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Case Reports in Otolaryngology
Volume 2016, Article ID 6971491, 5 pages
http://dx.doi.org/10.1155/2016/6971491
Case Report

Primary Neuroendocrine Tumor of the Parotid Gland: A Case Report and a Comprehensive Review of a Rare Entity

1Department of Medical Oncology, Ramón y Cajal Hospital, University of Alcalá de Henares, Carretera Colmenar Viejo, Km 9.1, 28034 Madrid, Spain
2Department of Pathology, Ramón y Cajal Hospital, University of Alcalá de Henares, Carretera Colmenar Viejo, Km 9.1, 28034 Madrid, Spain
3Department of Otorhinolaryngology, Ramón y Cajal Hospital, University of Alcalá de Henares, Carretera Colmenar Viejo, Km 9.1, 28034 Madrid, Spain
4Department of Endocrinology, Ramón y Cajal Hospital, University of Alcalá de Henares, Carretera Colmenar Viejo, Km 9.1, 28034 Madrid, Spain

Received 1 May 2016; Accepted 25 July 2016

Academic Editor: Yorihisa Orita

Copyright © 2016 Olga Martínez-Sáez et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Neuroendocrine tumors (NETs) comprise a heterogeneous group of malignancies from cells derived from the neural crest with neuroendocrine differentiation. Despite the differences in the site of origin, nomenclature, biological behavior, and functional status, NETs share certain ultrastructural and immunohistochemical features. NETs are relative rare tumors with an annual incidence of 5.76 new cases per 100.000 inhabitants and they usually appear in the gastrointestinal tract or in the pulmonary system. Head and neck NETs are uncommon with limited information regarding frequency, most of them showing small cell carcinoma features. NETs that arise from the salivary glands are exceedingly rare. Regardless of their low frequency, it is imperative to accurately differentiate these tumors from the much more common squamous cell carcinomas and from metastasis from another primary tumor due to the completely different therapeutic approaches and prognosis. The diagnosis is based on the recognition of the typical neuroendocrine architecture and immunohistochemical staining and on an exhaustive work-up. Hereby, we report a case of a moderately differentiated NET of the parotid gland that was treated with a complete parotidectomy. We summarize the clues that led to the final diagnosis and major strategies that were employed to manage the patient. We also perform a comprehensive review of the scarce available literature on this topic.