Epithelioid Hemangioendothelioma Presenting in the Skin

Abstract

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

Epitheloid hemangio-endothelioma of right aryepiglottic fold: a rare case report with review of literature

“…EHE has been reported in the liver, lung, gastrointestinal tract, head, neck, central nervous system, heart, bone, and skin. [5][6][7][8][9][10][11][12][13] Liver and lung lesions are more common in females, whereas tumors of the bone and soft tissues have an equal sex distribution.…”

Primary pleural epithelioid haemangioendothelioma with metastases to the skin. A case report and literature review

“…1-3 It is extremely rare for EHE to arise from the skin as a primary tumour. 4 We describe the case of a 51 year old man who developed multiple metastatic cutaneous lesions of EHE from a pleural primary location. He had a history of exposure to asbestos.…”

Cutaneous Soft Tissue Tumors That Make You Say, “Oh $*&%!”

“…EHE typically presents in adults but with a relatively nondescript clinical appearance, usually a skin-colored nodule that lacks the 30,31 This vasculocentric pattern, however, is less frequently encountered in cutaneous tumors. 32 The most characteristic feature is the cord-like arrangement of the epithelioid endothelial cells that are embedded within a myxohyaline stroma. In some cases the cord-like morphology is difficult to appreciate causing potential confusion with ES.…”