Update on Neuroendocrine Carcinomas of the Larynx

Carolina Strosberg, MD; Alfio Ferlito, MD, DLO, DPath, FRCSEd ad hominem, FRCS (Eng, Glasg, Ir) ad eundem, FDSRCS ad eundem, FACS, FHKCORL, FRCPath, FASCP, IFCAP; Asterios Triantafyllou, PhD (Lond), FRCPath; Douglas R. Gnepp, MD; Justin A. Bishop, MD; Henrik Hellquist, MD, PhD, SAPath (Stockh), FRCPath; Primoz Strojan, MD, PhD; Stefan M. Willems, MD, PhD; Göran Stenman, DMD, PhD, FRCPath; Alessandra Rinaldo, MD, FRCSEd ad hominem, FRCS (Eng, Ir) ad eundem, FRCSGlasg, FACS; Juan C. Hernandez-Prera, MD


Am J Clin Pathol. 2019;152(6):686-700. 

In This Article


The current classification of neuroendocrine carcinomas of the larynx includes well-differentiated, moderately differentiated, and poorly differentiated neuroendocrine carcinomas (small cell and large cell types). There are major clinical and prognostic differences among the various subtypes and therefore the implications of correctly classifying these carcinomas are critical. To date, there is no evidence to support an etiologic role of HPV in these tumors. In the majority of cases, the diagnosis of neuroendocrine carcinomas can be made by incorporating light microscopy features, mitotic count, and evidence of neuroendocrine differentiation by immunohistochemistry. Nevertheless, tumors that have similar morphologic characteristics, including paraganglioma, medullary thyroid carcinoma, adenoid cystic carcinoma, and variants of squamous cell carcinoma, should be considered in the diagnostic workup of these lesions. The use of an algorithm, such as the one proposed here, is instrumental in assuring the exclusion of mimickers.