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Keywords:

  • Neuroendocrine carcinoma;
  • radiotherapy;
  • radiation-induced malignancy;
  • sinonasal tract;
  • carcinoid

Abstract

Objectives/Hypothesis: Neuroendocrine carcinoma (NEC) of the sinonasal tract is rare. In this present study, we report our treatment experience with sinonasal NEC.

Study Design: Retrospective case series.

Methods: A retrospective review of the clinical outcomes and pathology of 18 patients with NEC arising from the sinonasal region.

Results: Ten tumors were primary NEC without previous radiation, and eight tumors were postirradiated NEC occurring within the radiation field for previous nasopharyngeal carcinoma in six patients and tonsillar lymphoma in one and neck metastasis of unknown primary origin in one, with an interval between previous radiotherapy and diagnosis of NEC from 82 to 385 months, with a mean of 197 months. Fifteen tumors were small cell carcinoma, two were atypical carcinoid, and one was typical carcinoid tumor. Fifteen patients underwent surgery with/without postoperative adjuvant chemoradiotherapy. Three patients received induction chemotherapy or primary radiotherapy with further definitive treatment. The 5-year disease-free and overall survival rates of all 18 patients were 56.1% and 62.2%, respectively. In comparing primary NEC with postirradiated NEC, they were similar in age, sex distribution, stage, pathology, and treatment, and the 5-year overall survival rates were 70% and 62.5%, respectively.

Conclusions: In this series, postirradiated NEC is common, which may be the result of there being a large number of long-term nasopharyngeal carcinoma survivors. The prognoses of postirradiated NEC and primary NEC appear to be similar despite the relatively short follow-up period in the postirradiated NEC group.