Cutaneous composite hemangioendothelioma with satellitosis and lymph node metastases

The term hemangioendothelioma has been used in recent years to name a heterogeneous group of vascular neoplasms, intermediate in both biological behavior and histopathologic appearance between benign tumors (hemangiomas) and frankly malignant tumors (angiosarcomas). Thus, within the spectrum of hemangioendothelioma have been successively included epithelioid hemangioendothelioma, spindle cell hemangioendothelioma, retiform hemangioendothelioma, kaposiform hemangioendothelioma, polymorphous hemagioendothelioma of the lymph nodes, papillary intralymphatic angioendothelioma (PILA) and composite hemangioendothelioma. The latter is a vascular neoplasm showing varying combinations of benign, low-grade malignant and malignant vascular components. We herein report a case of composite hemangioendothelioma showing a combination of retiform hemangioendothelioma, epithelioid hemangioendothelioma, spindle cell hemangioma and PILA. The neoplasm showed a more aggressive behavior than other reported cases of composite hemangioendothelioma and it developed satellitosis and metastases to the inguinal lymph nodes. Neoplastic cells expressed immunoreactivity for Prox-1, supporting a lymphatic line of differentiation.