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Epithelioid hemangioendothelioma with multiple site involvement. Literature review and observations
Author(s) -
Bollinger Barbara K.,
Laskin William B.,
Knight Clement B.
Publication year - 1994
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(19940201)73:3<610::aid-cncr2820730318>3.0.co;2-3
Subject(s) - pathology , medicine , epithelioid hemangioendothelioma , hemangioendothelioma , cytokeratin , cd34 , immunohistochemistry , epithelioid cell , biology , stem cell , genetics
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.