Neuroendocrine carcinoma (NEC) is uncommon in the head and neck and rare in the salivary glands. In the latter location, it may manifest as pain or a palpable mass. These tumors can be quite aggressive, with a tendency toward recurrence and regional and distant metastasis. Because of the limited number of reported cases in the literature, no reliable treatment has been established. We describe the case of a 79-year-old man who presented with a persistent submandibular mass that was suspicious for a malignancy on imaging. Following surgical resection and histopathologic analysis, a diagnosis of poorly differentiated small-cell NEC was made. The patient was further treated with postoperative chemotherapy, and he exhibited no evidence of recurrence or metastasis on follow-up.
Neuroendocrine tumors include both neuroendocrine carcinoma (NEC), which is of epithelial origin, and nonepithelial neural tumors, such as olfactory neuroblastoma and paraganglioma. The paraganglion system functions in fetal life to serve the role that the adrenal medulla will largely assume after it develops-that is, to secrete and store catecholamines via chromaffin cells, which are neuroectodermally derived.