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VERIFIED CONTENTAuthor: lek. Łukasz Kujawa

Heart cancers are rare, but their location in such an important organ as the heart makes them a very important problem. We divide heart tumors into primary and secondary. What are the causes and symptoms of heart cancer? How is their treatment going?

Heart neoplasmsare rare, but heart tumors may be primary, that is, originate directly from the heart, or secondary - be the result of metastasis from another location. Primary neoplasms are extremely rare. Based on postmortem examinations, their incidence is estimated at 0.002-0.33% (less than 3 cases per 10,000 sections).

Metastases occur much more frequently in the population. Primary tumors can be divided into:

  • benign heart tumors(benign lesions account for as much as 75%),
  • malignant heart tumors .

Benign heart cancer

The most common primary heart cancer is myxoma ( myxoma ). Usually it grows as a pedunculated mass and is most often located in the left atrium as a single tumor. There are two forms: sporadic and family, which makes up about 5-10%. Familial mucus are more often multifocal in nature, are more often located in the heart chambers and recur more often.

Familial occurrence may be associated with Carney's syndrome, which includes, among others: multiple myxomas in the heart and other body locations, endocrine disorders, skin pigmentation changes, thyroid cancer and testicular neoplasms from Sertoli cells.

Other, less common, benign heart tumors include :

  • fibroma ( fibroma ) - the second most frequent; usually recognized in the second year of life; 5% related to the occurrence of Gorlin syndrome;
  • papillomatous fibroma ( fibroelastoma papillare ) - the most common neoplasm of the valves; applies to people in 6-7. decade of life;
  • rhabdomyoma ( rhabdomyoma ) - the most common in children; is associated with tuberous sclerosis;
  • lipoma ( lipoma );
  • hemangioma / lymphangioma ( haemangioma / lymphangioma );
  • teratoma ( teratoma );
  • lipomatous hypertrophy of the atrial septum.

Not malignantheart cancer - symptoms

The symptoms of benign heart tumorsdepend to the greatest extent on their location and size. The histological type plays a much smaller role. Slow growth often means that they do not cause any symptoms for many years and are detected accidentally.

When the tumor reaches dimensions due to which it disturbs the blood flow within the cavities and valves of the heart - typical symptoms of heart failure may appear, such as:

  • shortness of breath,
  • impaired exercise tolerance,
  • paroxysmal nocturnal dyspnea,
  • fainting,
  • pulmonary congestion,
  • puffiness.

Tumors located near the valves may imitate a heart defect - most often mitral regurgitation in left atrial myxoma. In turn, those located near the structures of the conducting system or spreading intramuscularly within the heart muscle can cause arrhythmias.

Tumors within the heart cavities can also fragment and form the starting point for blockages in the systemic or pulmonary circulation. In 30% of cases of the most common heart cancer - myxoma, systemic symptoms may also occur, such as:

  • weakness,
  • weight loss,
  • skin lesions,
  • fever,
  • joint pain,
  • Raynaud's phenomenon,
  • stick fingers,
  • anemia.

Benign heart cancer - diagnosis

In the case of heart tumors, imaging diagnostics plays the most important role.Echocardiographic examination (ECHO of the heart) is of primary importance. Standard Transstageal ECHO (TTE) provides basic information on tumor location, morphology and size. It also allows the assessment of blood flow disorders.

There are often indications for a variant of this test, i.e. TEE. In this case, the ultrasound probe is inserted into the esophagus as high as the heart. From this point, we can more clearly visualize the main and pulmonary veins, the atria and the interatrial septum.

For even more precise tumor assessment, magnetic resonance imaging (MR) and computed tomography (CT) are used. MR is perfect for determining the type of lesion and differentiation of neoplasm, e.g. with clots or bacterial vegetations in the course of IE, i.e. infective endocarditis.

The final diagnosis is made on the basis of the histopathological examination of the removed tumor.

Benign heart cancer - treatment and prognosis

The treatment of choice is tumor resection. Each myxoma should be removed in the regimenurgent. If not operated on, it can lead to sudden cardiac death (caused by a sharp closure of the atrioventricular openings) and dangerous embolisms, especially in the cerebral circulation.

Most benign tumors can be completely removed, but sometimes the extent of resection is so large that it requires simultaneous repair of the valve or implantation of a pacemaker. In the case of brown rot, there is a risk of recurrence. The relapse rate after surgery is around 3%.

They are much more common in patients with Carney's syndrome. They usually happen in the first 4 years after surgery. For this reason, each patient undergoes an annual echocardiographic check.

Malignant heart tumors

Primary malignancies in the heart are rarer than benign. They account for 25% of all primary heart cancers. Most of them are sarcomas. The most common of these is angiosarcoma, i.e. angiosarcoma, which is located in the right atrium in 80% of cases.

It aggressively infiltrates the heart structure, and metastases are usually present when symptoms appear and diagnosis is made. The most common sites for metastasis are: lungs (>50%), thoracic lymph nodes, mediastinum, and spine.

Survival time from diagnosis is usually several months. Other histological types of sarcomas:

  • rhabdomyosarcoma ,
  • fibrosarcoma ( fibrosarcoma )
  • malignant mesothelioma ( mesothelioma malignom )
  • smooth cell sarcoma ( leiomyosarcoma )

Lymphomas are another group of primary malignant tumors of the heart. Their occurrence may be related to infection with Epstein Barr virus (EBV) and immunosuppression in the course of AIDS or immunosuppressive therapy. The incidence of this group of heart cancers is increasing.

Malignant heart tumors - symptoms

Symptoms of malignant heart tumorsare nonspecific. The most common are:

  • shortness of breath,
  • retrosternal pains,
  • pleural pains,
  • palpitations,
  • fainting.

Due to the more frequent location in the right part of the heart, more often we are dealing with clinical features of the so-called "right ventricular insufficiency" - its features include: overfilled jugular veins, lower limb edema, enlarged liver, pleural and pericardial effusions.

As with other malignancies, typical general symptoms may occur: fever, weakness, loss of appetite and loss of weight. The pericardium is often involved in the disease process, resulting in the sacthe pericardial area around the heart, there may be a pool of effusion. As a consequence, we can deal with a full-blown tamponade of the heart.

Malignant heart tumors - diagnosis

The scope of imaging examinations performed in the case of malignant heart tumors is the same as in the case of benign lesions.Initial diagnosis is made on the basis of the transthoracic ECHO of the heart, often supplemented by a transesophageal examination. Diagnostics by means of MRI or CT allows for a detailed examination of the extent of infiltration of the heart walls and structures within the chest. Therefore, it allows you to plan a possible surgery.

Tumor spreading can also be assessed with positron emission tomography - PET. The problem, however, is the limited availability of this study. In the imaging examination, we can distinguish a number of features, such as: multifocal nature, blurred tumor boundaries, pathological vascularization or myocardial infiltration, which allow us to conclude with a high degree of probability that we are dealing with a malignant process.

There are possibilities for pre-operative histological evaluation of the tumor. These include: cytological examination of the pericardial fluid obtained by pericardiocentesis and examination of a biopsy taken during cardiac catheterization. Knowing the type of cancer allows you to choose the appropriate treatment strategy.

The final diagnosis is made on the basis of the histopathological assessment of the removed tumor.

Malignant heart tumors - treatment and prognosis

The choice of treatment depends on many factors, such as: histological type, size, location, the degree of infiltration of other structures, and the severity of symptoms. Cardiac surgery removal of the primary malignant lesion may be considered in the case of a tumor confined to the heart. Often, resection is not complete, but is palliative in nature - its aim is to reduce symptoms by reducing the tumor mass.

In unresectable cases, e.g. when distant metastases are present, systemic treatment is used.

Sometimes, when it is impossible to remove the tumor within he althy tissues, the technique of referral autotransplantation is used for cardiac excision, removal of the tumor outside the patient's body, and then reimplantation of the heart. Radio- and chemotherapy can be used as a complementary therapy to the procedure.

In most sarcomas, the response to this type of treatment is negligible. In some types of cancer, however, they are the method of choice. Primary chemotherapy is effective in reducing discomfort and prolonging life in patients with heart lymphoma. Radiotherapy can be beneficial in your casepericardial mesothelioma.

The prognosis is usually unfavorable. The survival time of patients usually does not exceed one year from the moment of diagnosis.

Cancer metastases to the heart

It is worth emphasizing that secondary tumors, i.e. metastases from other locations, are the most common type of neoplastic changes in the heart. They occur several dozen times more often than the primary neoplasms presented above. Their presence indicates a very advanced stage of the disease. The most common sources of heart metastases are:

  • lung cancer,
  • breast cancer,
  • leukemias and lymphomas,
  • pleural mesothelioma,
  • esophageal cancer,
  • melanoma.

The heart can be seized by:

  • lymphatic,
  • blood,
  • continuity infiltration,
  • by growing into the lumen of the venous system.

The most common route of lymphatic vessels is used by epithelial neoplasms and concerns changes in the pericardium or epicardial layer. The blood spreading usually affects the heart muscle itself. The heart is involved in melanoma and sarcomas in this way.

Continuity infiltration is observed in neoplasms located in the immediate vicinity of the heart, e.g. lung cancer, esophagus, thymus, and nipple cancer. However, metastases are much more frequent. Some neoplasms can grow into the heart cavities through the lumen of large veins, especially the inferior vena cava. This is how advanced kidney cancer, pheochromocytoma, hepatocellular carcinoma or fetal nephroma can spread to the right atrium. Wilms' tumor.

Symptoms directly related to a heart invasion are rare. If present, they are rather nonspecific. Patients complain of dull chest pain, coughing and shortness of breath, and general symptoms. Symptoms of the underlying disease are predominant.

Pericardial involvement is very common. The fluid in the pericardial sac can be exudative, exudative or even hemorrhagic. The external pressure exerted by the growing fluid disrupts the diastolic function and the filling of the heart cavities, causing the so-called heart tamponade.

The cause of this condition may be both direct involvement of the pericardium by the neoplastic process, as well as reaction in response to radio- or chemotherapy treatment.

The presence of metastases in the heart most often proves that the disease is incurable. Therefore, the treatment options are very limited. They mainly come down to palliative chemotherapy or radiotherapy. Surgical treatment consisting of resection is performed very rarely when it is appropriatefavorable conditions.

It is especially appropriate for tumors that spread intravascularly, penetrating through the inferior vena cava or pulmonary veins, as they usually do not invade the heart muscle. Often the surgical procedure is reduced to palliative procedures aimed at evacuating fluid from the pericardial sac.

The simplest of them, performed under local anesthesia, is the puncture of the pericardial sac, i.e. pericardiocentesis. When recurrent exudate is a problem, intraocardial administration of sclerotics or cytostatics such as bleomycin and cisplatin may be effective.

Another possibility of decompression is the execution of the so-called fenestration - the window communicating with the pleural (or peritoneal) cavity. Unfortunately, the prognosis for most patients with heart metastases is very poor.

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Category:

Oncology