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

Abstract

Abstract: 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.