The poorly differentiated neuroendocrine carcinomas (NECs) of the larynx are rare tumors that comprise of a small cell-type (SCNEC) and a large cell-type (LCNEC). In order to consolidate the current knowledge about their characteristics and management a systematic review of the available literature was performed. The PubMed/Medline and Scopus databases search resulted in 141 articles published between 1972 and 2019, describing 273 patients: 230 cases were of SCNEC histology (84.2%) and 43 cases were LCNECs (15.8%). Comparing both histological entities, patients with LCNECs were older (63.2 vs. 58.7 years, p = 0.036) than SCNEC patients and had more often primary tumor in a supraglottic larynx (79.5 vs. 56.1%, p = 0.039), advanced-stage neck disease (N2-3, 56.8 vs. 40%, p = 0.061), surgery-based treatment (83.7 vs. 51.9%, p < 0.001) and had no radiotherapy (44.2 vs. 29%, p = 0.071). At presentation, systemic metastases were diagnosed in 12.1% of the patients, whereas disease relapse was experienced by two-thirds (65.3%) of those initially staged M0; systemic relapse, alone or in combination with local/regional recurrence, was the most frequent type of failure (in 71.9%). On multivariate analysis, more advanced disease stage and SCNEC histology adversely influenced disease-specific survival. Wide variations in the pattern of care, including radiotherapy doses and chemotherapy regimens, were found among long-term survivors without known disease at ≥ 24 months of follow-up (N = 36). We conclude that the most effective treatment for poorly differentiated NECs has yet to be determined.
Keywords: Large cell; Larynx; Neuroendocrine tumors; Small cell; Systematic review; Therapy.
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