Oncocytic carcinoma (OCA) is rare, and although its occurrence in the lacrimal gland has been documented, clinical and histologic data are lacking. The authors report the first case of OCA of the lacrimal gland histologically proven in a patient with neurofibromatosis (NF). An 81-year-old man affected by type 1 NF presented with a 2-year history of left proptosis and visual loss. Orbital CT scan showed a mass in the lacrimal gland fossa infiltrating the globe, lateral rectus muscle, and bone. An incisional biopsy revealed a primary high-grade malignancy, and the patient underwent orbital exenteration followed by postoperative adjunctive radiotherapy. Six months after the operation, the patient was alive, without evidence of recurrent or metastatic disease. OCA is a high-grade neoplasm with infiltrative growth pattern and tendency to recur and metastasize. Its occurrence in association with NF has not been documented before, but clinicians should be aware of this possibility when evaluating proptosis in NF patients. Radical surgical excision, followed by adjunctive radiotherapy, is considered the treatment of choice, but the prognosis remains guarded.
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