M. Quante et al., EPITHELIOID HEMANGIOENDOTHELIOMA PRESENTING IN THE SKIN - A CLINICOPATHOLOGICAL STUDY OF 8 CASES, The American journal of dermatopathology, 20(6), 1998, pp. 541-546
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.