Myxoid Dermatofibrosarcoma Protuberans of the Scrotum: Rare Tumor in an Uncommon Location

BACKGROUND: Dermatofibrosarcoma protuberans (DFSP) is an uncommon tumor that involves the skin with a low metastatic rate; however, this tumor has a tendency to recur locally. Myxoid DFSP is a rare variant, with a few cases mentioned in the literature, and may present diagnostic difficulties on histopathological examination. CASE PRESENTATION: We present a 9-year-old male with a history of a left scrotal mass for 3 years. An enhanced computed tomography (CT) scan of the pelvis showed a heterogeneously enhancing mass in the scrotum with extension into both inguinal regions. Histology showed spindle-shaped cells forming a storiform pattern in the hypercellular area and myxoid stroma with prominent thin-walled vessels. Immunohistochemically, the cluster of differentiation 34 (CD34) was positive. The mass was removed by surgical excision with negative resection edges, and then adjuvant radiotherapy of 60 Gy was given. The patient had a good outcome with treatment and was doing well on follow-up. CONCLUSION: This variant of sarcoma is rare and often presents a diagnostic challenge. Early detection is crucial to prevent both undertreatment and overtreatment.