tailieunhanh - Báo cáo khoa học: "Unusually young age distribution of primary hepatic leiomyosarcoma: case series and review of the adult literature"

Tuyển tập báo cáo các nghiên cứu khoa học quốc tế ngành y học dành cho các bạn tham khảo đề tài: Unusually young age distribution of primary hepatic leiomyosarcoma: case series and review of the adult literature | Shamseddine et al. World Journal of Surgical Oncology 2010 8 56 http content 8 1 56 WORLD JOURNAL OF SURGICAL ONCOLOGY CASE REPORT Open Access Unusually young age distribution of primary hepatic leiomyosarcoma case series and review of the adult literature Achraf Shamseddine 1 Walid Faraj 1 Deborah Mukherji2 Nadim El Majzoub2 Mohamed Khalife1 Ayman Soubra1 and Ali Shamseddine2 Abstract 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 . 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. Conclusion 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. Introduction Primary liver sarcoma is a rare tumor associated with rapid growth and poor prognosis. The majority of hepatic malignancies are carcinomas with sarcomas representing only to 2 of primary hepatic cancers 1 . Presenting symptoms are nonspecific and the diagnosis is often made post-operatively or even post-mortem. Surgery for resectable tumors combined with adjuvant chemotherapy is the current standard of care however very little data exists outside case series due to the rarity of the disease. Our institution is a leading tertiary referral centre for the treatment of hepatic malignancies in the region.