Abstract

Primary tumours of the heart are reported with a prevalence rate of around 0.01% in collective autopsy series. Malignant tumours account only for 25% of all primary cardiac neoplasms most of which are sarcomas and the most common cardiac sarcoma is Angiosarcoma.

We present a case report of a 46 year old female, who had history of dyspnoea, chest pain, weight loss, fever and body ache. Cardiac MRI revealed a huge Angiosarcoma of Right Atrium (10.8 x 8.2 x 6.6 cm) with extension into the Right Ventricle. Diagnosis of Angiosarcoma was established on histology. The literature on Angiosarcoma of heart is reviewed.

Introduction

Primary tumours of the heart are extremely rare with a prevalence rate of around 0.01% in collective autopsy series; malignant tumours account for only 25% of all primary cardiac neoplasms, most of which are sarcomas.^1-8

Bony, neurogenic and soft tissue sarcomas are known to arise from the heart, but tumours of vascular origin, particularly Angiosarcomas (40%) account for majority.1-2 In 90% of cases Angiosarcomas arise from the Right Atrium (Fig. 1).^1,2,7
Even though radical resection of tumour was planned on Cardiopulmonary Bypass.

Case Report

A female 46 years of age, presented with complaints of dyspnoea on exertion (NYHA class III-VI), chest pain, fever, generalized body ache and weight loss for a duration of three to four months. On clinical examination patient had raised Jugular venous pressure and tender hepatomegaly. Haematologic studies, Biochemical investigations and Coagulation profile were normal. Transthoracic Doppler revealed an enlarged Right Atrium with a Right Atrial mass of size 7.2 x 5.1 cm protruding into Right ventricle with diastolic peak gradient of 13 mm of Hg across tricuspid valve. Cardiac MRI (plain and contrast), showed a lobulated mass of size 10.8 x 8.2 x 6.6 cm occupying most of the Right Atrium upto the level of Superior vena cava, with extension via the tricuspid valve into the Right Ventricle (Fig. 2). The lateral Right Ventricular wall was involved with obliteration of planes with pericardium. The Ejection Fraction was quantified to be 23%. Computed Tomography Coronary Angiography was suggestive of encasement of distal Right Coronary artery due to tumour mass (Fig. 3).

Even though radical resection of tumour was planned on Cardiopulmonary Bypass, only a biopsy was possible due to extensive involvement of the right side of the heart. Biopsy report confirm it to be an Angiosarcoma of the heart (Fig. 4).

Fig. 1 : Large pleomorphic tumour cells arranged in a sinusoidal pattern - H & E x 40).		Fig. 2 : Cardiac MRI, showing a mass in Right Atrium extending into the Right Ventricle).
Fig. 3 : CT Coronary Angiography, showing encasement of distal Right coronary Artery due to tumour mass).		Fig. 4 : Operative photograph showing involvement of lateral Right Ventricular wall)

Discussion

Angiosarcomas of heart are common on right side while other types of sarcomas particularly osteosarcomas are predominantly left sided tumours.1,2 Angiosarcomas are seen equally in both the sexes and usually occur in third or fourth decade.^2,3

Clinical features : Harvey suggested that the clinical presentation of patients in a case of primary tumours of the heart is dictated by the structures they involve. Angiosarcomas are at times known to present with somewhat similar clinical features.^7-11 The clinical manifestations of malignant tumour can be due to 1) pericardial involvement causing effusion or constrictive pericarditis, 2) congestive cardiac failure due to mass effect of the tumour or due to obstruction to the flow of blood, 3) Embolisation, 4) chest pain due to compression of myocardial vessels, 5) constitutional symptoms.^1,3

Treatment : Extended radical resection of tumour with reconstruction of heart chambers and valves, or circulation via some Fontan type of cardiac reconstruction is advocated.^3,4,12,13 Ex-Vivo resection of tumour with cardiac auto transplantation has been shown to extend survival.^3,13

Doxorubicin based chemotherapy along with combination of cyclophosphamide, vincristine, dacarbazine or cisplatin is most commonly employed as adjunct to surgery.^7,14,15

Radiotherapy and Immunotherapy with Interlukin-II has been shown to reduce local recurrence and improve survival.^7,14,15

References

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*Registrar; **Associate Professor; ***HOD; +Observer; LTMGH Sion Hospital, Mumbai 400 022