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WHO Classification of Tumours
Neuroendocrine carcinoma, NOS
Esophagus


Definition

Neuroendocrine carcinoma (NEC) is a poorly differentiated, high-grade malignant neoplasm composed of small cells or large to intermediate cells, sometimes with organoid features resembling NET, diffusely expressing the general markers of neuroendocrine differentiation (diffuse expression of synaptophysin; faint or focal staining for chromogranin A), with marked nuclear atypia, multifocal necrosis and a high number of mitoses (> 20 per 10 HPF); high grade (G3) defined according to the proliferation fraction and histology. This definition refers to neoplasms previously classified as small cell carcinoma, large cell (neuro)endocrine carcinoma, or poorly differentiated (neuro)endocrine carcinoma.

Neuroendocrine tumours of the oesophagus are exceedingly rare. Most of the reported cases are neuroendocrine carcinomas (NEC) or mixed adenoneuroendocrine carcinoma (MANEC) , the latter with either an adenocarcinoma or a squamous cell carcinoma component

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Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



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> Histological subtypes
Large cell neuroendocrine carcinoma
Small cell neuroendocrine carcinoma

Commentary: Neuroendocrine tumours of the digestive system