Update on Neuroendocrine Carcinomas of the Larynx

Carolina Strosberg, Alfio Ferlito, Asterios Triantafyllou, Douglas R Gnepp, Justin A Bishop, Henrik Hellquist, Primoz Strojan, Stefan M Willems, Göran Stenman, Alessandra Rinaldo, Juan C Hernandez-Prera

Research output: Contribution to journalReview articlepeer-review


OBJECTIVES: Laryngeal neuroendocrine carcinomas are heterogeneous neoplasms characterized by neuroendocrine differentiation. Their prognoses are dependent on tumor type, therefore different classifications have been developed. Moreover, other tumors have overlapping pathologic features posing a range of diagnostic possibilities.

METHODS: A review of the literature was performed to comprehensively understand the classification and diagnosis of these tumors.

RESULTS: We review the past and present classification systems, with emphasis to the latest 2017 World Health Organization Classification of Head and Neck Tumors. We highlight salient clinicopathologic features and discuss the presumptive etiologic role of human papilloma virus. We share a practical algorithmic approach to the diagnosis of suspected neuroendocrine neoplasms of the larynx including a novel marker for neuroendocrine differentiation, insulinoma-associated protein 1.

CONCLUSIONS: Accurate diagnosis and grading of laryngeal neuroendocrine carcinomas is critical for prognostication and therapeutic decision making. The use of an algorithm is instrumental in assuring the exclusion of mimickers.

Original languageEnglish
Pages (from-to)686-700
Number of pages15
JournalAmerican journal of clinical pathology
Issue number6
Early online date15 Aug 2019
Publication statusPublished - Dec 2019


  • Human papillomavirus (HPV)
  • INSM1
  • Larynx
  • Neuroendocrine carcinoma
  • Neuroendocrine differentiation


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