An unusual presentation of angiomatoid fibrous histiocytoma as a perineo-scrotal swelling

Angiomatoid fibrous histiocytoma (AFH) is one of the spindle cell neoplasms having fibroblast and myofibroblast origin. The perineoscrotal region is an uncommon site for such tumors and never reported in the literature. We present a 31-year-old gentleman presented with a painless, rapidly growing scrotal swelling for 2 months duration. Guided core needle biopsy demonstrated spindle cell neoplasm; regional magnetic resonance imaging (MRI) revealed a large mass extending from the perineum to inter-testicular space within the scrotal sac. After staging evaluation, he underwent en bloc resection of the tumor. Histopathology showed features of AFH which is supported by immunohistostains positivity to smooth muscle actin, clusters differentiation (CD) 68, CD99, and desmin. Despite any adjuvant treatment, he remained disease-free on follow-up MRI for 4 years. AFH can also have an unusual origin from perineoscrotum, and it grows rapidly and has a good prognosis.