Neuroendocrine Carcinomas of the Larynx and Head and Neck: Challenges in Classification and Grading

Abstract

Primary neuroendocrine carcinomas (NECs) of the larynx and head and neck are an uncommon and heterogeneous group of neoplasms categorized by the 2017 WHO Classification of Head and Neck Tumors as: (a) well-differentiated (WD-NEC), (b) moderately-differentiated (MD-NEC), and (c) poorly-differentiated (PD-NEC) with small cell and large cell types. The classification incorporates elements of differentiation and grading and closely correlates to the 5-year disease specific survival of 100, 52.8, 19.3 and 15.3% for each diagnostic category. These survival rates are based on historical data limited by the previous lack of standard pathologic diagnostic criteria. The classification has de-emphasized the use of the terms "carcinoid" and "atypical carcinoid" as diagnostic categories. The adoption of uniform pathologic criteria for the classification of NECs of the head and neck should enable the design of high quality studies in order to understand the molecular alterations of these neoplasms.

Neuroendocrine Tumor of Larynx: A Case Report

“…indicated. 3,7 Our patient was treated with endoscopic excision of the lesion without neck dissection, and function of the larynx is preserved. Radiotherapy and chemotherapy are reserved for unfavorable histology and somatostatin analogues can be considered in patients with inoperable or metastatic disease to slow down growth and control carcinoid syndrome.…”

Coexistence of Basaloid Squamous Cell Carcinoma and Large Cell Neuroendocrine Carcinoma in the Larynx: A Case Report and Literature Review

“…11 Smoking seems to be associated with the development of these large cell carcinomas. Although LCNECs are rare, they are extremely aggressive and have a high mortality rate, 12 and a meta-analysis of 29 LCNEC cases revealed a 5-year disease-specific survival rate of only 15%. 13 Moreover, most patients develop distant metastatic disease and die within 2 years.…”

Nomograms Forecasting Long-Term Overall and Cancer Specific Survival of Patients With Head and Neck Neuroendocrine Carcinoma

“…This problem with neuroendocrine carcinoma of the head and neck persisted until the World Health Organization (WHO) in 2017 classified NEC into three sub-categories: well-differentiated NEC (typical carcinoid), moderately differentiated NEC (atypical carcinoid), and poorly differentiated NEC ( 4 ). The poorly differentiated NEC was further divided into large cell NEC and small-cell NEC ( 5 , 6 ). Since then, the classification of HNNEC has had a uniform standard.…”