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.