Angiosarcoma of the Heart: Case Report and Review of the Literature

Background: Primary angiosarcoma of the heart is an extremely rare malignant disease. Patients and Methods: A 32-year-old female with primary angiosarcoma of the heart at an advanced stage with lung and bone metastases is presented. The tumor showed extensive expression of c-erb-B2 and a moderate expression of c-kit. Chemotherapy (cisplatin, epirubicin and ifosfamide) was administered. Herceptin as well as glivec were added to the above combination. Results: There was a good partial response and the lung deposits almost disappeared. The duration of response was 6 months. Conclusion: This case of angiosarcoma of the heart is presented because of the extreme rarity of this disease, and its responsiveness to chemotherapy in combination with imatinib and herceptin. Primary tumors of the heart are rare entities with an autopsy-defined incidence of 0.2% (1); one quarter of these are benign tumors. The most frequent malignant cardiac tumors are sarcomas, with patients having a median survival of 6 months (2). Angiosarcoma of the heart constitutes 2% of primary cardiac neoplasms (including benign tumors) and it is the most common primary malignant cardiac tumor (3). More often it develops in the right ventricle, especially arising from the right atrium, and less often from the pericardium (4). These tumors may be asymptomatic over a long period of time and when the symptoms appear, the disease is already advanced. The main clinical signs are pulmonary hypertension, haemopericardium, cardiac tamponade and pulmonary embolism (5). The present study presents a case of primary cardiac angiosarcoma and a review of the literature. Case report A 32-year-old woman was admitted to hospital because of a 10-day fever, shortness of breath and pain in the left shoulder which radiated to the left arm. The ECG demonstrated sinus tachycardia with a pulse rate of 140 bpm which was persistent and not related to the high fever. She was clinically immobilized and any effort increased breathlessness. A transesophageal echocardiography was performed and showed a tumor 4.6x2 cm in the right ventricle. Computed tomography (CT) of the chest showed multiple metastatic nodules in both lungs and an osteolytic lesion with a soft- tissue mass attached to it, at the right first rib. A biopsy of this mass was performed. The histopathology showed angiosarcoma (malignant epithelioid hemangioendo- thelioma). At that time, CT of the abdomen and brain and a bone scan were negative with respect to the spread of the disease. Chemotherapy (epirubicin 75 mg/m 2 on day 1, cisplatin 80 mg/m 2 on day 1 and ifosfamide 2 gr/m 2 per day on days 1, 2 and 3, plus uromitexan 800 mg x 2 on days 1, 2 and 3) was administered. A histological specimen was examined for gene expression (particularly c-kit and c-erb-B2). Both were expressed, but c-erb-B2 in particular, was highly expressed. Trastuzumab (herceptin) 150 mg once weekly and imatinib (glivec 400 mg tablet) were added to the treatment. Patient follow-up. Within a short period of time, (4-6 weeks) the patient showed clinical improvement which was confirmed by a CT scan of the chest in the 8th week after the beginning of treatment. There was a good objective partial response and a highly improved clinical benefit (quality of life). The patient no longer required the administration of oxygen; the esophageal echogram, as well as the CT scan of the chest (both examined by experienced radiologists before treatment and one month and 6 months post treatment) showed a reduction in the size of the cardiac mass and the lung metastases had almost disappeared. The duration of response was 6 months; this