effects and stabilization in patients with leiomyosarcomas using a combination of doxorubicin and bevacizumab was shown. Cardiotoxicity limits the use of this combination. A camptothecin derivative - oral drug Gimatecan (Gimatecan) caused stabilization of the disease according to phase II data in 35% of patients with Ewing's sarcoma, leuo- and liposarcoma.

IX. chemotherapy regimens

Monochemotherapy

oxorubicin - 30 mg IV 2 times a week for 3 weeks.

Doxorubicin - 30 mg / m2 IV from the 1st to the 3rd day.

oxorubicin - 60-75 mg / m2 IV 1 time in 3 weeks.

pyrubicin (pharmacorubicin) - 100 mg / m2 IV once every 3 weeks.

phosphamide - 5 g / m2 intravenously or intravenously infusion on the 1st day or 1.6-2.5 g / m2 / day for 5 days with uroprotective agent (uromitexan) at the rate of 120% of the dose of ifosfamide at the same time with him.

emcitabine 1200 mg / m2 over 120 min Days 1 and 8 every 21 days at a fixed infusion rate of 10 mg / m2 / min.

inorelbin - 25-30 mg / m2 IV 1 time per week for 8-10 weeks. Polychemotherapy A1

oxorubicin - 75 mg / m2 as a 72-hour infusion.

ilgrastim - s / c 5-15 days or until the level of neutrophils is restored. Interval 3 weeks. GemTax

emcitabine - 900 mg / m2 as a 90-minute infusion on days 1 and 8 IV.

axoter - 100 mg / m2 on the 8th day. ilgrastim - s / c 5-15 days or until the level of neutrophils is restored.

For patients who have already received chemotherapy, the dose of gemcitabine is reduced to 675 mg / m2 on days 1 and 8 and taxotere to 75 mg / m2 is also performed against the background of CSF. Interval 3 weeks. MAID

Esna OD - 8000 mg / m2 in the form of a 96-hour infusion (2000 mg / m2 / day for 4 days).

oxorubicin - 60 mg / m2 as a 72-hour intravenous infusion.

phosphamide - 6000 mg / m2 as a 72-hour infusion or 2000 mg / m2 IV as a 4-hour infusion on days 1-3.

acarbazine - 900 mg / m2 as a 72-hour infusion, dissolved together with doxorubicin. Interval 3-4 weeks. ADIC

oxorubicin - 90 mg / m2 as a 96-hour IV infusion.

acarbazine - 900 mg / m2 in the form of a 96-hour infusion, dissolved together with doxorubicin. Interval 3-4 weeks. G / ADIC

iclophosphamide - 600 mg / m2 IV on the 1st day.

oxorubicin - 60 mg / m2 as a 96-hour intravenous infusion.

acarbazine - 1000 mg / m2 in the form of a 96-hour infusion, dissolved together with doxorubicin. Interval 3-4 weeks.

Chemotherapy regimens for VAI rhabdomyosarcoma

Inkristin OD - 2 mg on the 1st day. oxorubicin - 75 mg / m2 as a 72-hour infusion.

phosphamide - 2.5 g / m2 IV in the form of a 3-hour infusion on days 1-4.

esna - 500 mg / m2 on the 1st day together with ifosfamide, then 1500 mg / m2 as a 24-hour infusion for 4 days.

ilgrastim - s / c 5-15 days or until the level of neutrophils is restored. Interval 3 weeks VAC

incristine - 2 mg / m2 on the 1st and 8th days IV, interval 5 weeks.

actinomycin - 0.5 mg / m2 1-, 2-, 3-, 4-, 5th days (repeat every 3 months, up to 5 courses).

Ciclophosphamide - 300 mg / m2 daily for 7 days every 6 weeks. VAdriaC

incristine - 1.5 mg / m2 on the 1st, 8th, 15th days during the first 2 courses, then only on the 1st day.

oxorubicin - 60 mg / m2 as a 48-hour infusion.

iclophosphamide - 600 mg / m2 for 2 days. The interval is 3 weeks and beyond.

phosphamide - 1800 mg / m2 + mesna for 5 days.

topozide - 100 mg / m2 1-5th days. Interval 3 weeks

Literature

1. Frustaci, S. Adjuvant chemotherapy for adult soft tissue sarcomas of the extremities and girdles: results of the Italian randomized cooperative trial / S. Frustaci, F. Gherlinzoni, A. De Paoli // J. Clin. Oncol. - 2001. - Vol. 19 (5). - P. 1238-1247.

2. Gossot, D. Resection of pulmonary metastases from sarcoma: can some patients benefit from a less invasive approach? / D. Gossot, C. Radu, P. Girard // Ann. Thorac. Surg. - 2009. - Vol. 87 (1). - P. 238-243.

3. Pfannschmidt, J. Pulmonary metastasectomy for soft tissue sarcomas: is it justified? / J. Pfannschmidt, H. Hoffmann, T. Schneider, H. Dienemann // Recent. Results Cancer Res. - 2009. - Vol. 179. - P. 321-336.

4. Blackmon, S.H. Resection of pulmonary and extrapulmonary sarcomatous metastases is associated with long-term survival / S.H. Blackmon, N. Shah, J.A. Roth // Ann. Thorac. Surg. - 2009. - Vol. 88 (3). - P. 877-884.

5. Demetri, G.D. Efficacy and safety of trabectedin in patients with advanced or metastatic liposarcoma or leiomyosarcoma after failure of prior anthracyclines and ifosfamide: results of a randomized phase II study of two different schedules / G.D. Demetri, S.P. Chawla, M. von Mehren // J. Clin. Oncol. - 2009. - Vol. 27 (25). - P. 4188-4196.

6. Pacey, S. Efficacy and safety of sorafenib in a subset of patients with advanced soft tissue sarcoma from a Phase II randomized discontinuation trial / S. Pacey, M.J. Ratain, K.T. Flaherty // Invest. New Drugs. - 2009. - Dec. 18.

7. Stacchiotti, S. Sunitinib in advanced alveolar soft part sarcoma: evidence of a direct antitumor effect / S. Stacchiotti, T. Negri, N. Zaffaroni // Ann. Oncol. - 2011 .-- Jan 17.

8. Ganjoo, K.N. New developments in targeted therapy for soft tissue sarcoma. Curr / K.N. Ganjoo // Oncol. Rep. - 2010. - Vol. 12 (4). - P. 261-265.

- This is one of the varieties of malignant neoplasms emanating from the cellular elements of the connective tissue. Since there is not a single organ and anatomical segment in the human body that does not contain connective tissue, sarcoma does not have a strict localization. Any part of the human body is susceptible to such tumor transformation. In practice, this is associated with conflicting statistical data, according to which only 5% of all malignant neoplasms are sarcomas. But their feature is such that the occurrence of a similar one is associated with high mortality. Another feature of sarcomas is the predominant occurrence at a young age during the period of active growth of the body (the age of more than 35% of patients is less than 30 years).
General characteristics of sarcoma:

High grade of malignancy;

Invasive type of growth with germination of surrounding tissues;

Growing to large sizes;

Frequent and earlier metastasis to the lymph nodes and internal organs (liver, lungs);

Frequent relapses after tumor removal.

Each of the types of sarcomas has a favorite growth site, age range, connection with a specific sex and other factors. They differ from each other macroscopically and histologically, the degree of malignancy, different propensity for metastasis and recurrence, the depth of germination and prevalence. The vast majority of sarcomas grow in the form of nodes of different sizes and shapes, do not have clear boundaries and in the cut resemble fish meat of a pale gray shade with areas and a different number of vessels. Some sarcomas are characterized by rapid growth (weeks, months), but there are also tumors with a slow growth type (years, decades). Tumors of this type are always well supplied with blood.

The most common localizations of sarcoma

The main derivatives of connective tissue in the body are bones, blood vessels, muscles, ligaments, tendons, fascia, connective tissue membranes and capsules of internal organs and nerves, connective tissue constrictions of adipose tissue and cellular spaces.

Depending on this and localization, tumor growth is most susceptible to:

Limb bones;

Soft tissues of the extremities (together with bone sarcomas make up 60% of all sarcomas);

Soft tissues and bones of the trunk;

Soft tissues, tissue spaces and bones of the head and neck;

Connective tissue elements of the mammary glands and uterus;

Fiber of the retroperitoneal space;

Other rare localizations (internal organs, abdominal and pleural cavities, mediastinum, brain and peripheral nerves).

Histological classification and types of sarcoma

Among all malignant tumors, sarcoma has the largest variety of histological types. The category of sarcomas includes:

Sarcoma type

The structure and description of the tumor

Osteosarcoma

Formed from cellular components of bone tissue

Chondrosarcoma

Presented by cartilaginous tissue

Paraostal sarcoma

Formed from the periosteum and surrounding tissue

Reticulosarcoma

Tumor growth from bone marrow elements

Ewing's sarcoma

A type of osteosarcoma, affecting mainly the end sections of the long bones of the extremities

Fibrosarcoma

Tumor of connective tissue elements and fibrous fibers

Angiosarcoma

The basis of the tumor is the growth of vascular elements.

Stromal sarcomas of the gastrointestinal tract and other internal organs

Derived from the connective tissue that makes up the stroma of any organ

Liposarcoma

A tumor that grows from adipose tissue

Rhabdomyosarcoma

The predominance of elements of striated muscles

Kaposi's sarcoma

Multiple tumor growths of blood vessels of the skin and lymphoid tissue against the background of immunodeficiency

Lymphangiosarcoma

Tumor with proliferation of components of the lymphatic vessels

Dermatofibrosarcoma

Tumor from skin structures with a connective tissue base

Synovial sarocoma

Tumor growth of their synovial membranes of the joints

Lymphosarcoma

Tumor growth from lymphoid tissue

Neurofibrosarcoma

Grows from the sheaths of nerves

Fibrous histiocytoma

Contains different types of connective tissue cells and fibers

Spindle cell sarcoma

Affects mucous membranes and consists of large, spindle-shaped cells

Mesothelioma

The substrate of the tumor can be the mesothelium of the pericardium, peritoneum and pleura

Not always, even under a microscope, one can clearly distinguish between the structure of a sarcoma and its histological type. The most important thing that must be established is the very fact of the origin of the tumor from the connective tissue and the degree of its differentiation.

Depending on this, there are:

Poorly differentiated sarcomas. Tumors of this type have the lowest degree of malignancy, since in their structure they do not resemble the tissues from which they grow. They practically do not metastasize, grow slowly, are large, removal rarely causes relapses;

Highly differentiated sarcomas. They are the absolute opposite of poorly differentiated. In structure, they are similar to those tissues from which they originate, they are highly malignant, grow rapidly, metastasize early, and do not respond well to surgical treatment;

Moderately differentiated sarcomas. They occupy an intermediate position between the previous types.

All malignant tumors of the human body are globally divided into epithelial -, glandular - adenocarcinomas, and connective tissue - sarcomas. The latter type of tumors occurs less often than others, but is characterized by the greatest variety of histological types and the possibility of affecting any organs, tissues and anatomical segments!

Sarcoma symptoms

The clinical picture of a sarcoma depends on the location and characteristics of its malignancy. The main symptoms of the disease are shown in the table.

Symptom group

Manifestations

Pain syndrome

Intense or moderate pain at the site of tumor growth. More common in highly malignant sarcomas;

Discomfort, distension and foreign body sensation in the affected area. Characterizes slowly growing sarcomas with a low degree of differentiation;

The appearance of a tumor

Visual identification of a tumor on the surface of the skin;

Palpation determination of a tumor-like formation located at different depths from the skin surface;

Deformation and swelling of the affected limb;

Wound surface at the site of tumor growth, due to its decay;

Disintegrating tumors are always accompanied by copious fetid discharge from the surface of the disintegration.

Dysfunction of the affected organ or segment

Inability to perform movements or walking with tumors of soft tissues or bones of the extremities;

With the growth of tumors from internal organs, their size increases with dysfunction and organ failure.

Germination of surrounding tissue

With germination or compression of blood vessels - impaired blood circulation with gangrene of the limb or profuse bleeding;

With germination or compression of nerves - severe pain and weakness of the limb;

With the germination of the retroperitoneal space - a violation of the outflow of urine and hydronephrosis;

With compression of the mediastinal and neck organs - disorders of swallowing and breathing;

Enlargement of lymph nodes near the tumor focus.

The presence of any symptoms of sarcoma is a direct indication for its confirmation or exclusion as soon as possible.

The following diagnostic methods can help with this:

X-ray examination if osteosarcoma and other bone tumors are suspected;

Ultrasound procedure soft tissues or internal organs;

Tomography. For bone tumors, it is more advisable to perform computed tomography. Soft tissue tumors are better seen on MRI;

Radioisotope diagnostic methods. Their diagnostic value increases with deep localization of tumors in cavities and cellular spaces;

Tumor biopsy. With superficially located tumors, it is not difficult. Deeply located tumors can only be examined under ultrasound or tomographic guidance;

Angiography. The contrast agent injected into the arteries determines the local accumulation of blood vessels at the site of tumor growth and the nature of the circulatory disorders below the site of sarcoma growth.

Sarcoma causes

Any types of sarcomas, like all malignant neoplasms, belong to polyetiologic diseases that arise under the influence of many causal factors. It is possible to determine them in rare cases.

The main culprits of tumor transformation of connective tissue can be:

Complicated hereditary history and genetic predisposition;

The damaging effect of ionizing radiation on the DNA of cells;

The impact of oncogenic viruses on cells that trigger the mechanisms of uncontrolled division;

Violation of lymph outflow after operations and pathological processes;

Congenital and acquired immunodeficiencies;

Courses of chemotherapy and treatment with immunosuppressive drugs;

Internal organ transplant;

Traumatic injuries, extensive and long-term non-healing, unrecovered foreign bodies of soft tissues.

The realization of the oncogenic action of causal factors in the development of sarcomas most often occurs in a growing organism. This is because it is much easier to cause a breakdown in those cells that are actively dividing. The pattern is that the deeper the DNA damage, the more malignant the sarcoma will be!

The separation of sarcomas at the stage is based on:

The size of the primary tumor;

Spread outside the capsule of the organ or fascia of the anatomical formation from which the sarcoma grows (muscles, bones, tendons, etc.);

Involvement in the process and germination of surrounding tissues;

Presence of metastases to regional lymph nodes;

The presence of metastasis to distant organs.

The histological type of tumor does not affect the staging of sarcomas, in contrast to the primary site of tumor localization in the body. It is the organ in which the sarcoma began its growth that most of all affects the determination of the stage of the process.

Stage 1 sarcoma

Such sarcomas are small in size, do not go beyond the limits of the organ or segment from which they began to grow, do not disrupt its function, do not squeeze vital anatomical structures, are practically painless, do not metastasize. Revealing even a highly differentiated sarcoma at the first stage allows to achieve good treatment results.

Signs of the first stage of sarcoma, depending on the specific location, are as follows:

Sarcoma of the oral cavity and tongue - a tumor of about 1 centimeter comes from the mucous membrane or submucosa in the form of a small node with clear boundaries;

Sarcoma of the lip - located within the submucosal layer, mucous membrane or in the thickness of the lip;

Sarcoma of the cellular spaces and soft tissues of the neck - can be up to 2 cm in size and does not go beyond the fascia that limit the area of its location;

Laryngeal sarcoma - a node up to 1 cm bounded by the mucous membrane, or other layers of the larynx, without going beyond its fascial sheath, does not cause pronounced disturbances in phonation and respiration;

Thyroid sarcoma is a tumor up to 1 centimeter with an intraorgan location in the thickness of the tissues. The capsule does not germinate;

Breast sarcoma - defined as a node up to 2-3 cm, located within the lobule, from which its growth began;

Esophageal sarcoma - tumor size up to 1-2 cm, located in the thickness of the organ wall. The passage of food through the esophagus is not disturbed;

Lung sarcoma - affects one of the segmental bronchi. Does not go beyond the segment and does not violate the functions of the lung;

Testicular sarcoma - looks like a small node and does not involve the tunica albuginea;

Sarcoma of soft tissues of the extremities - the node can reach 5 cm, but does not go beyond the fascial sheaths.

General characteristics of sarcomas of the second stage: intraorgan location with the growth of all layers, an increase in tumor size, impaired organ function, absence of metastases.

With the defeat of specific organs, it looks like this:

Sarcoma of the oral cavity and tongue - the tumor is clearly visible upon visual examination, located in the thickness of the anatomical formations, but all its layers, including the mucous membrane and facies, grow;

Sarcoma of the lip - the node is located in the thickness of the lip, but the skin and mucous membranes grow;

Sarcoma of the cellular spaces and soft tissues of the neck - the tumor reaches 3-5 cm and extends beyond the fascia, limiting the space of its growth;

Laryngeal sarcoma - a node of more than 1 cm with spread through all layers of the organ, impaired phonation and respiration;

Thyroid sarcoma - the size of the node is about 2 cm, the capsule of the organ is involved in the pathological process;

Breast sarcoma - the size of the tumor is about 5 cm, several segments grow;

Esophageal sarcoma - the tumor invades the entire thickness of the esophageal wall from the mucous to the serous layer with the involvement of the fascia. Severe dysphagia;

Lung sarcoma - the tumor causes compression of the bronchi or spreads to adjacent segments of the lung;

Testicular sarcoma - tumor germination of the tunica albuginea;

Soft tissue sarcoma of the extremities is the invasion of fascial formations by a tumor that limits the anatomical segment (muscle, cellular tissue).

The principle of isolation of the second stage of sarcoma is that such tumors are located within the organ, but require extended tissue excision when they are removed. The results are worse than in the first stage of the process, but relapses do not occur often.

The third stage of sarcoma involves the growth of the tumor of fascia and organs located in the immediate vicinity of the tumor, or the presence of metastasis to the regional, in relation to it, lymph nodes.

For specific organs, it looks like this:

Sarcoma of the oral cavity and tongue is a large primary tumor, pain syndrome is expressed, normal anatomical relationships and chewing are disturbed. There are metastases to the submandibular and cervical lymph nodes;

Sarcoma of the lip is a large tumor that sharply deforms the lip with possible spread to the surrounding areas of the mucous membrane. Metastases in the submandibular or lymph nodes of the neck;

Sarcoma of the cellular spaces and soft tissues of the neck - pronounced signs of dysfunction of the neck organs (swallowing, breathing, disorders of innervation and blood supply). The tumor grows to a large size and grows vessels, nerves, adjacent organs of the neck. There are metastases in the superficial and deep cervical and thoracic lymph nodes;

Laryngeal sarcoma - abruptly disrupts breathing and voice. Vessels, nerves, neighboring fascia grow. There are metastases in the superficial and deep lymphatic collectors of the neck;

Thyroid sarcoma - invades tissues adjacent to the thyroid gland. There are metastases in the cervical lymph nodes;

Breast sarcoma - a large tumor with a sharp deformation of the mammary gland and metastases in the axillary or supraclavicular lymph nodes;

Esophageal sarcoma - a large tumor, spreads to the tissue of the mediastinum, sharply disrupts the passage of food. Metastasizes to the lymph nodes of the mediastinum;

Lung sarcoma - reaches large sizes, causes compression of the bronchi, metastases to the peribronchial and mediastinal lymph nodes;

Testicular sarcoma is large, deforms the scrotum and its layers grows. There are metastases to the inguinal lymph nodes;

Soft tissue sarcoma of the extremities is a tumor focus of about 10 cm, disrupts the function of the extremity, sharply deforms it. There are metastases in regional lymph nodes.

Stage III sarcoma is characterized by disappointing results of treatment, requires extensive surgical interventions and often recurs.

Sarcoma stage 4

The most unfavorable prognosis is the detection of sarcoma at stage 4 of the tumor process. The danger of such a situation is that such tumors are gigantic in size, sharply squeeze the surrounding tissues or grow in them, forming a continuous tumor conglomerate, often accompanied by decay and bleeding. There are always metastases to regional and lymph nodes of any localization. The presence of distant metastases in the liver, lungs, brain and bones is characteristic. There is no need to dwell in detail on the description of stage 4 of individual localizations of sarcomas, since they are similar to the third stage. It is distinguished only by the aggravation of local manifestations and the destructive effects of the tumor, as well as the presence of distant metastases.