Adjuvant chemotherapy for primary cardiac sarcomas: the IGR experience

The effect of additional treatments after surgery in patients with primary cardiac sarcoma (PCS) remains unknown. The present study aims to evaluate t he benefit of chemotherapy in patients with non-metastatic cardiac sarcomas after optimal resection. Between October 1979 and December 1995, 15 patien ts with a median age of 45 (range 16-66) and a resected primary cardiac sar coma [angiosarcoma (six), malignant fibrous histiocytoma (three), leiomyosa rcoma (two), rhabdomyosarcoma (two), liposarcoma tone) and synoviosarcoma ( one)] received a doxorubicin-containing regimen within 6 weeks of surgery. Adjuvant chemotherapy combinations included cyclophosphamide, vincristine a nd dacarbazine in four patients; ifosfamide in nine; methotrexate and Vincr istine in one; and doxorubicin alone in one patient. At present, 13 patient s have relapsed (five during therapy), with a median time to progression of 10 months. Twelve patients developed local relapse, in four cases without metastatic disease. Two patients remain in complete remission 27 and 25 mon ths after surgery. The median time to progression was shorter in patients p resenting a cardiac angiosarcoma than other histological types (3; vs 14 mo nths, P< 0.01). Twelve patients have died, with a median overall survival o f 12 months. The 2-year survival rate is 26%. Survival was significantly lo nger for patients with completely resected tumours (22 vs 7 months; P = 0.0 2) and those who did not have angiosarcoma (18 vs 7 months; P = 0.04). In c onclusion, post-operative conventional doxorubicin-based chemotherapy faile d to modify the natural history of patients with resected cardiac sarcomas. Locoregional failure remains the main problem even after histologically co mplete resection. New approaches must be tested in patients with primary ca rdiac sarcoma.