Heart tumours

Realdo Columbo (also known as Columbus) was the first to describe an intracardiac tumor in his work "De re anatomica", published in Venice in 1559 and in Paris in 1562.

In the 19th century, clinical references were then presented for the first time, as in the work "Observations of some of the most frequent and important diseases of the heart" by Allen Burns, published in London in 1809, and the descriptions of left atrial tumors by T. W. King, published in 1845.

In 1934, Barnes was the first to confirm a primary sarcoma pre-mortem by electrocardiography and biopsy.

Ivan Maheim published the first monograph on cardiac tumors in 1945, which for many years also set the standard for the classification of neoplasms.

Prichard published the first histopathologically oriented overview of benign and malignant neoplasms in 1951.

In 1952 Goldberg succeeded in the first cineangiographic imaging of a myxoma. Surgical consequences could not be drawn at that time.

Notes:

• Until the middle of the last century the diagnosis of a cardiac tumor was made mainly during autopsy.
• The first surgical treatments of heart tumors with satisfactory long-term results were only achieved after the introduction of extracorporeal circulation in the mid 1950s. In 1954 Craford was the first to resect a myxoma with the aid of the heart-lung machine (Hoffmeister 2014; Unger 1995).

A heart tumour is a benign or malignant new formation in the area of all parts of the heart. They can occur on the internal structures of the heart as well as in the myocardium, pericardium and epicardium (Lindinger 2019).

Cardiac tumours are divided into primary, secondary, malignant and benign tumours.

Primary benign tumours include:

• myxoma 30%
• papillary fibroelastom 10 %
• lipoma 10 %
• Rhabdomyoma 7 % (occurs preferentially in children [Herold 2020])
• Fibroma 4
• Hemangioma 3 %
• Mesothelioma of the AV node 3%.

and a total of approximately 2 %:

• Granulosa cell tumor
• Neurofibroma
• Lymphangioma (Bob 2001)

Primary malignant tumors include:

• Angiosarcoma 9%.
• Rhabdomyosarcoma 8
• Mesothelioma 4 %
• Fibrosarcoma 3

total approx. 4 %:

• malignant lymphoma
• Teratoma
• Thymoma
• Osteosarcoma (Bob 2001)

Secondary cardiac tumors are metastases of solid tumors (such as bronchial Ca, mammary Ca, renal Ca, melanoma, pleural mesothelioma, etc.) or lymphomas (Pinger 2019).

Overall, heart tumors occur only rarely. In autopsies they are found in about 0.02%. The gender ratio female / male is 3: 1, with an age peak between 40 - 60 years (Herold 2020).

Primary heart tumours are benign in approx. 80 % of cases and malignant in approx. 20 % (Herold 2020).

• Primary benign tumours are more frequently found in the atrium than in the ventricle (Stierle 2017). Of these, approx. 70 % are myxomas in adults and rhabdomyomas predominate in children (Herold 2020).
• In primary malignant tumors, sarcomas predominate (Herold 2020).

Secondary cardiac tumors are up to 40 times more common than primary tumors. They thus represent the largest group of cardiac tumours (Stierle 2017).

A familial accumulation of heart tumors occurs in about 5 % - 10 % and is called "myxoma syndrome" or "carney syndrome" together with pigmented naevi, endocrine disorders and subcutaneous myxomas.

In all other cases the aetiopathogenesis is unclear.

Localizations of the heart tumors:

• Primary cardiac tumors: Primary cardiac tumors are located mainly in the left heart. Myxomas usually originate with a short stem from the atrial septum. They can grow into the left ventricle or mitralostium. Rhabdomyomas are more likely to be found in the area of the tip of the left ventricle (Eichstädt 1996).
• Secondary cardiac tumors: Secondary cardiac tumors either metastasize per continuitatem or are washed into the heart by the venous blood. As a result, secondary tumours are more frequently found in the right heart. The pericardium is most frequently affected, followed by myocardium and endocardium (Bob 2001).
• The following primary tumors metastasize to the heart:
  • per continuitatem: bronchial Ca, mammary Ca
  • by venous infiltration: renal Ca, testicular Ca (Bob 2001)
  • and more rarely: melanoma, pleural mesothelioma (Pinger 2019)

The clinical symptoms are not uniform because the symptoms depend on the location of the tumour (Stierle 2017).

The following symptoms may be present:

• Palpitations
• Dyspnoea, which develops rapidly
• positional chest pain
• Swindle
• Syncopes
• Nausea
• Weight loss
• Fever
• arterial embolisms (often in the case of myxomas, then frequently occurring as the first symptom)
• Right heart failure (for tumours in the right atrium)
• sudden cardiac death

and predominantly in the case of malignant tumours located in the ventricle or intramural space)

• Pericarditis
• Pericardial tamponade (Stierle 2017)
• Pericardial effusion (Kasper 2015)

Since life-threatening complications can occur with all tumours - regardless of their histological type - early diagnosis is essential (Kasper 2015). Parallel to the diagnosis of tumours, the search for a primary tumour should also be carried out, as heart metastases are far more common than primary heart tumours (Stierle 2017).

Thoracic X-ray: In most cases an inconspicuous X-ray image is found. However, there may also be an enlargement of individual heart chambers or calcifications may be detectable. The latter occurs particularly with fibromas and myxomas (Stierle 2017).

Echocardiography: Echocardiography should always be performed as transesophageal echocardiography (TEE). Especially atrial tumors can be detected well. Malignancy criteria are:

• intramural growth
• multiple tumors
• Pericardial effusion (Stierle 2017)

Cardio- MRT / CT: A cardio- MRT or CT should be performed for planning the operation.

Here, the intramural expansion and possible infiltration of the pericardium can be detected better than in ultrasound. Tissue characterization and assessment of vascularization are also possible (Stierle 2017).

Cardiac catheterization: A cardiac catheterization is always indicated if preoperatively there is a coronary heart disease. If angiographic imaging of tumour vessels is possible, this is an indirect sign of malignancy (Stierle 2017). However, the imaging itself is associated with a high risk of tumor embolisation (Bob 2001)

There is almost always a:

• BSG- increase

exist less frequently:

• Leucocytosis
• Hb- Waste
• Change in platelet count

Auscultation

An uncharacteristic heart murmur may occur during auscultation (Herold 2020).

In case of a stalked tumor in the left atrium, typical signs of mitral insufficiency or mitral stenosis may be auscultable. A cramming 1st heart sound and a 4th heart sound may be present.

In case of a tumor in the right atrium a systolic sound may be present parasternally on the right side.

In case of a tumor in the left ventricle there may be a sound similar to HOCM (Stierle 2017).

• cardiac thrombi
• Vegetation in the area of the endocardium or the valves
• Pericardial cysts (Lindinger 2019)
• Li- Fraumeni Syndrome: This is an autosomal dominant tumor disease of the heart which occurs predominantly in patients < 45 years of age who have already undergone tumor disease (Hoffmeier 2014).

The complications often appear as initial symptoms. They include:

• Cardiac arrhythmia (found in > 50% of patients; often in the form of AV blockages)
• thromboembolic events (occur at about 25%) such as:
  • Brain Embolism
  • Pulmonary embolism
  • arterial embolisms
• Acute pulmonary oedema (as a result of left heart failure)
• sudden cardiac death
• Metastasis in primarily malignant heart tumors

Patients with a cardiac tumour should be immediately referred to an interdisciplinary centre (Hoffmeister 2014).

All primary benign tumors should be surgically removed as soon as possible to prevent potential complications (Bob 2001).

Primary malignant tumors generally have a very poor prognosis. In individual cases, palliative surgery or chemotherapy may be considered (Stierle 2017).

The treatment of secondary tumours generally depends on the prognosis of the primary tumour. Therapeutic measures may be considered:

• in case of signs of a pericardial tamponade, application of a pericardial drainage
• in case of rapid after-effusion, local chemotherapy with e.g. mitoxantrone 10 mg via the horizontal drainage
• Radiotherapy if the primary tumor is radiation-sensitive and symptoms are due to heart metastasis (Stierle 2017)

The prognosis for patients with benign heart tumours is good.

The recurrence rate is about 0 % - 3 % (Herold 2020).

In the case of myxomas, any recurrences occur mainly remotely from the site of the previously operated primary tumour. If younger patients are affected by recurrences, the exclusion of a familial myxoma syndrome is recommended, as recurrences occur more frequently than usual (Flachskampf 2011).

The prognosis is poor for all malignant tumours of the heart. The mean survival time is about 9 months (Herold 2020).

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