Heart tumors in children. Myxoma - benign cardiac tumor with dangerous consequences Cardiac tumor

A rare disease that is often diagnosed after the death of a patient. It must be remembered that a healthy lifestyle, a positive attitude will help to strengthen the body's immunity - the main internal doctor, who does not allow failures leading to the formation of heart cancer.

Concept and statistics

Cancer of the heart can mean a tumor formation inside the chambers, it can also be an organ muscle damage.

Myocardial oncology can pass unnoticed for a long time, disguising itself as others.

The symptoms of the disease begin to cause concern to the patient when metastases are detected.

Pathology is rare. This is due to the activity, which is due to the functional purpose of the chambers and other structural components of the heart. Blood circulation and tissue metabolism are usually high.

Types of cardiac tumors

Pathology has different manifestations, locations, and is based on different tissues.

  • Primary tumors- pathology formed in the heart; has a variety of forms, depending on which tissue cells got sick with atypism and gave a start to the oncological process.
  • Secondary tumors- cancer damage to neighboring organs or those that are located more distant from the heart, has delegated its presence to its area.

Primary formations account for a quarter of all cancers of the heart. They come in different forms:

  • sarcoma - a common type of tumor
  • - in the region of the heart is rare.

Sarcoma is more common in middle-aged people. The right sections are subject to tumor processes to a greater extent than the left side.

Sarcoma is dangerous with the rapid growth of the tumor body. Abnormal cells are capable of invading heart tissue and infecting adjacent organs. Valves, vessels that are found on the path of pathology are damaged to varying degrees by a growing tumor.

Sarcomas also have several subspecies:

  1. Liposarcoma - occurs in adulthood and is rare. The body of the tumor is made up of lipoblasts. Liposarcoma is located in the heart cavity and has an external similarity to myxoma. The formation is capable of creating a massive body, the color of which is usually yellowish. The tumor has a soft consistency. This type of pathology is responsive to medical procedures.
  2. - originates in muscle tissue. The tumor is a white mass, soft to the touch. If we look at the node under a microscope, then cells of several types of forms are found in its composition:
    • fusiform,
    • round,
    • oval
    • other.

    This type of pathology is rare. In the total number of primary tumors, rhabdomyosarcoma occurs in every fifth patient. Men have this type of heart tumor more often than women.

  3. - makes up a tenth of primary tumors. It is a formation with clear borders of a grayish-white color. The node has collagen fibers and fibroblast-like cells with varying degrees of differentiation.
  4. Angiosarcoma - according to statistics, this type occupies a third of all primary cardiac tumors. It affects more often men. Education has a lumpy structure of a dense constitution. This type of tumor is characterized by the presence of vascular cavities in the body of the formation, which have different shapes and sizes.

Heart Cancer Photos

Tumors in the region of the heart of secondary origin are much more common. They can appear as a result of oncological processes in the following organs:

  • stomach,
  • mammary gland,
  • kidneys,
  • thyroid gland,
  • lungs.

The spread of cancer cells occurs through the lymph, as well as through the circulatory system. Cancer tissues enter the heart, growing into the organ.

Causes of occurrence

To date, science does not know the exact reasons for the appearance of cancer in the region of the heart.

Primary formations can be triggered by the following phenomena:

  • the degeneration of myxoma (benign tumor), which, in turn, can occur after surgery on the organ;
  • as a result of toxic effects,
  • be a consequence of an infectious disease,
  • due to harmful influences caused by and.

Secondary oncological tumors arise as a consequence of the spread of oncology, which has developed in other organs, beyond their limits. Metastases can grow into the region of the heart from nearby organs and those that are more distant.

Heart cancer symptoms

Possible cancer of the heart is indicated by the following signs:

  • the appearance of pain in the chest,
  • dyspnea,
  • symptoms of oppression of the vena cava,
  • enlarged heart chambers,
  • high temperature
  • violation of rhythms in the work of the heart,
  • fast fatiguability,
  • hemorrhagic effusion is found in the pericardium,
  • swelling of the muscles of the face,
  • disturbances in the work of the conducting system,
  • tamponade,
  • significant weight loss,
  • sudden death.

Stages of development

The prognosis and treatment tactics depend on the extent to which a cancerous tumor has developed.

There are four stages:

  • The appearance of altered cells, which were the result of DNA damage to cells and their subsequent chaotic division. Such a violation is referred to as the first stage.
  • The formation of oncological formations in the place of appearance of atypical cells is the second stage of the disease.
  • The spread of the disease to other organs with lymph flow or through the blood. Germination of a cancerous tumor outside the heart - metastases are referred to as the third stage of the disease.
  • The primary focus is in a state of exacerbation. At the same time, the emergence of new pathological formations in other places is observed. The development of the oncological process is to such an extent defined as the fourth stage of the disease.

Diagnostics

Cancer of the heart is difficult to identify. This is because its manifestations are similar to other heart diseases. Therefore, several methods are used to diagnose the problem.

  • ECG - an informative check that shows whether there are irregularities in the rhythms of the heartbeat. You can also get information about the state of the conduction function.
  • MRI - will show the state of the chambers of the heart and surrounding tissues and organs. CT will also add detailed information, including abnormalities in hard tissues. These methods are necessary if a controversial issue has occurred.
  • EchoCG - one of the main methods for clarification:
    • the location of the cancer,
    • determining the size of the tumor,
    • clarification of the issue of the presence of fluid in the pericardial zone.
  • Laboratory research:
    • to clarify the diagnosis, a biopsy study is performed,
    • do blood sampling for clinical analysis and biochemical studies,
    • tumor markers.

Heart cancer treatment

In the early stages, heart cancer is most often not detected. By the beginning of the treatment process, the tumor may have many metastases to other organs. Therefore, surgery is not done in most cases.

The main methods of treatment:

  • chemotherapy,
  • irradiation,
  • supportive therapy.

The procedures allow:

  • slow down the development of pathology,
  • reduce metastasis,
  • improve the patient's quality of life.

How long do they live with him?

If heart cancer is detected before metastases appear, it is possible to extend the patient's life up to five full years. In advanced cases, the patient, from the moment the pathology is diagnosed, dies throughout the year, despite the ongoing treatment.

If the tumor is removed by surgery, a new cancerous tumor may form within two years.

Oncological formations very rarely affect the heart. This is due to the muscular nature of the organ and the protection of the pericardium - the pericardial sac filled with fluid.

Cardiac formations can be represented by both benign and malignant processes. Tumor of the heart it is also primary (first arising in the tissues of the heart) and secondary (penetrated from other organs due to the circulation of cancer cells).

Primary lesions are rare. Most of them are not aggressive. But even cellular benignity due to location cannot speak of safety for human life and health.

Reasons for education

A small percentage of people with heart lumps have a family history. Sometimes heart tumors develop in the presence of health problems such as Lamb or Carney syndrome.

In some cases, cardiac tumors develop as a result of pulmonary or peripheral artery embolism. The reason may also lie in the dominance of conditions:

  • the effects of certain industrial chemicals such as cadmium, nickel, benzene and vinyl chloride;
  • excessive smoking;
  • bacteria or viral infections.

But most often such education develops without any conditions.

Benign heart tumor: types and description

  1. Mixoma- the most common heart formation, which accounts for 24-37% of cases. Such formations in 75-80% are located in the left atrium. In 10-20% they are located in the right part of the atrium and in 5-10% - in both at the same time or in the ventricle.
  2. Rhabdomyoma- prevails in childhood. In 60-80%, it disappears on its own.
  3. Papillary fibroelastoma- a small lump on the pedicle located on the mitral or aortic valve.
  4. Lipomatosis- benign growth of the interatrial septum due to fatty deposits.
  5. Teratoma Is a tumor in the region of the heart, namely in the pericardial cavity. Paragangliomas have the same localization. sometimes also intracardiac. Although generally considered benign, relapses after treatment and malignancy are possible.

Rare types of noncancerous heart lesions are:

  1. : appear in infancy. Can lead to obstruction of blood flow, valvular dysfunction, arrhythmias.
  2. Hemangiomas: prone to spontaneous regression, but sometimes have a poor prognosis due to the occurrence of heart failure, hemorrhage from damaged vessels, thrombocytopenia, ventricular tachycardia.
  3. : often located in the left ventricle or right atrium.

Malignant tumors of the heart: types and description

Only 25% of primary heart lesions are cancerous. Of these, 75% are. The following are distinguished by the prevalence:

  1. Angiosarcomas(33%) - come from the vascular endothelium. They mainly consist of the proliferation of malignant cells that form the vascular channels.
  2. Rhabdomyosarcoma(20%) - neoplasms of striated muscles. occurs at any age and can affect the chambers of the heart. The process expands from the myocardium to the pericardium.
  3. Mesothelioma(15%) - an oncotype that originates from the mesothelium - a thin wall surrounding an organ and internal structures. the pericardium occurs in the heart lining.
  4. Fibrosarcomas(10%) - soft polypoid “gelatinous” tumors that fill the atrium and penetrate the ventricles or pericardium.

Symptoms

Signs depend on the size, quality of the neoplastic cells, histological characteristics and include:

  • deterioration of intracardiac blood flow, resulting in fainting, shortness of breath, prolonged heart failure;
  • atrial fibrillation;
  • arterial hypotension, fatigue;
  • tiny particles of lumps can separate and travel through the bloodstream to other organs (embolism), thus blocking their function. The condition affects the brain, lungs, etc .;
  • embolization leads to stroke and seizures;
  • subsiding of heart sounds;
  • hemorrhagic effusion;
  • constant pain in the region of the heart.

Heart tumor - photo:

Diagnostics

By mimicking other heart diseases with similar symptoms, cardiac tumors pose a serious problem in diagnosis. The main methods represent:

Echocardiogram

The most effective diagnostic tool that allows you to see the exact size and location of the lesion. This is a non-invasive procedure that uses sound waves. They create images on a monitor using an ultrasonic sensor.

Electrocardiogram

A test that records cardiac electrical activity, depicting abnormal rhythms and detecting damage to the heart muscle. However, the ECG can indicate other problems as well.

Computed and magnetic resonance imaging

Diagnostic imaging procedures that are used to depict the condition of an organ and find out the main characteristics of the formation.

Cardiac catheterization

By means of X-rays, a contrast agent is injected into the artery, which fills narrowings, occlusions and other anomalies.

Treatment and removal

Therapeutic measures, first of all, foresee surgical methods for treating cardiac tumors:

Simple resection

Usually used for benign tumors. For safety, a heart-lung machine is connected. During the procedure, both atria are opened from the right superior pulmonary vein without damaging the formation, which is removed completely. It also checks all chambers of the heart to rule out the presence of additional tumors.

Complex resection

It foresees the removal of a heart tumor that has not penetrated into adjacent tissues. It is mainly used for right-sided formation, which is usually removed entirely from the right half of the organ.

Excision in place

It is performed if the tumor occupies the posterior wall of the left atrium or large dorsal vessels. For ease of operation and optimal visualization of all structures, the heart is sometimes completely removed from the chest.

Artificial heart implantation

Possible only in young patients in the absence of metastases. Complications: thrombosis, infection, bleeding.

Heart transplant

An extreme treatment option when there are no distant metastases.

Forecast

For cancers of the heart, the prognostic data are not encouraging. In total, the 5-year survival rate is:

  • 83% for non-cancerous formations;
  • 30% for cancerous tumors of all types. For sarcomas, the prognosis is worse - 17% with an average life expectancy of 9 months;
  • 26% for metastatic patients.

Heart tumor: how long do they live?

Survival, from the time of diagnosis, ranges from 7 months to a maximum of 2 years. The cause of death in most cases is distant metastases.

Simple resection lengthens life by only a few months.

Organ transplantation with pre- or postoperative therapy can increase the patient's life expectancy up to 18 months and even up to 37 months.

The average survival rate for patients who have conservatively received chemotherapy or radiation therapy is 1 year.

Tumor of the heart- a complex disease that is difficult to identify and adequate treatment, and therefore has a poor outcome and survival results.

Heart tumors is a concept that includes a number of pathological formations in a given organ. They are different in structure and origin.

Neoplasms are formed from the tissues and membranes of the organ and affect a person regardless of age and gender. This pathology is also diagnosed in utero in the fetus at the sixteenth week.

Heart tumors are:

  • primary;
  • secondary (metastatic).

Primary ones occur no more often than 0.2%, but tumors that have grown as a result of the spread of metastases affect the heart thirty times more actively.

Such heart diseases pose a serious threat to human life. They can trigger a number of health problems.

Classification of pathological seals in the heart

As already indicated, tumors in the heart are divided into primary and secondary. It is rather difficult to name the true primary source provoking the appearance of primary tumors, but the appearance of metastatic neoplasms is caused by the progressive stages of cancer of other organs.

By nature, benign and malignant heart tumors are distinguished. The percentage of diagnosing these pathologies is 75 and 25, respectively.

Cancer tumors are also divided into:

Medical classification distinguishes between tumors and their origin.

Benign heart tumors are of the following types:

  • myxoma of the heart (diagnosed in 8 out of 10 cases);
  • teratoma;
  • rhabdomyoma;
  • fibroma;
  • hemangioma;
  • paranglioma and others.

Malignant neoplasm of the heart is represented by sarcomas, pericardial masothelioma, lymphomas.

Pseudotumors in the heart are also distinguished, which are thrombus formation with inflammatory processes.

Also diagnosed are extracardiac tumors of the mediastinum and pericardium, which compress the heart and disrupt the normal functioning of its left and right ventricles.

Primary tumors of the heart of a benign nature have the following etymology. Among all the variety of tumors, myxoma is predominantly found. This type of tumor primarily affects the female body. Myxoma is localized mainly in the left atrium. They have a varied morphological structure. Loose tumors are the most dangerous.

After myxoma, papillary fibroelastoma is the second most frequently diagnosed. It grows in the aortic and mitral valves. In structure, they resemble avascular papillomas with branches and a leg.

Rhabdomyomas, benign tumors that affect a child's body. The heart organ is permeated with multiple formations - rhabdomyomas, which damage the septum or wall of the left ventricle and disrupt the work of the cardiac conduction system. Provokes tachycardia, arrhythmia, heart failure.

Also, fibroids are also referred to as children's tumors. They predominantly grow into valves and the conductive system. Fibroids provoke mechanical obstruction, which leads to false valve stenosis and heart failure.

Less common are hemangiomas and pheochromocytomas. Pathological formations of the heart of this type are found in ten cases out of a hundred. Symptoms when they appear are absent, therefore, they are most often diagnosed during professional examinations. In rare cases, with the growth of hemangiomas can lead to atrioventricular conductivity. If the tumor has grown into the atrium of the ventricle, it can be instantly fatal.

Pheochromocytomas are benign neoplasms of the heart, which are intrapericardial and myocardial localization. The symptoms of the disease are almost not pronounced, but under certain conditions they contribute to the compression of the chest organs.

The malignant neoplasms of the heart include sarcomas and lymphomas, which have their own subspecies.

The most common are sarcomas. They affect most people at a young age. Sarcomas are localized in the left atrium and cause a number of complications.

Lymphomas are primary tumors, this type is especially rapidly progressing.

Malignant neoplasms of the heart have serious consequences.

Symptoms of heart tumors

Neoplasms in the heart organ, depending on their type, nature, structure and stage of development, cause certain symptoms in the body.

Localization of the tumor outside the heart provokes the occurrence of:

  • increased body temperature;
  • chills;
  • weight loss;
  • arthralgia;
  • rash.

When the neoplasm compresses the heart chamber and coronary arteries, it appears:

  • breathing problems;
  • pain in the chest;
  • bleeding;

These symptoms lead to cardiac tamponade.

Neoplasms that are in the conducting system lead to the appearance of paroxysmal tachycardia and blockages.

Tumors located inside the heart disrupt the normal functioning of the valves and prevent blood from flowing out of the heart chambers. With this pathology, symptoms of heart failure are observed.

As the first bells about the presence of pathological seals in the heart, regardless of its nature, it is customary to consider:

  • vascular thromboembolism;
  • pulmonary embolism;
  • cerebral echemia;
  • stroke;
  • myocardial infarction.

Diagnostics of the heart for the presence of neoplasms (ultrasound, mri, MCT and others)

A heart exam is not an easy task. In view of the huge number of varieties of tumor inclusions, as well as the places of their localization, in order to establish an accurate diagnosis and obtain an extensive picture of the disease, an extensive diagnosis of the organ is required using various techniques.

If a tumor is suspected, the first step is to do an ultrasound of the heart. This procedure is most likely to identify the seal. There are two types of ultrasound diagnostics: transesophageal ultrasound - for examining the atrium and transtoral ultrasound - studying the left and right ventricles.

If the ultrasound data turns out to be incomplete, then an examination by MRI and MSCT methods is prescribed, plus radioisotope scanning and heart sounding are performed. Ventriculography is also sometimes used.

To study the nature of the tumor, even if the ultrasound has accurately determined its presence and origin, a biopsy is performed. For this, catheterization or trial thoracotomy is performed. Also, for histology, fluid from the pericardium obtained as a result of puncture can be used.

The reasons why tumors form in the heart are not fully understood, so today we cannot talk about them. But some patterns are still studied and it is possible to identify those factors that can cause pathological changes in cells at the gene level, which lead to their disorderly division and the appearance of tumors. Among them:

  • large body weight;
  • adverse effects of harmful substances;
  • wrong lifestyle;
  • bad habits (smoking, alcoholism);
  • infectious diseases;
  • cancer metastases from other primary sites in other organs.

Treatment of neoplasms of the heart

Only after a complete examination and establishment of an accurate diagnosis, the treatment is determined that will have the maximum effect and the minimum risk of complications. It must be said that malignant tumors have a completely different tactics and methods than a malignant tumor. Treatment is based on:

  • surgical intervention (removal of the primary tumor);
  • radiation therapy and its latest techniques: brachytherapy and gamma knife;
  • chemotherapy.

Let us also indicate that if a tumor in the heart contains cancer cells, then surgery alone will not be enough. Oncology needs complex treatment. Let us consider more broadly the issues of oncological pathologies in the heart.

Heart cancer is a rare disease. For the most part, it does not damage the heart muscle, since it is actively supplied with blood. It is too difficult to define cancer because it has been taking a long time without any particular symptoms. Therefore, most of it is detected at a later date or after a fatal outcome.

Cancer is primary and secondary. A malignant primary tumor (sarcoma, angiosarcoma, rhabdomyosarcoma, fibrosarcoma, and others) quickly develops and migrates to the lymph nodes, lungs, and brain.

Secondary tumors of the heart arise as a result of metastasis of malignant formations of other organs. This type of oncology is much more common than primary neoplasms. In ten out of a hundred cancer patients, metastases are found in the heart. The first localization of a metastasized tumor occurs in the pericardium of the heart.

Cancer symptoms appear depending on its location and stage of development. If it does not penetrate into the myocardium, then the pathogenic neoplasm remains undetected for a long time and continues to increase in size. At later stages, an increase in temperature is noted, joints begin to hurt, weakness, numbness of the limbs, a rash throughout the body, weight loss appear. Laboratory studies indicate pathological changes in the composition of the blood.

A malignant tumor, not only in the myocardium, but also outside it, is growing rapidly. It causes arrhythmia, disrupts conduction, painful sensations in the chest are felt almost constantly, the heart enlarges. Acute heart failure can lead to sudden death.

The appearance of the following symptoms should alert and serve as a call to undergo a full examination:

  • pain in the chest area;
  • swelling of the face and hands;
  • dyspnea;
  • accumulation of fluid in the lungs;
  • arrhythmia;
  • weight loss of fingers and their thickening at the tips;
  • dizziness, loss of consciousness.

Heart cancer is tricky enough. The first step is in the diagnosis, the second is in the rapid development, and thirdly, in the treatment.

At the initial stage, a malignant tumor does not reveal itself for a long time, therefore, in most cases, it is detected at later stages using ultrasound, when the cancer began to actively spread metastases. The prognosis for diagnosing heart cancer depends on the stage of the disease at which the treatment was carried out.

Diagnosis of a malignant tumor is performed using ultrasound, magnetic resonance imaging, computed tomography, radioisotope ventriculography, angiocardiography, echocardiography.

Ultrasound allows you to examine the condition of the heart muscle, atrium and left and right ventricles. This method provides complete information about the presence of a tumor formation in the organ. In addition to ultrasound, other examination methods are also used.

Treatment of heart cancer, due to late detection, is more palliative in nature. In most cases, the operation is already quite late, so measures are taken to reduce symptoms and improve the general condition of the patient.

Surgical treatment is used only in the early stages. In this case, the tumor is removed, but only if it has a primary nature of origin. Such a radical treatment still does not give a guarantee. In four out of ten people, cancer comes back within the first two years after surgery.

Treatment of cardiac oncology involves an integrated approach. It includes the use of chemotherapy and radiation. To eliminate pathologies that have arisen as a result of the development of a tumor, symptomatic treatment is used. A course of supportive and restorative therapy is mandatory. The use of medicines is selected taking into account the individual characteristics of the organism.

Despite the fact that the prognosis for oncological neoplasms is very unfavorable, complex therapy can prolong the patient's life by five or more years.

If you do not start timely therapy, the cancer will quickly "eat" the person. The prognosis of the development of the disease, after the onset of symptoms of oncology, is unfavorable. In less than a year, death occurs.

4290 0

Definition

Heart tumors are benign or malignant neoplasms that affect the heart wall, pericardium, or have intracavitary growth. Malignant tumors of the heart can be both primary and secondary, caused by the invasion or metastasis of the tumor. More often, such a generalized, malignant tumor process is detected in cancer of the lung, esophagus and lymphogranulomatosis.

Epidemiology

Not developed.

Prophylaxis

Not developed.

Classification

The classifications of primary cardiac tumors are predominantly of a working nature and are based mainly on the pathomorphological picture of the tumor.

According to this criterion, it is customary, first of all, to isolate benign and malignant tumors. By localization in the heart, intracavitary, myocardial and epicardial tumors, as well as tumors with damage to the heart valves, are isolated.

Benign tumors account for at least 2/3 of all cardiac neoplasms; among the tumors diagnosed in vivo, this indicator is even higher. Primary malignant tumors are rare and occur mainly in children.

  • Benign cardiac tumors: myxoma, papillary fibroelastoma, rhabdomyoma, fibroma, lipoma, hemantioma, neurinoma, pheochromocytoma.
  • Malignant tumors of the heart: angiosarcoma, myosarcoma, fibrosarcoma, liposarcoma.

Etiology

The etiology of most cardiac tumors has not been determined. A number of benign neoplasms of the heart have a family hereditary character (myxomas, papillary fibroelastomas).

Pathogenesis

Pathogenesis depends not only on histology, but also on the macrostructure and primary localization of the tumor. Potentially, cardiac tumors of any localization have a "malignant" course. Even with a benign nature, intracardiac, intrapericardial or intramural tumor growth can cause significant dysfunction of the heart and its valves.

Most often (about 90% of observations of cardiac tumors) in clinical practice, it is necessary to deal with myxomas, most of them - derivatives of mesenchymal tissue. Myxomas are formed over many years of the patient's life. From a pathogenetic point of view, these tumors are referred to as intracardiac endodermal heterotopia. In women, myxomas are detected 2-3 times more often than in men.

Rice. 1. Mixoma LP. a - echocardiography, myxoma is fixed on the pedicle to the interatrial septum, jamming of the left AV hole; b - MRI, the same patient before surgery

By localization, the majority of myxomas have intra-atrial growth (LA at least 75%, PP - 20%). Two-atrial growth is much less common, and multiple - as casuistry.

Clinical picture

Depends on the location and size of the tumor. With the left atrial location, the main symptomatology is associated either with the clinic of "mitral stenosis" or with the consequences of systemic embolism. Symptoms of mitral valve stenosis are most pronounced when the left AV opening is jammed (Fig. 1, 2). However, most often myxomas and other primary cardiac tumors are detected accidentally during echocardiography, without specific clinical symptoms. With advanced stages of tumor growth, congestive heart failure and heart rhythm disturbances are possible. With lesions of the myocardium and pericardium, signs of damage to the contractile function of the ventricle or symptoms of pericarditis (more often in primary malignant formations) are revealed.

Rice. 2. Preparation of the tumor removed during the operation

According to sectional studies, cardiac tumors are detected in 0.0017 - 0.28% of all autopsies. Metastatic (secondary) tumors are 10-40 times more common.

The possibilities of modern imaging methods, advances in cardiac surgery have turned the problem into a clinical one. The largest group of heart tumors is represented by benign rhabdomyomas, fibromas, teratomas and myxomas. Malignant tumors are 3 times less common. Usually these are sarcomas, of which angiosarcoma is more often recorded, affecting mainly boys, rapidly and massively metastasizing through the blood vessels.

Despite significant advances in the detection of cardiac tumors, the introduction of magnetic resonance imaging into everyday practice, the experience of intravital diagnosis of this pathology in childhood is insignificant. We have our own data on the successful diagnosis of heart tumors, which are presented in this work.

Benign tumors occur in 75% of all primary cardiac tumors.

Rhabdomyomas are the most common heart tumors in children. In 50-80% of cases, they are combined with tuberous sclerosis. Histologically, they are represented by large spindle-shaped vacuolated cells with large reserves of glycogen, a nucleus shifted to the periphery and granular cytoplasm. Macroscopically, rhabdomyomas are often multiple, gray-yellow in color, ranging in size from a small pea to a giant node relative to the heart, affect the walls of the heart chambers and septa, and can spread both into the cavity of the ventricle (Fig. 1) and extracardially (Fig. 2).

Rice. one. A heart tumor (indicated by markers) originating from the interventricular septum into the cavity of the left ventricle.


Rice. 2. Tumor of the left ventricle, spreading mainly extracardially and emanating from the wall of the left ventricle with the transition to the interventricular septum.

Heart teratomas are rarely malignant. The tumor is built from all three embryonic tissues and usually grows into the anterior mediastinum, usually proceeding from the pericardium. Teratomas can form in the interchamber septa of the heart, right atrium and ventricle. In newborns, the volume of the tumor may exceed the volume of the heart.

Fibroids look like solitary formations in the free wall of the left ventricle (rarely - the interventricular septum), in 40% they are found in children 1 year of age.

Myxomes are built from lipid-laden multinucleated cells immersed in a myxoid stroma rich in glycosaminoglycans. The cells are arranged in groups around the vessels. In 10% of cases, calcium deposits are found in the tumor. Myxomas are typical for adolescents (Fig. 3), they are rare in children. More than 80% of all mixomas come from the atrial septum and mitral valve. Myxoma can be either sporadic (90% of cases) or a familial tumor (10%) inherited autosomally dominantly (myxoma syndrome) within the framework of Carney's syndrome. In the familial variant, myxoma is usually multiple and prone to recurrence.


Rice. 3. Left atrial myxoma.
a) 1 and 2 - the wall of the aorta, 3 - myxoma.


b) Macro-specimen of removed myxoma.

In contrast to the sporadic variants of myxoma, familial cases are more often recorded in children and females. 70-75% of all cases of Carney's syndrome are caused by a mutation of the PRKAR1A oncosuppressive gene on chromosome 17q23-q24, acting through the protein 1A of the regulatory subunit of protein kinase A. Less commonly, Carney's syndrome is caused by a mutation on chromosome 2p16. There are no phenotypically differences between the two genetic variants of the syndrome. In patients with Carney's syndrome and myxoma, a mutation in the protein kinase A gene is found. The syndrome is manifested by multiple lentigenesis with a predominant location of age spots on the face, eyelids, conjunctiva, and oral mucosa. Endocrine hyperactivity is characteristic. Actually myxoma, in addition to heart failure, can manifest itself with fever, joint pain, shortness of breath, rough diastolic murmur and "tumor click": the impact of the tumor on the valve leaflets. Looseness of myxomatous tissue often leads to embolism.

Angiomas (hemangiomas or, very rarely, lymphangiomas) originate from proliferating endothelial cells. Infiltration of the interventricular septum can lead to a complete blockage of the conducting system. A complication of hemangiomas is hemorrhagic cardiac tamponade.

Lipomas, leiomyomas, mesotheliomas, fibroelastomas are extremely rare.

Malignant tumors account for 25% of primary cardiac tumors. Sarcomas originate from mesenchymal cells, more often occur in the right atrium, rarely occur in children. They are characterized by histological diversity, aggressive growth, and the intensity of metastasis. Rhabdomyosarcomas, characterized by invasive growth and metastasis, are very rare. The vast majority of all cases of rhabdomyosarcoma are found in children, which is explained by their origin from embryonic cells. Fibrosarcomas are built of intertwining strands of fusiform cells with elongated nuclei and numerous mitoses, affect several chambers of the heart, in the tumor nodes, foci of hemorrhage and necrosis are clearly defined. Angiosarcomas, the most common malignant tumors of the heart, are built up of numerous vascular tubules of various diameters formed by atypical endothelial cells.

In isolated cases, lymphomas, histiocytomas, leiomyosarcomas, liposarcomas and osteosarcomas have been described.

The clinical picture of cardiac tumors nonspecific and is determined not so much by the histological type as by localization and paraneoplastic reactions that mimic systemic diseases. In our observations, the initially erroneous diagnoses were hemorrhagic vasculitis, epilepsy, pneumonia. Benign tumors located in the region of the external tracts or in the inflow area, changing the natural flow of blood, can give more vivid symptoms than malignant ones. Tumors in the right heart are quickly manifested by general weakness, shortness of breath, jugullary pulse, inferior vena cava syndrome, edema, and ascites. Left heart tumors manifest with unproductive cough, fever, chills, shortness of breath, dizziness, cold sweats on exertion or at night. A tumor located in the cavity of the left ventricle or atrium can be a source of emboli with unexpected episodes of cerebral ischemia with convulsions or impaired blood circulation in the peripheral vessels. Anxiety, loss of appetite, and developmental delay may be the first common symptoms in infants and young children. At any age, periods of pallor, tachycardia, shortness of breath, hemoptysis, fainting are typical. Invasive tumor growth can cause disturbances in rhythm, conduction, and coronary blood flow. In our 4-month-old patient, ECG showed infarct-like changes in the myocardium in the anterolateral wall and apex of the left ventricle.

The results of the clinical examination depend on the location of the tumor. Common manifestations of cardiac tumors can be fever, heart failure, the appearance of a new murmur in the heart, an increase or the appearance of a new timbre of an already existing murmur, rhythm disturbances, and pericardial effusion. Right-sided tumors quickly manifest with heart failure, edema, jugullary pulse, pericardial effusion, ascites, hepatomegaly, inferior vena cava syndrome, pulmonary embolism. Left-sided tumors can manifest with syncope, cerebrovascular accident, peripheral vascular embolization. In adolescents, unproductive coughing and hemoptysis are possible. The myxoma of the left atrium is characterized by a diastolic clap (due to the impact of the myxoma against the valve leaflets) or diastolic murmur when the myxoma is introduced into the left atrioventricular opening. In our observations, 45% of all cases of heart tumors occurred in children in the first 3 months of life, and all heart tumors in children in the first 7 years of life were represented by rhabdomyomas. Myxomas were found in children 10 years of age and older.

Electrocardiography reveals nonspecific changes: changes in the ST-T segment (often recorded in obstructive variants of rhabdomyomas or fibroids), decreased QRS voltage (especially in pericardial effusion), rhythm disturbances (in tumors, arrhythmias respond very poorly to therapy) and conduction.

Laboratory research informative only for differential diagnostics. With cardiac tumors, an increase in ESR, paraspecific changes in the blood count is possible. With stagnation in the systemic circulation, the activity of hepatic transaminases increases. The activity of the myocardial fraction of creatine kinase reflects primarily myocardial dysfunction, but not the malignancy or benignity of the tumor.

Differential diagnosis is carried out with heart defects, carditis, cardiomyopathies, myocardial fibroelastosis, idiopathic pulmonary hypertension and other conditions, the manifestations of which can be simulated by a heart tumor.

Chest x-ray allows you to identify an increase in the size of the heart, stagnation in the pulmonary circulation, calcifications.

Echocardiography (EchoCG)- the first and leading method for diagnosing heart tumors. With the minimum cost of the study and time expenditures, it is possible to obtain complete information about the presence of a volumetric lesion of the heart, its spread in the heart cavity, extracardially or invasively intramyocardially, the size of the lesion, the state of the membranes of the heart, the valve apparatus, the degree of myocardial dysfunction. Echographically, the tumor looks like a deformation of the heart wall or, in cases of intracavitary myxus, as a mass formation. In the presence of a pedicle, myxoma can prolapse into an adjacent chamber under the action of blood flow. In such cases, earlier in the M-mode, images during the diastole period described multiple linear echoes (the phenomenon of "coin bars") between the leaflets of the mitral valve. Difficulties are possible with small tumors, especially intramyocardial tumors, which are characterized by persistent rhythm disturbances. But the echographic conclusion in itself does not allow us to speak absolutely reliably about the type of volumetric formation. For example, the detection of a rounded formation in the left atrium with high reliability testifies in favor of myxoma, but a spherical thrombus is also possible.

Magnetic resonance imaging (MRI) the most informative imaging technique for diagnosing heart tumors, which allows more accurately than echocardiography to establish the size and extent of the tumor, to present volumetric reconstruction and to differentiate the tumor from a thrombus.

Conclusion

Thus, primary cardiac tumors in children and adolescents are characterized by a nonspecific polymorphic clinical picture. The first diagnostic method is an ultrasound of the heart, which is able to identify a space-occupying mass, assess its size, and its connection with the structures of the heart. According to EchoCG data, it is impossible to make unconditional conclusions about the type of mass formation without clinical interpretation, other imaging techniques and histological examination. Clinical interpretation of the data obtained allows for differential diagnosis and selection of the most justified treatment method.

Literature

  1. Lam K., Dickens P., Chan A. Tumors of the heart. A 20-year experience with a review of 12,485 consecutive autopsies // Archive of Pathol. Lab. Medicine. 1993. V. 117. P. 1027-1031.
  2. Rodriguez-Cruz E. Pediatric cardiac tumors // http: // emedicine.medscape. Last Updated 01 December 2011.
  3. Basson C., MacRae C., Korf B., Merliss A. Genetic heterogeneity of familial atrial myxoma syndromes (Carney complex) // American Journal of Cardiology. 1997. V. 79. P. 994-995.
  4. Leiber B., Olbrich G. Die klinischen Syndrome. Urban & Schwarzenberg, Muenchen, 1993. Bd. 12.
  5. M.A. Nechaenko Primary heart tumors: M., Diss. ... Dr. med. Sciences, 1993.
  6. Casey M., Vaughan C., He J. et al. Mutations in the protein kinase A R1alpha regulatory subunit cause familial cardiac myxomas and Carney complex // Journal Clinical Invest. 2000. V. 106. P. 31-38.
  7. Goldstein M., Casey M., Carney J., Basson C. Molecular genetic diagnosis of the familial myxoma syndrome (Carney complex) // American Journal of Med. Genet. 1999 V. 86. P. 62-65.
  8. Reynen K. Cardiac myxomas // New England Journal of Medicine. 1995. V. 333. P. 1610-1617.
  9. Veugelers M., Bressan M., McDermott D. et al. Mutation of perinatal myosin heavy chain associated with a Carney complex variant // New England Journal of Medicine. 2004. V. 351. P. 460-469.
  10. Yamomoto T., Nejima J., Ino T. et al. A case of massive left atrial lipoma occupying pericardial space // Jpn. Heart Journal. 2004. V. 45. P. 715-721.
  11. Girrbach F., Mohr F., Misfeld M. Epicardial lipoma - a rare differential diagnosis in cardiovascular medicine // European J. of Cardiothorac. Surg. 2012. V. 41. P. 699-701.
  12. Beckwith C., Butera J., Sadaniantz A. et al. Non Hodghins Lymphoma involving the Heart // Journal of Clinical Onkology. 2000. V. 18. P. 1996-1999.
  13. Terada T. Primary Cardiac Lymphoma // Tokai Journal of Experimental and Clinical Medicine. 2007. V. 32. P. 14-16.
  14. Yalimiz H., Salik O., Tokcan A. Malignant Fibrous Histiocytoma of the Heart // Texas Heart Institute Journal. 2008. V. 35. P. 84-85.
  15. Miyake C., Del Nido P., Alexander M. et al. Cardiac tumors and associated arrhythmias in pediatric patients, with observations on surgical therapy for ventricular tachycardia // Journal of American College Cardiology. 2011. V. 58. P. 1903-1909.
  16. Beroukhim R, Prakash A, Buechel E et al. Characterization of cardiac tumors in children by cardiovascular magnetic resonance imaging: a multicenter experience // Journal of American College Cardiology. 2011. V. 58. P. 1044-1054.
Loading ...Loading ...