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Case ReportsAbstract
Epithelioid hemangioendothelioma (EHE) is a rare vascular neoplasm with variable clinical behavior. Though more commonly found in visceral organs such as the liver or lungs, cutaneous EHE (c-EHE) represents an unusual and underrecognized presentation. Histologic and immunophenotypic features are critical for diagnosis, given its potential for local invasion and metastasis.
A 33-year-old African American female presented with a firm, painless, slowly enlarging nodule on the mucosal lower lip. Shave biopsy was performed which revealed a proliferation of epithelioid endothelial cells forming irregular vascular channels with cytologic atypia and lymphocytic infiltrate. Immunohistochemistry was positive for CD31 and D2-40, with a low proliferative index on Ki-67. The findings were diagnostic of cutaneous EHE. The patient was referred for definitive surgical excision by ENT.
This case highlights a rare cutaneous presentation of EHE on the mucosal lip. Accurate diagnosis hinges on recognition of its unique histopathologic and immunohistochemical profile. Excision remains the standard of care, and multidisciplinary management is key.