Primary Monophasic Synovial Sarcoma of the Liver in a 13-year-old Boy.

Pediatric and developmental pathology : the official journal of the Society for Pediatric Pathology and the Paediatric Pathology Society

PubMedID: 23845006

Xiong B, Chen M, Ye F, Zhang Z, Yin L, Huang H, Chen H, Zhang H. Primary Monophasic Synovial Sarcoma of the Liver in a 13-year-old Boy. Pediatr Dev Pathol. 2013;.
Abstract Synovial sarcoma originating in the liver is extremely rare and thus far only 3 cases have been reported in the English-language literature. Herein, we report a primary hepatic synovial sarcoma in a 13-year-old Chinese boy. This patient present with a 10-day right upper quadrant pain and a heterogeneous mass was documented in the right hepatic lobe by computed tomography. Subsequently, the patient underwent right hepatectomy. Histologically, the tumor exhibited classic features of monophasic synovial sarcoma. The diagnosis was confirmed by the presence of SS18 gene rearrangement and identification of SS18-SSX1 fusion transcript. Unfortunately, a relapsing mass was detected 11 months following the surgery. To the best of our knowledge, the current case is the first published example developing in pediatric population.