Epithelioid hemangioendothelioma presenting in the skin : A clinicopathologic study of eight cases

摘要: Epithelioid hemangioendothelioma arising in the skin is extremely rare, and majority of documented cases have developed association with an underlying bone tumor. We report eight patients age range 29-84 years (mean 53), who presented primary cutaneous tumors at a variety sites including palm, shin, neck, knee, nose, back, penis duration between 6 12 months. Histologically, all as circumscribed nodules overlying acanthotic epidermis, three showing striking acrosyringeal proliferation, reminiscent eccrine syringofibroadenoma. The were composed admixture slightly pleomorphic spindle epithelioid cells abundant, sharply defined eosinophilic cytoplasm vesicular nuclei containing single nucleoli. Mitoses generally sparse. All showed intracytoplasmic lumina intraluminal erythrocytes occasionally apparent. tumor embedded myxoid or hyaline matrix. In contrast to visceral lesions, vascular origin was not evident any our cases. variably expressed CD31, CD34, factor VIII-Rag, smooth-muscle actin but pankeratin epithelial membrane antigen. Follow-up ranged from 4 months 3 years. None lesions has thus far recurred there been no metastases.