Liver transplantation for primary hepatic leiomyosarcoma: a case report and review of the literatures.

Primary hepatic leiomyosarcoma (PHL), a malignant mesenchymal tumor of myogenic origin, is exceedingly rare. Patients may be afflicted with a wide spectrum of nonspecific symptoms, and most of the laboratory data are unhelpful to diagnosis. Here, we report a 44-year-old woman with anorexia and right upper quadrant pain who was diagnosed as having malignant hepatic tumor within the Milan criteria and treated by orthotopic liver transplantation (OLT), the pathologic diagnosis of PHL was made by postoperative immunohistochemical study. She underwent six courses of intravenous chemotherapy and enjoyed a 14-month tumor recurrence-free survival until the lymph node metastasis was detected, then we switched the tacrolimus to sirolimus and the patient survived for 20 months additionally. Unfortunately, she finally died of extensively lymphatic metastasis and cachexia. The diagnostic and therapeutic features of PHL are also discussed by reviewing the literatures. To our knowledge, this is the second report of administering OLT to PHL patients.