Rapid Malignant Transformation: A Rare Case of Epithelioid Angiosarcoma

Abstract Epithelioid angiosarcoma is an exceedingly rare and highly aggressive vascular malignancy that predominantly affects adults, with pediatric cases being exceptionally uncommon. We report the case of an 8-year-old female from Kashmir who presented with progressive pain and swelling in her right shoulder. Initially diagnosed with infantile hemangioma based on histopathological examination, the patient experienced rapid malignant transformation to epithelioid angiosarcoma within a 3-month period. The clinical course was complicated by spontaneous rupture requiring surgical intervention, with intraoperative findings revealing a large soft tissue mass extending from the deltoid to the scapular region. Subsequent comprehensive histopathological review with immunohistochemical studies confirmed the diagnosis of epithelioid angiosarcoma. The patient was treated with multimodal therapy including surgery, radiotherapy, and chemotherapy with positron emission tomography-computed tomography surveillance. This case demonstrates the diagnostic challenges inherent in pediatric vascular tumors and emphasizes the critical importance of comprehensive histopathological evaluation, vigilant clinical monitoring, and multidisciplinary management approaches to optimize patient outcomes in rare pediatric malignancies.