scholarly journals Primary Mesenteric Carcinoid Tumor Presenting with Carcinoid Syndrome

2018 ◽  
Vol 12 (2) ◽  
pp. 396-401
Author(s):  
Oluwaseun Shogbesan ◽  
Abdullateef Abdulkareem ◽  
Binu Pappachen ◽  
John Altomare
Keyword(s):  
Carcinoid Tumor ◽  
Primary Tumor ◽  
Carcinoid Syndrome ◽  
The Body ◽  
Carcinoid Tumors ◽  
Neuroendocrine Cells ◽  
Neuroendocrine Neoplasms ◽  
Diverse Group ◽  
Poorly Differentiated ◽  
Well Differentiated

Neuroendocrine neoplasms (NENs) are a diverse group of tumors arising throughout the body with a common origin from neuroendocrine cells. Well-differentiated NENs, also known as neuroendocrine tumors (NETs), are generally indolent and are often found incidentally, while poorly differentiated tumors are more aggressive. Carcinoid tumors are NETs arising from the gastrointestinal tract and less commonly from the lungs, thymus, and kidneys. NETs in the mesentery arise from metastasis from primary tumor, and carcinoid syndrome in this setting results from concomitant metastasis to the liver. Primary mesenteric carcinoid tumors are very rare. We present a 64-year-old man with carcinoid syndrome from a mesenteric carcinoid tumor without evidence of liver metastasis or other primary tumor sites.

Correlation of DLL3 expression in gastroenteropancreatic neuroendocrine neoplasms with loss of RB1 and prognostic significance.

2020 ◽  
Vol 38 (15_suppl) ◽  
pp. 4611-4611
Author(s):  
Chiara Liverani ◽  
Alberto Bongiovanni ◽  
Laura Mercatali ◽  
Federica Pieri ◽  
Chiara Spadazzi ◽  
...  
Keyword(s):  
Ct Scan ◽  
Clinical Characteristics ◽  
Neuroendocrine Cells ◽  
Neuroendocrine Neoplasms ◽  
High Grade ◽  
Cell Lung Carcinoma ◽  
Pet Ct ◽  
Poorly Differentiated ◽  
Well Differentiated ◽  
Pet Ct Scan

4611 Background: Neuroendocrine neoplasms (NENs) are a rare subgroup of tumors with challenging management due to their unpredictable and heterogeneous behaviour. The identification of clinically useful biomarkers is a top priority need in this disease. The negative notch regulator DLL3 has gained increasing attention in small cell lung carcinoma, large cell neuroendocrine carcinoma and neuroendocrine prostate cancer, confirming the tumor suppressor function of Notch-1 signaling in neuroendocrine cells. Methods: A retrospective immunohistochemical analysis of DLL3, PD-L1 and RB1 was performed on FFPE samples from 43 patients with gastroenteropancreatic (GEP)-NENs and correlated with clinical characteristics. Results: DLL3 was expressed in high-grade (G3) GEP-NENs. The presence of DLL3 was significantly associated with poorly differentiated NEC (77.8% positive tumors), while none of the patients with well-differentiated NET expressed this marker. Expression of DLL3 was correlated with loss of RB1 and negative 68Ga-PET/CT scan. The 85.7% of DLL3- positive tumors showed loss of RB1 expression, while only 1 out of 35 DLL3- negative tumors had RB1 loss. DLL3 was expressed in 75% of patients with negative 68Ga-PET/CT, while only in 25% of patients with positive 68Ga-PET/CT scan. The presence of DLL3 was negatively associated with PFS and OS. Median PFS was 41.9 months in DLL3-negative patients versus 7.9 months in DLL3-positive patients; median OS was 72.9 months in DLL3-negative patients versus 11.7 months in DLL3-positive patients. No correlation was found with DLL3 and PD-L1 expression. The presence of PD-L1 was not associated with any clinical characteristics. Conclusions: DLL3 is expressed in high grade GEP-NENs and is associated with loss of RB1, negative 68Ga-PET/CT scan and unfavourable clinical outcome. The presence of DLL3 discriminates poorly differentiated NEC from well-differentiated NET. DLL3 could represent the ideal prognostic factor to stratify patients with GEP-NENs and a candidate therapeutic target in NEC patients.

scholarly journals Somatostatin-Dopamine Chimeric Molecules in Neuroendocrine Neoplasms

2021 ◽  
Vol 10 (3) ◽  
pp. 501
Author(s):  
Maria Celeste Cantone ◽  
Alessandra Dicitore ◽  
Giovanni Vitale
Keyword(s):  
Somatostatin Receptors ◽  
Somatostatin Analogues ◽  
The Body ◽  
Neuroendocrine Cells ◽  
Neuroendocrine Neoplasms ◽  
Magic Bullet ◽  
Comprehensive Overview ◽  
New Class ◽  
Well Differentiated ◽  
Chimeric Molecules

Neuroendocrine neoplasms (NENs) are a widely heterogeneous family of neoplasms arising from neuroendocrine cells, which are interspersed throughout the body. Despite NENs are relatively rare, their incidence and prevalence are constantly increasing probably due to the improvement in earlier diagnosis and patients’ management. When surgery is not curative, particularly for patients with metastatic disease, several medical options are available. Somatostatin analogues (SSA) are the first-line medical therapy for well-differentiated NENs. Interestingly, the heterodimerization of somatostatin receptors (SSTs) with dopamine receptors (DRs) has been discovered in NENs. This phenomenon results in hybrid receptors with enhanced functional activity. On these bases, chimeric molecules embracing somatostatin and dopamine features have been recently developed. The aim of this review is to provide a comprehensive overview of the available preclinical and clinical data regarding chimeric somatostatin-dopamine agonists as a new class of “magic bullet” in the therapy of NENs.

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.

scholarly journals Poorly Differentiated Small-Cell-Type Neuroendocrine Carcinoma of the Prostate: A Case Report and Literature Review

2018 ◽  
Vol 11 (3) ◽  
pp. 676-681 ◽  
Author(s):  
Kishore Kumar ◽  
Rafeeq Ahmed ◽  
Chime Chukwunonso ◽  
Hassan Tariq ◽  
Masooma Niazi ◽  
...  
Keyword(s):  
Neuroendocrine Tumors ◽  
Tumor Grade ◽  
Small Cell ◽  
The Body ◽  
Neuroendocrine Cells ◽  
Cell Type ◽  
Variable Response ◽  
Ki 67 ◽  
Platinum Based Chemotherapy ◽  
Poorly Differentiated

Neuroendocrine cells are widespread throughout the body and can give rise of neuroendocrine tumors due to abnormal growth of the chromaffin cells. Neuroendocrine tumors divide into many subtypes based on tumor grade (Ki-67 index and mitotic count) and differentiation. These tumors can be further divided into secretory and nonsecretory types based on the production of peptide hormone by tumor cells. Poorly differentiated small-cell-type neuroendocrine tumors are one of the subtypes of neuroendocrine tumors. These tumors are less common; however, they tend to be locally invasive and aggressive in behavior with poor overall median survival. Treatment of the nonsecretory small-cell type is modeled to small-cell lung cancer with a regimen consisting of platinum-based chemotherapy and etoposide with variable response. Here, we present a case of poorly differentiated small-cell neuroendocrine tumor originating from the prostate.

scholarly journals INSM1 Is a Highly Specific Marker of Neuroendocrine Differentiation in Primary Neoplasms of the Gastrointestinal Tract, Appendix, and Pancreas

2020 ◽  
Vol 153 (6) ◽  
pp. 811-820 ◽  
Author(s):  
Kelsey E McHugh ◽  
Sanjay Mukhopadhyay ◽  
Erika E Doxtader ◽  
Christopher Lanigan ◽  
Daniela S Allende
Keyword(s):  
Goblet Cell ◽  
Neuroendocrine Differentiation ◽  
Neuroendocrine Neoplasms ◽  
Specific Marker ◽  
Low Grade ◽  
Ki 67 ◽  
Neuroendocrine Markers ◽  
Primary Neoplasms ◽  
Poorly Differentiated ◽  
Well Differentiated

Abstract Objectives INSM1 has been described as a sensitive and specific neuroendocrine marker. This study aims to compare INSM1 with traditional neuroendocrine markers in gastrointestinal neuroendocrine neoplasms. Methods Retrospective review (2008-2018) was used to retrieve paraffin-embedded tissue from 110 gastrointestinal neuroendocrine neoplasms and controls that was subsequently stained with INSM1, synaptophysin, chromogranin, CD56, and Ki-67. Results INSM1 was positive in 16 of 17 (94.1%) gastric, 17 of 18 (94.4%) pancreatic, 13 of 18 (72.2%) small bowel, 17 of 21 (81.0%) colonic, and 26 of 36 (72.2%) appendiceal tumors. INSM1 was positive in 58 of 70 (82.9%) well-differentiated neuroendocrine tumors, 17 of 20 (85.0%) poorly differentiated neuroendocrine carcinomas, 8 of 11 (72.7%) low-grade goblet cell adenocarcinomas (grade 1), and 6 of 9 (66.7%) high-grade goblet cell adenocarcinomas (grade 2/3). INSM1 sensitivity for neuroendocrine neoplasms (80.9%) was less than that of synaptophysin (99.1%), chromogranin (88%), and CD56 (95.3%); specificity was higher (95.7% vs 86.0%, 87.3%, and 86.0%, respectively). Conclusions INSM1 is a useful marker of neuroendocrine differentiation in gastrointestinal neuroendocrine and mixed neuroendocrine neoplasms. Compared with traditional neuroendocrine markers, INSM1 is less sensitive but more specific.

Classification and pathology of gastroenteropancreatic neuroendocrine neoplasms

2011 ◽  
Vol 18 (S1) ◽  
pp. S1-S16 ◽  
Author(s):  
Günter Klöppel
Keyword(s):  
Gastrointestinal Tract ◽  
Neuroendocrine Tumours ◽  
Neuroendocrine Tumour ◽  
Tnm Stage ◽  
Neuroendocrine Neoplasms ◽  
Gastroenteropancreatic Neuroendocrine Neoplasms ◽  
Poorly Differentiated ◽  
Well Differentiated ◽  
Neuroendocrine Carcinomas

Gastroenteropancreatic neuroendocrine neoplasms (GEP-NENs) are composed of cells with a neuroendocrine phenotype. The old and the new WHO classifications distinguish between well-differentiated and poorly differentiated neoplasms. All well-differentiated neoplasms, regardless of whether they behave benignly or develop metastases, will be called neuroendocrine tumours (NETs), and graded G1 (Ki67 <2%) or G2 (Ki67 2–20%). All poorly differentiated neoplasms will be termed neuroendocrine carcinomas (NECs) and graded G3 (Ki67 >20%). To stratify the GEP-NETs and GEP-NECs regarding their prognosis, they are now further classified according to TNM-stage systems that were recently proposed by the European Neuroendocrine Tumour Society (ENETS) and the AJCC/UICC. In the light of these criteria the pathology and biology of the various NETs and NECs of the gastrointestinal tract (including the oesophagus) and the pancreas are reviewed.

scholarly journals Paranasal Sinus Neuroendocrine Carcinoma: A Case Report and Review of the Literature

2013 ◽  
Vol 2013 ◽  
pp. 1-5 ◽  
Author(s):  
Nagesh T. Sirsath ◽  
K. Govind Babu ◽  
Umesh Das ◽  
C. S. Premlatha
Keyword(s):  
Paranasal Sinus ◽  
Neuroendocrine Carcinoma ◽  
Neuroendocrine Tumour ◽  
Neuroendocrine Differentiation ◽  
Complete Response ◽  
The Body ◽  
Intracranial Extension ◽  
Neuroendocrine Neoplasms ◽  
Rare Site ◽  
Poorly Differentiated

Neuroendocrine neoplasms are defined as epithelial neoplasms with predominant neuroendocrine differentiation. They can arise in almost every organ of the body although they are most commonly found in the gastrointestinal tract and respiratory system. Nasal cavity and paranasal sinuses are a rare site for neuroendocrine carcinoma. In contrast to the other regions, neuroendocrine tumours of the sinuses have been reported to be recurrent and locally destructive. Very few cases of paranasal sinus neuroendocrine carcinoma have been reported till date. Difficulty in pathologic diagnosis and rarity of this malignancy have hindered the progress in understanding the clinical course and improving outcomes. We herein report a case of poorly differentiated neuroendocrine tumour of ethmoid and sphenoid sinus with invasion of orbit and intracranial extension. The patient had complete response at the end of chemoradiation and he was disease-free for 9 months duration after which he developed bone metastasis without regional recurrence.

scholarly journals Neuroendocrine neoplasms of lung, pancreas and gut: a morphology-based comparison

2020 ◽  
Vol 27 (11) ◽  
pp. R417-R432
Author(s):  
Atsuko Kasajima ◽  
Günter Klöppel
Keyword(s):  
Detailed Comparison ◽  
The Body ◽  
Neuroendocrine Neoplasms ◽  
Genetic Changes ◽  
Organ Systems ◽  
Hereditary Tumor Syndromes ◽  
Poorly Differentiated ◽  
Therapy Strategies ◽  
Genetic Profiles ◽  
Therapeutical Options

The bronchopulmonary (BP) and gastroenteropancreatic (GEP) organ systems harbor the majority of the neuroendocrine neoplasms (NENs) of the body, comprising 20 and 70% of all NENs, respectively. Common to both NEN groups is a classification distinguishing between well- and poorly differentiated NENs associated with distinct genetic profiles. Differences between the two groups concern the reciprocal prevalence of well and poorly differentiated neoplasms, the application of a Ki67-based grading, the variety of histological patterns, the diversity of hormone expression and associated syndromes, the variable involvement in hereditary tumor syndromes, and the peculiarities of genetic changes. This review focuses on a detailed comparison of BP-NENs with GEP-NENs with the aim of highlighting and discussing the most obvious differences. Despite obvious differences, the principle therapeutical options are still the same for both NEN groups, but with further progress in genetics, more targeted therapy strategies can be expected in future.

scholarly journals Pancreatic Neuroendocrine Neoplasms: Landscape and Horizon

2020 ◽  
Vol 144 (7) ◽  
pp. 816-828
Author(s):  
Laura H. Tang
Keyword(s):  
Neuroendocrine Neoplasms ◽  
Specific Gene ◽  
Important Distinction ◽  
Pancreatic Neuroendocrine Neoplasms ◽  
Poorly Differentiated Neuroendocrine Carcinoma ◽  
Poorly Differentiated ◽  
Well Differentiated ◽  
Initial Description ◽  
Definition Of ◽  
Hereditary Conditions

Context.— Since the initial description of pancreatic endocrine physiology and the recognition of islet cell tumors in the 1800s, there have been noteworthy advances in the pathobiology of pancreatic neuroendocrine neoplasms (PanNENs), and definition of the important distinction between well-differentiated neuroendocrine tumor (PanNET) and poorly differentiated neuroendocrine carcinoma (PanNEC). The evolving knowledge has resulted in a continuous update in terminology, classification, and grading system for this group of neoplasms. Pancreatic neuroendocrine tumors associated with hereditary conditions have been linked to unique molecular and genetic events, and sporadic PanNETs have specific gene signatures. Based on accumulative experience and knowledge, therapeutic strategies have been defined for this group of neoplasms. Objective.— To review the evolution and description of the pathologic-genomic evolution of PanNENs, and to facilitate accurate pathologic interpretation for the corresponding clinical management. Data Sources.— Literature review of published studies and author's own work. Conclusions.— Evolving experience and knowledge have established subtypes of pancreatic neuroendocrine neoplasms, based on their genotype and phenotype. Accurate pathologic interpretation of the specific neoplasm has significant implications for therapy and prognosis.

The Spectrum of Neuroendocrine Tumors: Histologic Classification, Unique Features and Areas of Overlap

2015 ◽  
pp. 92-103 ◽  
Author(s):  
David S. Klimstra ◽  
Himisha Beltran ◽  
Rogerio Lilenbaum ◽  
Emily Bergsland
Keyword(s):  
Neuroendocrine Tumors ◽  
Prostate Gland ◽  
Neuroendocrine Differentiation ◽  
Large Cell ◽  
Genetic Alterations ◽  
Neuroendocrine Cells ◽  
Neuroendocrine Neoplasms ◽  
Platinum Based Chemotherapy ◽  
Well Differentiated ◽  
Grade 3

Neuroendocrine neoplasms are diverse in terms of sites of origin, functional status, and degrees of aggressiveness. This review will introduce some of the common features of neuroendocrine neoplasms and will explore the differences in pathology, classification, biology, and clinical management between tumors of different anatomic sites, specifically, the lung, pancreas, and prostate. Despite sharing neuroendocrine differentiation and histologic evidence of the neuroendocrine phenotype in most organs, well-differentiated neuroendocrine tumors (WD-NETs) and poorly differentiated neuroendocrine carcinomas (PD-NECs) are two very different families of neoplasms. WD-NETs (grade 1 and 2) are relatively indolent (with a natural history that can evolve over many years or decades), closely resemble non-neoplastic neuroendocrine cells, and demonstrate production of neurosecretory proteins, such as chromogranin A. They arise in the lungs and throughout the gastrointestinal tract and pancreas, but WD-NETs of the prostate gland are uncommon. Surgical resection is the mainstay of therapy, but treatment of unresectable disease depends on the site of origin. In contrast, PD-NECs (grade 3, small cell or large cell) of all sites often demonstrate alterations in P53 and Rb, exhibit an aggressive clinical course, and are treated with platinum-based chemotherapy. Only WD-NETs arise in patients with inherited neuroendocrine neoplasia syndromes (e.g., multiple endocrine neoplasia type 1), and some common genetic alterations are site-specific (e.g., TMPRSS2-ERG gene rearrangement in PD-NECs arising in the prostate gland). Advances in our understanding of the molecular basis of NETs should lead to new diagnostic and therapeutic strategies and is an area of active investigation.

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