Poorly Differentiated Neuroendocrine Larynx Carcinoma: Clinical Features and miRNAs Signature-A New Goal for Early Diagnosis and Therapy?

Filippo Ricciardiello,Amedeo Boscaino,Giuseppe Tortoriello,Takashi Takeuchi,Alessia Maria Cossu,Marianna Scrima,Marianna Abate,Raffaele Addeo,Ciro Coppola,Alfonso Scarpa,Anna Grimaldi,Giuseppe Russo,Raul Pellini,Giuseppe Longo,Michele Caraglia,Alessandro Ottaiano,Brigida Iorio,Gabriella Misso,Marco Bocchetti,Ferdinando Riccardi,Michela Falco,G Motta

doi:10.3390/jcm10092019

Abstract

Laryngeal neuroendocrine carcinomas (LNECs) are rare and highly heterogeneous malignancies presenting a wide range of pathological and clinical manifestations. Herein, we retrospectively characterize ten patients diagnosticated with LNEC, five of which were defined as well-moderately differentiated neuroendocrine carcinomas, and five that were defined as poorly differentiated neuroendocrine carcinomas, according to the latest WHO classification. Clinical features were analyzed and compared between the two subgroups together with a microRNA study which evidenced a peculiar signature likely related to poorly differentiated larynx neuroendocrine carcinomas. These findings may offer new useful insights for clinicians to improve diagnosis efficiency, therapy response, and patients’ outcome for this aggressive neoplasm.

Highlights

Laryngeal neuroendocrine carcinomas (LNECs) are malignant heterogeneous epithelial neoplasms characterized by neuroendocrine differentiation
The study cohort was formed by 10 cases of LNEC (7 M and 3 F)
MicroRNAs are becoming more and more important given their crucial role in both clinic and translational medicine

Summary

Introduction

Laryngeal neuroendocrine carcinomas (LNECs) are malignant heterogeneous epithelial neoplasms characterized by neuroendocrine differentiation. LNECs are rare, representing approximately 1% of all organ’s neoplasms [1], but are the second most common group of Larynx neoplasms after squamous cell carcinoma [2]. Paraneoplastic syndromes have been reported in patients with these tumors as well [3] The classification of these tumors has evolved throughout the years, and the latest one was described in 2017 in the World Health Organization (WHO) Classification of head and neck tumors [4]. The goal of the current classification is to combine histological characteristics with clinical and prognostic behaviour; neoplasms were differentiated into epithelial and neural. Ki-67 scoring by immunohistochemistry is a common method for differential undifferentiated NET diagnosis, as it reflects the percentage of invading undifferentiated cells [5,6,7]

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