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Spindle Cell/Sclerosing Rhabdomyosarcoma : Congenital

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The congenital/infantile form of sclerosing/spindle cell rhabdomyosarcoma is characterized by gene fusions involving VGLL2 and NCOA2 or CITED2 genes. Less common are TEAD1-NCOA2 and SRF-NCOA2 fusions.

The tumor is present at birth or develops within 1 year of age, usually on the trunk. It consists of fascicles of low-grade appearing spindle cells that resemble low-grade leiomyosarcoma. Scattered rhabdomyoblasts may be present. It carries an excellent prognosis.

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