Epithelioid and spindle-celled leiomyosarcoma of the heart - Report of 2 cases and review of the literature

Background,-Primary cardiac leiomyosarcomas are rare. isolated reported cas es and small series generally describe spindle-celled, high-grade tumors wi th poor shortterm survival; however, the pathologic features of many of the se tumors are incompletely documented. The authors report in detail the cli nicopathologic features of 2 relatively low-grade epithelioid and spindle-c elled primary cardiac leiomyosarcomas. Methods.-Cases 1 and 2 were studied using standard histochemical and immuno histochemical techniques, and case 1 was examined by electron microscopy. T he literature was reviewed with regard to primary cardiac leiomyosarcomas. Results.-Both tumors showed epithelioid and spindle-celled areas. The tumor in case 1 was low grade, and the tumor in case 2 was predominately low gra de with a high- grade focus. A review of 28 reported cases revealed a wide age range (mean, 43 years), equal male-to-female ratio, and a predilection for the left atrium (48%). Follow-up of reported cases with fewer than 5 mi toses per 10 high-power fields showed a mean survival of 22 months compared with a 9-month mean survival for all others. Conclusions.-Short-term follow-up of reported cases of high-grade cardiac l eiomyosarcoma suggests a poor prognosis. Long-term follow-up in our case 2, along with follow-up of reported cases that were histologically similar to our cases, suggests that cardiac leiomyosarcomas with lowgrade Features or mixed low- and high-grade features also have a poor overall long-term surv ival, with a high rate of local recurrence and systemic spread.