Epithelioid hemangioendothelioma with multiple site involvement. Literature review and observations

• Published in: Cancer 1994-02, Vol.73 (3), p.610-615
• Main Authors: Bollinger, Barbara K., Laskin, William B., Knight, Clement B.
• Format: Article
• Language: English
• Subjects: Adult; Antigens, CD - analysis; Antigens, CD34; Biological and medical sciences; Blood and lymphatic vessels; Cardiology. Vascular system; Diseases of the peripheral vessels. Diseases of the vena cava. Miscellaneous; epithelioid hemangioendothelioma; Factor VIII‐related antigen; Female; Hemangioendothelioma, Epithelioid - diagnostic imaging; Hemangioendothelioma, Epithelioid - pathology; Hemangioendothelioma, Epithelioid - ultrastructure; Humans; Immunohistochemistry; intracytoplasmic lumina; Keratins - analysis; Lung Neoplasms - pathology; Medical sciences; multifocal vascular tumors; Neoplasms, Second Primary - pathology; Neoplasms, Second Primary - ultrastructure; Receptors, Estrogen - analysis; Receptors, Progesterone - analysis; Skin Neoplasms - pathology; Tomography, X-Ray Computed; von Willebrand Factor - analysis
• ISSN: 0008-543X; 1097-0142
• DOI: 10.1002/1097-0142(19940201)73:3<610::AID-CNCR2820730318>3.0.CO;2-3

This report is a case of epithelioid hemangioendothelioma presenting as multiple lytic lesions of the ilium with radiographic findings of diffuse, bilateral lung involvement and biopsy‐proven scalp involvement. Histologically, the tumor within bone and skin exhibited cords and nests of plump, epithelioid‐appearing cells exhibiting rudimentary vascular differentiation within a myxohyaline stroma. Aggressive histologic features were not present. Immunohistochemical reactivity for Factor VIII‐related antigen, Q‐bend 10 (CD34), and cytokeratin were demonstrated. Ultrastructural studies revealed abundant intermediate cytoplasmic filaments, pinocytotic vacuoles, and Weibel‐Palade bodies. The concurrent bone, skin, and lung involvement, low‐grade histologic type, and female sex of the patient aroused speculation about the role of hormones in the development and possible treatment of the tumor, but estrogen and progesterone receptors were not detected. Despite intense combination chemotherapy, the patient died of widely metastatic disease. This report demonstrates the aggressive potential of histologically low‐grade epithelioid hemangioendothelioma and the need for a thorough evaluation for metastases.