High-grade, monophasic synovial sarcoma comprises small, uniform spindle cells forming densely packed sheets demonstrating a fascicular growth pattern and hemangiopericytoid vascularity.
Although monophasic fibrous synovial sarcoma resembles fibrosarcoma, synovial sarcoma shows a hemangiopericytoid vascularity that is not found in fibrosarcoma. A distinctive profile of expression of immunohistochemical markers and specific molecular abnormalities is diagnostic of synovial sarcoma and not present in fibrosarcoma.
Although hemangiopericytoma shares with monophasic fibrous synovial sarcoma a predilection for the lower extremities and some morphologic features, it is thought to arise from pericytic smooth muscle and glomus cell lines, whereas synovial sarcoma is biphasic (epithelial and mesenchymal). Only hemangiomas express CD34.
Despite similarities including uniform small cell population, hemangiopericytoid vascularity, pseudorosettes, zones of necrosis, and the expression of CD99, Ewing sarcoma/PNET and synovial sarcoma have distinctive molecular characteristics, such as a consistent translocation t[11,22] [q24,q12] with fusion of Fli-1 and EWS genes resulting in the Fli-1/EWS chimeric transcript (Ewing sarcoma/PNET) and a chromosomal translocation with fusion of SYT (on chromosome 18) and SSX1 or SSX2 (on chromosome X) resulting in the fusion transcript SYT/SSX1 or SYT/SSX2 (synovial sarcoma).
Mesenchymal chondrosarcoma shares with synovial sarcoma the monotonous, uniform, small cell population hemangiopericytoid pattern of vascularity. However, synovial sarcoma shows no foci of cartilage (proven by negative S100).