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. 2014 Mar;8(1):24-32.
doi: 10.1007/s12105-014-0531-y. Epub 2014 Mar 5.

Neuroendocrine neoplasms of the head and neck: some suggestions for the new WHO classification of head and neck tumors

Bin Xu  1 , Runjan Chetty, Bayardo Perez-Ordoñez
Affiliations

Neuroendocrine neoplasms of the head and neck: some suggestions for the new WHO classification of head and neck tumors

Bin Xu et al. Head Neck Pathol. 2014 Mar.

Abstract

As knowledge and understanding in pathology evolve, classifications and nomenclature also change to reflect those advances. The 2005 World Health Organization Classification of Head and Neck Tumours was a significant step towards diagnostic standardization of head and neck neuroendocrine carcinomas; however, in the last 10 years there have been new data supporting the recognition of "large cell neuroendocrine carcinoma" as a distinctive high grade carcinoma in the head and neck, a lesion not included in the 2005 Classification. In addition, the terms "middle ear adenoma" and "carcinoid tumor of middle ear" are still widely used to describe a neoplasm that is neither a pure adenoma nor a carcinoid tumor but a lesion with variable mixed exocrine and endocrine differentiation. Largely using the diagnostic criteria of the WHO classification of neuroendocrine carcinomas of the lung, we propose the terms "neuroendocrine carcinoma, grade 1"; "neuroendocrine carcinoma, grade 2"; "neuroendocrine carcinoma, grade 3, large cell type"; and "neuroendocrine carcinoma, grade 3, small cell type" for the classification of neuroendocrine carcinomas of the head and neck in a future WHO classification. In addition, we also proposed the term "mixed epithelial neuroendocrine tumor" of the middle ear as an alternative for "middle ear adenoma" and "carcinoid tumor of the middle ear".

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Figures

Fig. 1
Fig. 1
Neuroendocrine carcinoma, grade 1. Small submucosal NEC of larynx with solid and nested architecture (a). The tumor cells have eosinophilic cytoplasm with coarse but evenly dispersed chromatin. Despite the variation in nuclear size, this NEC displayed no mitotic activity thus qualifying for a NEC, grade 1 (b)
Fig. 2
Fig. 2
Neuroendocrine carcinoma grade 2 of the nasopharynx showing insular and trabecular patterns (a). The tumor is composed of cuboidal and polygonal cells with eosinophilic cytoplasm and nuclei displaying coarse chromatin and occasional mitotic figures (b). Laryngeal NEC grade 2 with central necrosis (c). Grade 2 NEC of larynx with high Ki-67 index. The prognostic value of Ki-index is currently unknown in NECs of the head and neck (d)
Fig. 3
Fig. 3
Neuroendocrine carcinoma grade 3, large cell type. At low power, the tumor is composed of well defined and confluent nests (a). The tumor cells are large with abundant eosinophilic cytoplasm with vesicular nuclei and prominent nucleoli (b). The tumor cells are positive for chromogranin (c)
Fig. 4
Fig. 4
Neuroendocrine carcinoma grade 3, small cell type of the parotid gland (a). The tumor cells are diffusely positive for keratin 20 (b). NEC grade 3, small cell type of oropharynx (c) with diffuse expression of p16 (d). Linear Array confirmed the presence of human papillomavirus-16 (HPV16) in this tumor
Fig. 5
Fig. 5
Mixed epithelial neuroendocrine tumor (MENET) of the middle ear showing prominent glandular and trabecular architecture (a). Keratin 7 in MENET only highlights cells with glandular differentiation. Neuroendocrine cells are negative (b). Only isolated cells with neuroendocrine differentiation are stained with chromogranin in this MENET (c). More intense and diffuse staining of neuroendocrine cells with human pancreatic polypeptide in this example of MENET (d)
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References

    1. Rindi G, Arnold R, Bosman FT, et al. et al. Nomenclature and classification of neuroendocrine neoplams of the digestive system. In: Bosman FT, Carneiro F, Hruban RH, et al.et al., editors. WHO classification of tumours of the digestive system. Lyon: International Agency for Research on Cancer; 2010. pp. 13–14.
    1. Klimstra DS, Modlin IR, Coppola D, et al. The pathologic classification of neuroendocrine tumors: a review of nomenclature, grading, and staging systems. Pancreas. 2010;39:707–712. doi: 10.1097/MPA.0b013e3181ec124e. - DOI - PubMed
    1. Wenig BM, Hyams VJ, Heffner DK. Moderately differentiated neuroendocrine carcinoma of the larynx: a clinicopathologic study of 54 cases. Cancer. 1988;62:2658–2676. doi: 10.1002/1097-0142(19881215)62:12<2658::AID-CNCR2820621235>3.0.CO;2-M. - DOI - PubMed
    1. Wenig BM, Gnepp DR. The spectrum of neuroendocrine carcinomas of the larynx. Semin Diagn Pathol. 1989;6:329–350. - PubMed
    1. Barnes L, et al. Tumours of the hypopharynx, larynx, and trachea: neuroendocrine tumors. In: Barnes L, Eveson JW, Reichart P, et al., editors. Pathology and genetics head and neck tumours. Lyon: IARC Press; 2005. pp. 135–139.

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