Neuroendocrine carcinoma of the larynx and pharynx : a clinical and histopathological study

Strojan, Primož (author), Šifrer, Robert (author), Ferlito, Alfio (author), Grašič-Kuhar, Cvetka (author), Lanišnik, Boštjan (author), Plavc, Gaber (author), Zidar, Nina (author)

Neuroendocrine carcinomas (NECs) of the head and neck are rare and the experience scanty. The Cancer Registry of Slovenia database was used to identify cases of laryngeal and pharyngeal NECs diagnosed between 1995–2020. Biopsies were analyzed for the expression of standard neuroendocrine markers (synaptophysin, chromogranin, CD56), INSM1, Ki-67, p16, and PD-L1 (using the combined positive score, CPS). In situ hybridization for human papillomavirus (HPV) and Epstein–Barr virus (EBV) was performed. Twenty patients (larynx, 12; pharynx, 8) were identified. One tumor was well differentiated (WD), five were moderately differentiated (MD), and 14 were poorly differentiated (PD). Disease control was achieved solely by surgery in 4/4 MD/PD T1-2N0-1 tumors. Eight patients died of the disease, seven of which were due to distant metastases. All three traditional markers were positive in 11/17 NECs and the INSM1 marker in all 20 tumors. Two of fourteen p16-positive tumors were HPV-positive, but all three nasopharyngeal NECs were EBV-negative. Three tumors had CPSs ≥ 1. In conclusion, INSM1 was confirmed to be a reliable marker of neuroendocrine differentiation. Except in WD and early-stage MD/PD tumors, aggressive multimodal therapy is needed; the optimal systemic therapy remains to be determined. p16, HPV, and EBV seem to bear no prognostic information.

• Language: English
• Keywords: electrochemotherapy, head and neck cancer, neuroendocrine carcinoma, diagnosis, therapy, prognosis
• Work type: Article
• Typology: 1.01 - Original Scientific Article
• Organization: MF - Faculty of Medicine
• Publication status: Published
• Publication version: Version of Record
• Year: 2021
• Number of pages: 16 str.
• Numbering: Vol. 13, iss. 19, art. 4813
• PID: 20.500.12556/RUL-136299
• UDC: 615.82/.84
• ISSN on article: 2072-6694
• DOI: 10.3390/cancers13194813
• COBISS.SI-ID: 79949315
• Publication date in RUL: 22.04.2022

Record is a part of a journal

• Title: Cancers
• Shortened title: Cancers
• Publisher: MDPI
• ISSN: 2072-6694
• COBISS.SI-ID: 517914137

Licences

• License: CC BY 4.0, Creative Commons Attribution 4.0 International
• Link: http://creativecommons.org/licenses/by/4.0/
• Description: This is the standard Creative Commons license that gives others maximum freedom to do what they want with the work as long as they credit the author.
• Licensing start date: 01.10.2021

Secondary language

• Language: Slovenian
• Keywords: elektrokemoterapija, rak glave in vratu, nevroendokrini rak

Projects

• Funder: ARRS - Slovenian Research Agency
• Project number: P3-0307
• Name: Rak glave in vratu - analiza bioloških značilnosti in poskus izboljšanja zdravljenja

• Funder: ARRS - Slovenian Research Agency
• Project number: P3-0054
• Name: Patologija in molekularna genetika