Abstract
<h3>Objectives</h3> To summarize available data published in English literature reporting oral and maxillofacial neuroendocrine carcinoma in a systematic review. <h3>Study Design</h3> An electronic search was undertaken in April 2021 in 4 databases. Publications having enough clinical and histopathologic data, with at least 1 neuroendocrine marker to confirm the diagnosis, were included. Cases originating in the oropharynx, including the base of the tongue and tonsils, were excluded. Data were evaluated descriptively. <h3>Results</h3> Twenty-one articles were retrieved (25 cases). The mean age of the individuals was 58.8 (±15.5) years, with a slight male predilection. Symptomatology was present in 45% of informed cases. Regarding clinical presentation, nodules (75%) located in the tongue (25%) with a mean size of 3.4 cm were most often reported. Concomitant metastasis was identified in 6 individuals. Histopathologically, the neoplasms were majority small cells type. Radical surgery was the treatment of choice in almost cases, with or without adjuvant therapy. The mean follow-up was 21.4 (±22.1) months, and only 3 patients developed recurrences. Nine individuals died from the disease (45%). <h3>Conclusions</h3> Neuroendocrine carcinomas are rare lesions in the oral and maxillofacial region. Our findings demonstrated that the long-term prognosis of this lesion remains poor.
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