Oculocutaneous oncocytic tumors (OCOTs) are uncommon neoplasms that have been reported only rarely in the dermatopathology literature and whose immunophenotypic profile has not been well characterized. The clinical, histologic, and immunophenotypic features of 2 cases seen by the authors were assessed, and relevant publications in the literature were reviewed. Both patients with OCOTs were adult women with gradually enlarging, asymptomatic lesions involving the caruncle; they were locally excised. Histologically, the tumors were well-circumscribed nodules comprised of large oxyphilic cells arranged in confluent sheets and forming glandular spaces with secretory material. Microcystic areas and sparse intermingled goblet-cells were also apparent. Nuclear atypia and infiltrative growth were absent. Mitotic activity was absent in one case; a single mitotic figure was identified in the other. Immunostains demonstrated uniform expression of pankeratin and mitochondrial antigens. Both neoplasms were also labeled for markers associated with cutaneous adnexal, lacrimal, and minor salivary glandular tissue, including alpha-1-antitrypsin, gross cystic disease fluid protein-15, carcinoembryonic antigen, lysozyme and MUC1; each case expressed 4 of the 5 substances. Some cells expressed cytokeratins 5/6 and p63 consistent with the presence of basal-type differentiation in a subset of cells. No definite evidence of myoepithelial differentiation was demonstrated, as stains for smooth muscle actin, muscle-specific actin, and S100 protein were negative. Estrogen and progesterone receptor proteins were absent; strong cytoplasmic immunoreactivity for androgen receptor protein was evident, but nuclear staining was absent. The authors conclude that OCOTs show glandular differentiation. A review of the literature disclosed that none of these lesions arising in the caruncle behaved aggressively, in contrast to occasional tumors in other oculocutaneous sites.

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