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Spindle cell/sclerosing rhabdomyosarcoma (SSRMS) is a clinicopathologically and molecularly heterogeneous disease. Gene fusions have been identified in intraosseous SSRMS, consisting predominantly of  EWSR1 / FUS :: TFCP2  and  MEIS1 :: NCOA2 . The former often follow an aggressive clinical course; there is limited clinical follow-up available for the latter. We report here a new case of the very rare intraosseous SSRMS with  MEIS1 :: NCOA2  gene fusion and include the detailed treatment course and 52 months of clinical follow-up. SSRMS with  MEIS1 :: NCOA2  gene fusion appears biologically distinct from other intraosseous SSRMS, following a course characterized by local recurrence with rare reports of metastasis to date.

Intraosseous Spindle Cell Rhabdomyosarcoma with MEIS1 :: NCOA2 Fusion – Case Report with Substantial Clinical Follow-up and Review of the Literature