Spindle Cell Hemangioendothelioma

background . Spindle cell hemangioendothelioma lesions are uncommon, affect a wide age range in both sexes, and show a predilection for skin and subcutaneous tissue of the extremities. objective . To present three cases of spindle cell hemangioendothelioma and review the literature. methods . Three cases of spindle cell hemangioendothelioma are presented. results . Two of our cases first presented very early in life and progressed by local recurrences over many years. None of our cases showed evidence of metastases, but the lesions exhibited local aggressive and invasive behavior. Histologically, the lesions consist of alternating areas of dilated, thin walled cavernous vascular spaces and solid areas composed predominantly of spindle cells and clusters of epithelioid endothelial cells with intracytoplasmic vacuoles. That spindle cell hemangioendothelioma is a non‐neoplastic lesion and not a neoplasm of borderline malignancy is suggested by the following observations from our cases: the repeated presence of organized intravascular thrombi in all cases, the early clinical presentation in two cases, and the presence of some degree of vascular malformation at the periphery of lesions. conclusion . Both clinicians and pathologists should be aware of the existence of this lesion in order to diagnose and treat an affected patient correctly.