Neuroendocrine neoplasms (NEN) arise in virtually every organ. The embryologic development of NEN has been the subject of controversy whether there is or is not a common neural crest origin. Under the premise of a common neural crest origin, the existence of a common biochemical pathway in neuroendocrine cells consisting of uptake and decarboxylation of amine precursors was postulated allowing for these cells to be identified histochemically and resulting in the use of the acronym APUD (Amine Precursor Uptake and Decarboxylation) to describe the cells in this system. Subsequent studies debunked a common neural crest origin and the classification Dispersed Neuroendocrine Cell System was adopted for NEN. The classification of NEN has undergone an evolution whereby current schemes irrespective of site of origin have suggested utilizing nomenclature established for gastrointestinal and pancreatic NEN. Aside from the gastrointestinal tract, pancreas and lung, the head and neck region is among the more common sites of occurrence for NEN and within this region neuroendocrine carcinoma is most common type of site specific NEN. This talk will focus on neuroendocrine carcinomas of the head and neck reviewing their clinicopathologic features and discussing their inclusion within a uniform classification framework proposed for other site specific NEN.
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