Composite hemangioendothelioma: An unusual presentation of a rare vascular tumor

Composite hemangioendothelioma (CHE) has been recently recognized as a low- to intermediate-grade vascular tumor. CHEs are rare vascular tumors that are clinically similar to more common vascular tumors but histologically exhibit a composite of hemangioendothelioma variants. We report the first case of a CHE on the scalp and the fifth case to show findings supportive of regional metastasis.

Our patient had a multilobulated, violaceous scalp nodule, which on histologic examination revealed epithelioid, retiform, and spindle-cell components and rare foci of intermediate-grade mitotic activity consistent with CHE.

Imaging studies were performed and revealed an abnormal uptake of contrast media in a posterior neck nodule that, when examined via fine-needle aspiration, revealed clumps of atypical cells.

This unique case presentation is representative of the variability seen in the presentation of CHE and highlights the importance of considering CHE on the clinical and histologic differential of vascular tumors.