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Neuroendocrine tumours are very rare in the nasal cavity, paranasal sinuses or nasopharynx. The recognizable types are typical carcinoid, atypical carcinoid and small cell carcinoma neuroendocrine type. It is unclear whether large cell neuroendocrine carcinoma that corresponds to the pulmonary counterpart occurs in these sites. There are also rare cases that do not fit these categories, and the diagnostic label "neuroendocrine carcinoma, not otherwise specified" may be applied.

Carcinoid tumour ICD-O codes

Typical carcinoid 8240/3

Atypical carcinoid 8249/3

Typical and atypical carcinoids of the nasal cavity and paranasal sinuses are exceedingly rare, possibly because they are under-reported or have been included under other non-descriptive categories, such as "neuroendocrine carcinoma" {1676,2007,2384,2776}. They are otherwise similar to carcinoids in other sites.

Patients have ranged in age from 13-65 years, and present with nasal obstruction, epistaxis and/or facial pain. Most tumours arise in the nasal cavity but may extend into adjacent sinuses. A patient with two carcinoids - nasal and pulmonary - has been described {2384}. Another individual with the Multiple Endocrine Neoplasia Type I (MEN1) has been reported to have a carcinoid of the sphenoid sinus {2776}. Paucity of cases and lack of significant follow-up preclude definitive statements about the prognosis. The tumours are at least locally aggressive.

Small cell carcinoma, neuroendocrine type (SCCNET)

Definition

Small cell carcinoma, neuroendocrine

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