Primary salivary neuroendocrine neoplasms are extremely rare, with only 1 case of carcinoid tumor and 7 cases of primary large-cell neuroendocrine carcinomas having been reported. Salivary small-cell neuroendocrine carcinomas are more frequent, accounting for less than 2% of all salivary malignancies. In addition to a literature review on the subject, the authors describe a rare case of malignant intermediate-grade neuroendocrine tumor of the submandibular gland.
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