Treatment and outcomes in adult patients with primary cardiac sarcoma: the British Columbia Cancer Agency experience.

BACKGROUND: To evaluate treatment and survival in a cohort of patients referred to a Canadian institution with newly diagnosed primary cardiac sarcoma.

METHODS: Between 1990 and 2006, 16 patients were referred to the British Columbia Cancer Agency with pathologically confirmed sarcoma of cardiac origin. Retrospective chart review was performed to document patient, tumor, and treatment characteristics. Disease-free survival and overall survival (OS) were calculated by Kaplan-Meier methods and compared in different subgroups by log rank statistics.

RESULTS: The cohort comprised 10 female and 6 male patients. The mean age was 51 years (range, 27-81 years). The most common histologic subtype was angiosarcoma. Surgical resection, alone or in combination with chemotherapy or radiotherapy, was undertaken in 10 of 12 patients with localized and 3 of 4 patients with metastatic disease. At a median follow-up of 8 months, all patients had died of disease. In the entire cohort, mean disease-free survival and OS were 6 months and 14 months, respectively. Patients with localized disease had significantly longer survival compared to metastatic disease (mean OS 18 months vs. 2 months, P = 0.001). Patients treated with complete resection had improved OS compared to incompletely resected disease (25 months vs. 6 months, P = 0.042). Age, sex, tumor grade, location, and subtype were not associated with statistically significant survival differences.

CONCLUSIONS: Patients with nonmetastatic cardiac sarcoma amenable to complete resection experienced improved survival. However, the high overall rates of disease progression and mortality highlight the need for more effective local and systemic treatments that may be used in conjunction with surgery to improve patient outcomes.