Primary cutaneous scrotal angiosarcoma; a case report and literature review

Abstract Background Cutaneous angiosarcoma is a malignant, aggressive, vascular tumor that usually arises in association with prior radiation and chronic lymphedema. A primary cutaneous angiosarcoma originating in the scrotum is extremely rare, with few previous cases reported in the literature. Case presentation We report a 43-year-old man who presented with a unilateral scrotal lesion. The diagnosis of cutaneous angiosarcoma was confirmed by microscopic examination which revealed multiple vascular spaces lined by atypical cells, wide areas of necrosis were also seen. Immunohistochemistry showed strong positivity for ERG, focal positivity for CD31, and negative staining for CK, CD34, desmin, S100, SMA, and HHV8. Excision of the right hemiscrotum was performed with no evidence of local recurrence or metastasis in the following eleven months. Conclusion This is the first reported case of primary scrotal cutaneous angiosarcoma without identifiable risk factors. Despite its rarity, cutaneous symptoms on the scrotum should be investigated for the potential of a scrotal angiosarcoma.