Unusually young age distribution of primary hepatic leiomyosarcoma: case series and review of the adult literature.
BACKGROUND
Primary hepatic leiomyosarcoma is a rare disease diagnosed in older aged adults with a median age of 58 and occasionally in children with a history of immunosuppression.


METHODS
From 1998 to 2009, 215 patients were diagnosed with primary hepatic malignancies at our institution, 4 of which were diagnosed with primary hepatic sarcoma (1.8%). Three cases were primary hepatic leiomyosarcomas (LMS) and one case was primary undifferentiated embryonal sarcoma of the liver; median age 30 (range 20-39) years.


RESULTS
One patient is currently 12 months post-resection with no evidence of recurrence. Two patients passed away at 19 days and 22 months from small for size liver and tumor recurrence respectively.


CONCLUSIONS
We have presented 3 cases of primary hepatic leiomyosarcoma diagnosed at our institution with an unusually young age distribution and no evidence of immunosuppression. These cases highlight the diagnostic and therapeutic challenges of this rare tumour.
Shamseddine
Achraf
A
Department of Surgery, HPB and Liver Transplantation Unit, American University of Beirut Medical Center, Beirut, Lebanon.
Faraj
Walid
W
Mukherji
Deborah
D
El Majzoub
Nadim
N
Khalife
Mohamed
M
Soubra
Ayman
A
Shamseddine
Ali
A
D000328:Adult; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007890:Leiomyosarcoma / Q000175:diagnosis* / Q000628:therapy; D008113:Liver Neoplasms / Q000175:diagnosis* / Q000628:therapy; D008297:Male; D012189:Retrospective Studies; D012509:Sarcoma / Q000175:diagnosis* / Q000628:therapy; D015996:Survival Rate; D016896:Treatment Outcome