A Rare Case With a Review of Cutaneous Composite Hemangioendothelioma and the Role of Neuroendocrine Markers

Composite hemangioendothelioma comprises permutations of different histological patterns few of which have been found to have specific genetic alteration and immunohistochemical expression. It comprises retiform or epithelioid hemangioendothelioma-like areas, with a variable proportion of hemangioma or low-grade angiosarcoma-like areas. It was found to express neuroendocrine markers and was seen to have a worse prognosis in recurrence or distant metastasis. A 29-year-old woman presented with a lesion of 22 cm in size in her right leg. Biopsy and wide local excision showed features of composite hemangioendothelioma. This is a recurrent lesion after initial resection 2 years back, along with a cutaneous metastasis in the thigh. We report this rare case with a literature review, highlighting the importance of uncommon histomorphology and neuroendocrine marker expression in predicting local recurrence and cutaneous metastasis.