This review aimed to critically analyse data pertaining to the clinical presentation and treatment of neuroendocrine carcinomas of the larynx.

A PubMed search was performed using the term ‘neuroendocrine carcinoma’. English-language articles on neuroendocrine carcinoma of the larynx were reviewed in detail.

While many historical classifications have been proposed, in contemporary practice these tumours are sub-classified into four subtypes: carcinoid, atypical carcinoid, small cell neuroendocrine carcinoma and large cell neuroendocrine carcinoma. These tumours exhibit a wide range of biological behaviour, ranging from the extremely aggressive nature of small and large cell neuroendocrine carcinomas, which usually have a fatal prognosis, to the less aggressive course of carcinoid tumours. In small and large cell neuroendocrine carcinomas, a combination of irradiation and chemotherapy is indicated, while carcinoid and atypical carcinoid tumour management entails conservation surgery.

We present the first report of a case of neuroendocrine carcinoma of the paranasal sinuses treated successfully with radiotherapy alone.

A case report and literature review are presented.

Fewer than 50 cases of paranasal sinus neuroendocrine carcinoma have been reported. We present an 82-year-old man referred with recurrent epistaxis. He was investigated by biopsy, computed tomography and magnetic resonance imaging, and was found to have a rare neuroendocrine carcinoma. He declined any surgery or chemotherapy but consented to radiotherapy. Thirty months later, he remained clinically free from cancer.

There is no consensus for the management of paranasal sinus neuroendocrine carcinoma. Most cases are treated with surgery with or without chemoradiotherapy. This case shows that radiotherapy alone may be a viable treatment option for some cases.