Challenges in the Diagnosis of Pediatric Spindle Cell/Sclerosing Rhabdomyosarcoma

Rhabdomyosarcoma (RMS) is the most common pediatric soft tissue sarcoma, representing approximately 40% of all pediatric soft tissue sarcomas. The spindle cell/sclerosing subtype of RMS (SSRMS) accounts for roughly 5% to 10% of all cases of adult and pediatric RMS. Historically, SSRMS were described as paratesticular tumors with an excellent outcome. However, more recent studies have identified unique molecular subgroups of SSRMS, including those with MYOD1 mutations or VGLL2/NCOA2 fusions, which have widely disparate outcomes. The goal of this article is to better describe the biological heterogeneity of SSRMS, which may allow the pathologist to provide important prognostic information.