Testicular and paratesticular rhabdomyosarcomas (RMSs) in children are uncommon tumors. Histologically though embryonal RMS is common, the spindle cell variant is considered rare. Paratesticular RMS presents in children and adolescents with a unilateral, painless scrotal swelling or mass above the testis. We report the case of a 15-year-old boy, with a left paratesticular mass who underwent high inguinal orchidectomy. Histopathological examination of the specimen demonstrated spindle cell RMS (SC-RMS). Because of its morphological resemblance to spindle cell neoplasms such as leiomyosarcomas and fibrosarcomas, SC-RMS may pose diagnostic difficulties for the pathologist. This problem can be overcome by a careful search for rhabdomyoblasts in sections and immunohistochemistry for myogenin. We are reporting this case as paratesticular RMS itself is uncommon, and the spindle cell variant of embryonal RMS is all the more rare. There are lacunae in our knowledge about their presentation, diagnosis, response to treatment, and cure.
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