Paratesticular epithelioid sarcoma in a pediatric patient

Here we describe the first case of paratesticular epithelioid sarcoma in an adolescent male. Paratesticular epithelioid sarcoma is an exceedingly rare entity with an aggressive clinical course and poor prognosis. The patient underwent wide hemiscrotectomy with radical inguinal orchiectomy and adjuvant chemoradiotherapy for inguinal nodal metastasis. Subsequent retroperitoneal lymph node dissection revealed metastasis to the para-aortic lymph nodes. Paratesticular sarcomas can be challenging to diagnose and treat. This report outlines the pathology and management of the first pediatric paratesticular epithelioid sarcoma in the English literature and highlights the need for multidisciplinary collaboration.