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Oncocytes are characterized by a remarkable number of mitochondria as demonstrated by electron microscopy. Oncocytomas are very rare tumors that are usually benign and typically occur in the parotid gland. Oncocytic carcinomas are exceedingly rare in the salivary glands. We describe a 69-year-old Japanese man with an oncocytic carcinoma of the submandibular gland. We reviewed 38 articles describing oncocytic carcinoma of the head and neck that included clinicopathologic features. Oncocytic carcinomas appear to arise from benign oncocytomas, or may also arise de novo. In oncocytic carcinoma of the head and neck, the presence of distant, rather than local lymph node, metastasis is the most important prognostic indicator.
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