Neuroendocrine Neoplasms of the Larynx: A Clinicopathologic Analysis of 27 Neuroendocrine Tumors and Neuroendocrine Carcinomas

2021 ◽  
Author(s):  
Munita Bal ◽  
Aishwarya Sharma ◽  
Swapnil Ulhas Rane ◽  
Neha Mittal ◽  
Devendra Chaukar ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Neuroendocrine Neoplasms ◽  
Neuroendocrine Carcinomas

scholarly journals Molecular Pathology of Well-Differentiated Pulmonary and Thymic Neuroendocrine Tumors: What Do Pathologists Need to Know?

2021 ◽  
Vol 32 (1) ◽  
pp. 154-168 ◽  
Author(s):  
Marco Volante ◽  
Ozgur Mete ◽  
Giuseppe Pelosi ◽  
Anja C. Roden ◽  
Ernst Jan M. Speel ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Young Patients ◽  
Carcinoid Tumors ◽  
Neuroendocrine Neoplasms ◽  
Grey Zone ◽  
Cell Hyperplasia ◽  
Ki 67 ◽  
Familial Form ◽  
Well Differentiated ◽  
Neuroendocrine Carcinomas

AbstractThoracic (pulmonary and thymic) neuroendocrine tumors are well-differentiated epithelial neuroendocrine neoplasms that are classified into typical and atypical carcinoid tumors based on mitotic index cut offs and presence or absence of necrosis. This classification scheme is of great prognostic value but designed for surgical specimens, only. Deep molecular characterization of thoracic neuroendocrine tumors highlighted their difference with neuroendocrine carcinomas. Neuroendocrine tumors of the lung are characterized by a low mutational burden, and a high prevalence of mutations in chromatin remodeling and histone modification-related genes, whereas mutations in genes frequently altered in neuroendocrine carcinomas are rare. Molecular profiling divided thymic neuroendocrine tumors into three clusters with distinct clinical outcomes and characterized by a different average of copy number instability. Moreover, integrated histopathological, molecular and clinical evidence supports the existence of a grey zone category between neuroendocrine tumors (carcinoid tumors) and neuroendocrine carcinomas. Indeed, cases with well differentiated morphology but mitotic/Ki-67 indexes close to neuroendocrine carcinomas have been increasingly recognized. These are characterized by specific molecular profiles and have an aggressive clinical behavior. Finally, thoracic neuroendocrine tumors may arise in the background of genetic susceptibility, being MEN1 syndrome the well-defined familial form. However, pathologists should be aware of rarer germline variants that are associated with the concurrence of neuroendocrine tumors of the lung or their precursors (such as DIPNECH) with other neoplasms, including but not limited to breast carcinomas. Therefore, genetic counseling for all young patients with thoracic neuroendocrine neoplasia and/or any patient with pathological evidence of neuroendocrine cell hyperplasia-to-neoplasia progression sequence or multifocal disease should be considered.

Neuroendocrine Tumors of Larynx

1992 ◽  
Vol 101 (8) ◽  
pp. 710-714 ◽  
Author(s):  
Adel K. El-Naggar ◽  
John G. Batsakis ◽  
Mario A. Luna
Keyword(s):  
Survival Rate ◽  
Neuroendocrine Tumors ◽  
Small Cell ◽  
Neuroendocrine Neoplasm ◽  
Neuroendocrine Neoplasms ◽  
Neural Basis ◽  
Small Cell Neuroendocrine Carcinoma ◽  
Typical Carcinoid ◽  
Clinical Behavior ◽  
Neuroendocrine Carcinomas

Neuroendocrine neoplasms of the larynx have either an epithelial or a neural basis. The former are more numerous and are classified as typical or atypical carcinoids and small cell neuroendocrine carcinomas. Paraganglioma is the sole type of neural neuroendocrine neoplasm. There is a significant worsening of prognosis from typical carcinoid to small cell neuroendocrine carcinoma, with the latter having a dismal 5-year survival rate regardless of therapy. Paragangliomas are the most benign of laryngeal neuroendocrine neoplasms, but their clinical behavior may not be predictable on the basis of their histologic appearance.

scholarly journals Tumor size and perineural invasion predict outcome of gastric high grade neuroendocrine neoplasms

2021 ◽  
Author(s):  
Qi Zhang ◽  
Hongshan Wang ◽  
Yanhong Xie ◽  
Suming Huang ◽  
Ke Chen ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Tumor Size ◽  
Who Classification ◽  
Perineural Invasion ◽  
Radical Resection ◽  
Neuroendocrine Neoplasms ◽  
High Grade ◽  
Ki67 Index ◽  
Grade 3 ◽  
Neuroendocrine Carcinomas

A new subcategory, grade 3 neuroendocrine tumors, is incorporated into the grading system of pancreatic neuroendocrine neoplasms in the 2017 WHO classification in order to differentiate grade 3 neuroendocrine tumors from neuroendocrine carcinomas. The 2019 WHO classification extends the concept of grade 3 neuroendocrine tumors to gastrointestinal high grade neuroendocrine neoplasms. However, there is still limited study focusing on the gastric grade 3 neuroendocrine tumors and gastric neuroendocrine carcinomas. We retrospectively enrolled 151 gastric high grade neuroendocrine neoplasms patients, who underwent radical resection from January 2007 to December 2015. Clinicopathologic and prognostic features were studied. The Surveillance, Epidemiology, and End Results database was used to verify the prognostic determinants found in Zhongshan cohort. Neuroendocrine carcinomas showed higher Ki67 index and higher mitotic count than grade 3 neuroendocrine tumors. We identified 109 (72.2%) patients of neuroendocrine carcinomas, 12 (7.9%) patients of grade 3 neuroendocrine tumors and 30 (19.9%) patients of mixed neuroendocrine non-neuroendocrine neoplasms. Although neuroendocrine carcinomas demonstrated higher Ki67 index (p=0.004) and mitoses (p=0.001) than grade 3 neuroendocrine tumors, their prognosis after radical resection did not demonstrate significant differences (p=0.709). Tumor size, perineural invasion and TNM stage were independent prognostic factors of gastric high grade neuroendocrine neoplasms.

scholarly journals Neuroendocrine neoplasms of the pancreas: diagnosis and pitfalls

2021 ◽  
Author(s):  
Björn Konukiewitz ◽  
Moritz Jesinghaus ◽  
Atsuko Kasajima ◽  
Günter Klöppel
Keyword(s):  
Neuroendocrine Tumors ◽  
Chromogranin A ◽  
Molecular Profile ◽  
Neuroendocrine Neoplasms ◽  
Histological Differentiation ◽  
Pancreatic Neuroendocrine Neoplasms ◽  
Poorly Differentiated ◽  
Well Differentiated ◽  
Diagnostic Pitfalls ◽  
Neuroendocrine Carcinomas

AbstractCommon to neuroendocrine neoplasms of the pancreas is their expression of synaptophysin, chromogranin A, and/or INSM1. They differ, however, in their histological differentiation and molecular profile. Three groups can be distinguished: well-differentiated neuroendocrine neoplasms (neuroendocrine tumors), poorly differentiated neuroendocrine neoplasms (neuroendocrine carcinomas), and mixed neuroendocrine-non-neuroendocrine neoplasms. However, the expression of synaptophysin and, to a lesser extent, also chromogranin A is not restricted to the neuroendocrine neoplasms, but may also be in a subset of non-neuroendocrine epithelial and non-epithelial neoplasms. This review provides the essential criteria for the diagnosis of pancreatic neuroendocrine neoplasms including diagnostic clues for the distinction of high-grade neuroendocrine tumors from neuroendocrine carcinomas and an algorithm avoiding diagnostic pitfalls in the delineation of non-neuroendocrine neoplasms with neuroendocrine features from pancreatic neuroendocrine neoplasms.

Modern approaches to the treatment of gastric neuroendocrine tumors

2016 ◽  
Vol 21 (3) ◽  
pp. 165-168
Author(s):  
Ivan N. Peregorodiev ◽  
V. Y Bokhian ◽  
I. S Stilidi ◽  
V. V Delektorskaya
Keyword(s):  
Neuroendocrine Tumors ◽  
Activity Index ◽  
Morphological Type ◽  
Neuroendocrine Neoplasms ◽  
Type I ◽  
Wide Range ◽  
High Proliferative Activity ◽  
Well Differentiated ◽  
The One ◽  
Neuroendocrine Carcinomas

Gastrointestinal neuroendocrine tumors (GI-NETs) include a wide range of tumors with different variants of the course of the disease. At the one end of the clinical spectrum there are highly differentiated type I GI-NETs, with five-year survival rate over 95%, at the another end there are low-differentiated neuroendocrine carcinoma (large-, smallcell cancers) representing a tumor with extremely poor prognosis. Therapeutic approaches to different types of tumors are different. It is necessary to distinguish the treatment of well-differentiated neuroendocrine neoplasms (I and II clinical-morphological type) and low-differentiated neuroendocrine carcinomas. At the same time, it should be noted how different is the treatment of well-differentiated neuroendocrine tumors with high proliferative activity index (III clinico-morphological type of tumor) and low-differentiated neuroendocrine carcinomas (large-, small-cell cancers).

Epidemiology of Neuroendocrine Neoplasmas in Japan: Based on Analysis of Hospital-Based Cancer Registry Data, 2009 – 2015

2021 ◽  
Author(s):  
Tomonobu Koizumi ◽  
Kengo Otsuki ◽  
Yuriko Tanaka ◽  
Shintaro Kanda
Keyword(s):  
Neuroendocrine Tumors ◽  
Cancer Registry ◽  
Neuroendocrine Carcinoma ◽  
Cancer Registries ◽  
The Body ◽  
National Database ◽  
Organ Distribution ◽  
Neuroendocrine Neoplasms ◽  
Using Data ◽  
Neuroendocrine Carcinomas

Abstract Purpose: Neuroendocrine neoplasms are rare disease and could originate from throughout the body, however, there have been little epidemiological studies in Japan, especially the organ distribution. This study was to examine the epidemiological information of neuroendocrine neoplasms in the Japanese population using data from a hospital-based cancer registry. Methods: Using data from the national database of hospital-based cancer registries, we examined the organ distribution, the stage and initial treatment of neuroendocrine neoplasms newly diagnosed and treated in designated and non-designated cancer care hospitals between 2009 and 2015. In the present study, neuroendocrine neoplasms consisted of neuroendocrine tumors and carcinoma. Results: A total of 33,215 (17485 neuroendocrine carcinomas and 15730 neuroendocrine tumors) cases were diagnosed. The majority in neuroendocrine carcinoma occur in lung (31.1%) followed in decreasing frequency by stomach (12.9%), pancreas (7.5%), rectum (6.7%) and esophagus (5.8%). On the other hand, the half of neuroendocrine tumor originated rectum (50.9%) and followed by pancreas (13.9%), duodenum (9.0%), lung/bronchus (8.9%), and stomach (8.7%). Neuroendocrine carcinoma presented at more advanced stage and higher age than neuroendocrine tumors.　Most cases of neuroendocrine tumors were treated surgically, while half of neuroendocrine carcinomas were treated with non-surgical therapy consisting of chemotherapy with or without radiotherapy. Conclusions: Our results demonstrated that neuroendocrine neoplasms could originate from various organs and the site distribution was different between neuroendocrine carcinoma and tumor. The national database of hospital-based cancer registries in Japan is a valuable source for evaluating the organ distribution of the rare systemic disease.

scholarly journals Open issues on G3 neuroendocrine neoplasms: back to the future

2018 ◽  
Vol 25 (6) ◽  
pp. R375-R384 ◽  
Author(s):  
Maria Chiara Zatelli ◽  
Elia Guadagno ◽  
Erika Messina ◽  
Fabio Lo Calzo ◽  
Antongiulio Faggiano ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Current Knowledge ◽  
Neuroendocrine Neoplasms ◽  
Clinical Implications ◽  
The Future ◽  
Grade 3 ◽  
The Right ◽  
Neuroendocrine Carcinomas ◽  
Open Issues

The recent recognition that grade 3 (G3) neuroendocrine neoplasms (NENs) can be divided into two different categories according to the histopathological differentiation, that is G3 neuroendocrine tumors (NETs) and G3 neuroendocrine carcinomas (NECs) has generated a lot of interest concerning not only the diagnosis, but also the differential management of such new group of NENs. However, several issues need to be fully clarified in order to put G3 NETs and G3 NECs in the right place. The aim of this review is to focus on those issues that are still undetermined starting from the current knowledge, evaluating the available evidence and the possible clinical implications.

scholarly journals Mixed Neuroendocrine/Non-neuroendocrine Neoplasm (MiNEN) of the Ovary Arising from Endometriosis: Molecular Pathology Analysis in Support of a Pathogenetic Paradigm

2021 ◽  
Author(s):  
Roberta Maragliano ◽  
Laura Libera ◽  
Ileana Carnevali ◽  
Valeria Pensotti ◽  
Giovanna De Vecchi ◽  
...  
Keyword(s):  
Large Cell ◽  
Neuroendocrine Neoplasm ◽  
Molecular Profile ◽  
Neuroendocrine Neoplasms ◽  
Fish Analysis ◽  
Sequencing Analysis ◽  
Endometrioid Carcinoma ◽  
Preneoplastic Lesions ◽  
Ovarian Endometriosis ◽  
Neuroendocrine Carcinomas

AbstractPrimary ovarian neuroendocrine neoplasms (Ov-NENs) are infrequent and mainly represented by well-differentiated forms (neuroendocrine tumors — NETs — or carcinoids). Poorly differentiated neuroendocrine carcinomas (Ov-NECs) are exceedingly rare and only few cases have been reported in the literature. A subset of Ov-NECs are admixed with non-neuroendocrine carcinomas, as it occurs in other female genital organs, as well (mostly endometrium and uterine cervix), and may be assimilated to mixed neuroendocrine/non-neuroendocrine neoplasms (MiNENs) described in digestive and extra-digestive sites. Here, we present a case of large cell Ov-NEC admixed with an endometrioid carcinoma of the ovary, arising in the context of ovarian endometriosis, associated with a uterine endometrial atypical hyperplasia (EAH). We performed targeted next-generation sequencing analysis, along with a comprehensive immunohistochemical study and FISH analysis for TP53 locus, separately on the four morphologically distinct lesions (Ov-NEC, endometrioid carcinoma, endometriosis, and EAH). The results of our study identified molecular alterations of cancer-related genes (PIK3CA, CTNNB1, TP53, RB1, ARID1A, and p16), which were present with an increasing gradient from preneoplastic lesions to malignant proliferations, both neuroendocrine and non-neuroendocrine components. In conclusion, our findings underscored that the two neoplastic components of this Ov-MiNEN share a substantially identical molecular profile and they progress from a preexisting ovarian endometriotic lesion, in a patient with a coexisting preneoplastic proliferation of the endometrium, genotypically and phenotypically related to the ovarian neoplasm. Moreover, this study supports the inclusion of MiNEN in the spectrum ovarian and, possibly, of all gynecological NENs, among which they are currently not classified.

scholarly journals Advances in Molecular Imaging and Radionuclide Therapy of Neuroendocrine Tumors

2020 ◽  
Vol 9 (11) ◽  
pp. 3679
Author(s):  
Anna Yordanova ◽  
Hans-Jürgen Biersack ◽  
Hojjat Ahmadzadehfar
Keyword(s):  
Molecular Imaging ◽  
Neuroendocrine Tumors ◽  
Pet Imaging ◽  
Neuroendocrine Neoplasms ◽  
New Agents ◽  
Positron Emission ◽  
Tailored Approach ◽  
Grade 3 ◽  
New Applications ◽  
Chemokine Receptor 4

Neuroendocrine neoplasms make up a heterogeneous group of tumors with inter-patient and intra-patient variabilities. Molecular imaging can help to identify and characterize neuroendocrine tumors (NETs). Furthermore, imaging and treatment with novel theranostics agents offers a new, tailored approach to managing NETs. Recent advances in the management of NETs aim to enhance the effectiveness of targeted treatment with either modifications of known substances or the development of new substances with better targeting features. There have been several attempts to increase the detectability of NET lesions via positron emission tomography (PET) imaging and improvements in pretreatment planning using dosimetry. Especially notable is PET imaging with the radionuclide Copper-64. Increasing interest is also being paid to theranostics of grade 3 and purely differentiated NETs, for example, via targeting of the C-X-C motif chemokine receptor 4 (CXCR4). The aim of this review is to summarize the most relevant recent studies, which present promising new agents in molecular imaging and therapy for NETs, novel combination therapies and new applications of existing molecular imaging modalities in nuclear medicine.

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