Neuroendokrine Neoplasien der Kopf-Hals-Region

Abstract

Common to all neuroendocrine neoplasms (NENs), irrespective of their site of origin, is the expression of synaptophysin and chromogranin A. NENs of the head and neck region derive either from epithelial or neural/neuroectodermal tissues. The epithelial-type NENs express cytokeratins and include the well-differentiated typical and atypical carcinoids (also called low- and intermediate-grade neuroendocrine carcinomas by WHO), the poorly differentiated high-grade neuroendocrine carcinomas of small and large cell type and the mixed neuroendocrine-nonneuroendocrine neoplasms. The neural-neuroectodermal-type NENs comprise olfactory neuroblastoma and paraganglioma, each of them with distinct clinicopathological characteristics. Olfactory neuroblastomas show a spectrum of histologic differentiation and are prognostically classified by Hyams grading. Paragangliomas often occur multiple and show a familial background. Most head and neck NENs occur in the upper respiratory system. Their diagnosis follows the general guidelines for NENs, focusing on immunohistochemical profiling. Molecular examinations are so far only required in individual cases.

Seltene Erkrankungen der Nase, der Nasennebenhöhlen und der vorderen Schädelbasis

“…den Kopf-Hals-Paraganglien [303]. Entsprechend der WHO Klassifikation werden neuroendokrine Tumore als Neoplasie Grad I (früher low-grade neuroendokriner Tumor oder typisches Karzinoid), Neoplasie Grad II (früher intermediärer neuroendokriner Tumor oder atypisches Karzinoid) und Neoplasie Grad III, kleinzelliges Karzinom (früher high-grade Neuroendokriner Tumor oder kleinzelliges Karzinom) sowie Neoplasie Grad III, großzelliges Karzinom (früher high-grade neuroendokriner Tumor oder großzelliges neuroendokrines Karzinom) eingeteilt [304].…”

“…Sinunasale neurendokrine kleinzellige und großzellige Karzinome weisen eine Zytokeratin-Expres sion auf, was eine Differenzierung z. B. gegenüber dem Olfaktoriusneuroblastom ermöglicht [304].…”

“…Neuroendocrine carcinoid (typical carcinoid, grade I neoplasm) More than 90 % of neuroendocrine carcinoids manifest in the larynx (supraglottic). Sinonasal manifestations occur more rarely and lead to well delimited, submucous and often polypoid tumors [304]. Especially poorly differentiated neuroendocrine carcinomas are found in the nasal cavity, the paranasal sinuses, or at the skull base.…”

Small Cell Neuroendocrine of the Head and Neck: A Rare Presentation and Review of the Literature

“…4 Regardless of the site of origin, cells from NENs tend to express synaptophysin and chromogranin A. 5 Neuroendocrine neoplasms of the head and neck tend to be rare; with the most common tumor histology of this anatomical region being squamous cell carcinoma, arising from squamous cells that line the mucosal surface. Recently, the World Health Organization published classification guidelines for head and neck tumors, 6 where NENs are divided into 2 categories: well-differentiated neuroendocrine tumors (NETs) and poorly differentiated neuroendocrine carcinomas (NECs).…”