Epithelioid angiosarcoma of the scalp: An advanced and rare presentation

Abstract

Epithelioid angiosarcoma (EA) is a rare, aggressive vascular malignancy that often presents with nonspecific cutaneous findings, leading to delayed diagnosis. We report the case of a 73-year-old man with a persistent, painful lesion on the vertex and parietal scalp that failed to respond to multiple courses of antibiotics and topical therapies for presumed infectious and inflammatory dermatoses. Examination revealed crusted erosions and plaques with a nodular component, initially mimicking chronic inflammatory skin disease or a potentially neoplastic entity. Histopathologic evaluation of a punch biopsy demonstrated sheets of atypical epithelioid cells with marked nuclear pleomorphism and frequent mitoses. Immunohistochemical staining confirmed endothelial origin with diffuse positivity for ERG and CD31, while excluding squamous, melanocytic, lymphoid, and epithelial neoplasms. These findings established the diagnosis of EA. The patient was referred for oncology evaluation and initiated on radiation therapy. Cutaneous EA most commonly affects elderly White men and arises on the scalp or face. Prognosis remains poor, with 5-year survival rates below 15% because of high recurrence and early metastatic potential. This case underscores the importance of maintaining a high index of suspicion for EA in nonhealing scalp lesions and highlights the central role of histopathology and immunohistochemistry in establishing the diagnosis.