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 cases and small series generally describe spindle-celled, high-grade tumors with poor short-term survival; however, the pathologic features of many of these tumors are incompletely documented. The authors report in detail the clinicopathologic features of 2 relatively low-grade epithelioid and spindle-celled primary cardiac leiomyosarcomas.


METHODS
Cases 1 and 2 were studied using standard histochemical and immunohistochemical techniques, and case 1 was examined by electron microscopy. The 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 grade 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 mitoses 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 leiomyosarcoma 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 low-grade features or mixed low- and high-grade features also have a poor overall long-term survival, with a high rate of local recurrence and systemic spread.
Pins
M R
MR
Department of Pathology, Northwestern Memorial Hospital, Chicago, IL 60611, USA.
Ferrell
M A
MA
Madsen
J C
JC
Piubello
Q
Q
Dickersin
G R
GR
Fletcher
C D
CD
D000328:Adult; D017809:Fatal Outcome; D005260:Female; D006338:Heart Neoplasms / Q000175:diagnosis* / Q000473:pathology / Q000648:ultrastructure; D006801:Humans; D007150:Immunohistochemistry; D007890:Leiomyosarcoma / Q000175:diagnosis* / Q000473:pathology / Q000648:ultrastructure; D008279:Magnetic Resonance Imaging; D008297:Male; D008854:Microscopy, Electron