Spindle cell hemangioendothelioma

Abstract

Prior to the 1980s, the term hemangioendothelioma (HE) loosely applied to a spectrum of vascular tumors ranging from benign tumors, such as capillary hemangiomas, to fully malignant angiosarcomas. In the early 1980s, the term epithelioid HE was used to describe a heterogeneous group of vascular tumors with an intermediate clinical course between hemangiomas and conventional angiosarcomas, thereby bringing to notice the borderline nature of these tumors. Since then, HEs have become a distinct entity, being further classified into spindle cell HE, retiform HE, kaposiform HE, and polymorphous HE. We present a case of spindle cell HE that began as an interdigital nodule and progressively became multifocal to involve the entire right upper extremity and chest wall, despite multiple surgical excisions, resulting in severe ongoing disfigurement of the patient.