Angiosarcoma of the heart: case report and review of the literature.

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