PT - JOURNAL ARTICLE AU - Farnan, Jessica AU - Morrison, Ellen AU - Hennessey, Derek Barrry TI - Para-spermatic cord proximal-type epithelioid sarcoma AID - 10.1136/bcr-2019-232385 DP - 2021 Jan 01 TA - BMJ Case Reports PG - e232385 VI - 14 IP - 1 4099 - http://casereports.bmj.com/content/14/1/e232385.short 4100 - http://casereports.bmj.com/content/14/1/e232385.full SO - BMJ Case Reports2021 Jan 01; 14 AB - Proximal-type epithelioid sarcoma is an ultra-rare, high-grade soft tissue malignancy usually presenting as a deep-seated painless mass in the proximal extremities. Most patients are diagnosed as young adults, between 20 and 40 years of age. Perineal and genital masses do occur but are extremely rare and represent a challenging tumour to diagnose and treat. Early radical excision is recommended due to its aggressive behaviour and poor prognosis. Median overall survival from initial diagnosis is 30 months. We present the case of a 22-year-old man with a left groin proximal-type epithelioid sarcoma who is sadly deceased 12 months after initial presentation despite early surgical excision, completion of both first-line and palliative chemotherapy, and palliative radiotherapy.