EPITHELIOID HEMANGIOENDOTHELIOMA PRESENTING IN THE SKIN - A CLINICOPATHOLOGICAL STUDY OF 8 CASES

Epithelioid hemangioendothelioma arising in the skin is extremely rare , and the majority of documented cases have developed in association w ith an underlying bone tumor. We report eight patients with an age ran ge of 29-84 years (mean 53), who presented with primary cutaneous tumo rs at a variety of sites including the palm, shin, neck, knee, nose, b ack, and penis with a duration of between 6 and 12 months. Histologica lly, all eight cases presented as circumscribed nodules with an overly ing acanthotic epidermis, three showing striking acrosyringeal prolife ration, reminiscent of eccrine syringofibroadenoma. The tumors were co mposed of an admixture of slightly pleomorphic spindle and epithelioid cells with abundant, sharply defined eosinophilic cytoplasm and vesic ular nuclei containing single nucleoli. Mitoses were generally sparse. All tumors showed intracytoplasmic lumina and intraluminal erythrocyt es were occasionally apparent. The tumor cells were embedded in a myxo id or hyaline matrix. In contrast to visceral lesions, a vascular orig in was not evident in any of our cases. The tumor cells variably expre ssed CD31, CD34, factor VIII-Rag, and smooth-muscle actin but not pank eratin or epithelial membrane antigen. Follow-up ranged from 4 months to 3 years. None of the lesions has thus far recurred and there have b een no metastases.