Endocrine Abstracts

Background: The well-differentiated grade 3 neuroendocrine tumor (G3NET) category was introduced in the 2017 WHO classification as a new category of high-grade neuroendocrine neoplasm (NEN). G3NET is thought to have worse overall survival (OS) than lower grade NET, but better OS than poorly differentiated neuroendocrine carcinoma (NEC). However, challenges in pathologic diagnosis and changes in terminology have limited our understanding of the G3NET category. We compared outco...

Background: Patients with neuroendocrine neoplasms (NENs) have chronic symptoms from hormone overproduction, tumor growth and metastasis, and side effects from prolonged treatment. We assessed the feasibility of using a digital health platform to recruit patients with NENs to evaluate symptoms and quality of life.Methods: eNET is a prospective, web-based, cohort study for patients with advanced NENs of any primary site, histologic grade, differentiation,...

Background: Pancreatic neuroendocrine neoplasms (panNENs) are heterogeneous, with grade (G) defined by Ki67 proliferation index (<3% G1, 3-20% G2, >20% G3) or mitotic rate. The G3 NEN subgroup is further divided into well differentiated neuroendocrine tumors (NETs) and poorly differentiated neuroendocrine carcinomas (NEC). Grade progression can occur over time, with low-to-high grade progression (L-to-H; G1/2 to G3) the most clinically relevant form. It is associated w...

Background: The epidemiology of typical (TC) and atypical carcinoid (AC) bronchopulmonary neuroendocrine tumors (pulmNETs) is not well understood. We aim to describe the incidence of pulmNETs and differences by sex and race and ethnicity (R&E) in the diverse state of California.Methods: All patients with malignant pulmNETs diagnosed from 1992-2019 in the population-based California Cancer Registry were identified by histology (ICD-O-3 code 8240 for T...

Background: Patients with neuroendocrine neoplasms (NENs) have a high burden of chronic symptoms. We assessed symptom burden and overall quality of life (QOL) for eNET study participants and analyzed differences by age, sex, and NEN primary site.Methods: eNET is a prospective, cohort study on the Eureka digital health platform enrolling individuals with advanced NENs. Participants completed validated surveys on an internet-supported device at baseline an...

Background: Gastrointestinal neuroendocrine tumors (GI NETs) are subdivided into grades (G) G1-G3 based on Ki-67 proliferation index (%) (G1<3%, G2 3-20%, and G3 >20%) or mitotic rate, with tumor grade informing prognosis and treatment. Grade progression (GP) over time in GI and pancreatic NETs has recently been identified, with low(G1/2)-to-high(G3) grade progression (L-to-H) the most clinically relevant form. L-to-H is associated with worse survival, yet the timefram...

Background: Grade 3 pancreatic neuroendocrine tumor (G3-PanNET) and neuroendocrine carcinoma (PanNEC) are both defined by Ki67>20% and/or mitoses >20 per 2mm2. PanNET and PanNEC are thought to be molecularly distinct entities and progression from PanNET to PanNEC is considered rare. MEN1, ATRX, DAXX, and TSC1/2 mutations are common in PanNET, while TP53, RB1, KRAS, and SMAD4 mutations are typical of PanNEC. Immunostains for ATRX/DAXX/p53/Rb aid in the classification of...

Background: Pancreatic neuroendocrine tumors (panNETs) are heterogeneous, with grade (G) defined by Ki67 proliferation index (<3% G1, 3-20% G2, and >20% G3) or mitotic rate. Previous studies suggest that baseline Ki67 index may be confounded by biopsy site (primary or metastasis), biopsy technique and primary tumor size. Ki67 differences leading to grade discordance in PanNETs at baseline is relatively understudied. Our study aims to evaluate grade discordance in synch...

Background: The incidence of germline pathogenic/likely pathogenic variants (P/LPV) is relatively well described in low grade well differentiated neuroendocrine tumors (NETs). However, germline findings in G3 NENs including grade 3 NETs (G3NET) and poorly differentiated neuroendocrine carcinoma (NEC) is gravely understudied, and guidance related to germline testing in G3NEN is lacking.Methods: An IRB approved, single institution, retrospective chart revi...

Background: Germline pathogenic or likely pathogenic (P/LP) variants occur in approximately 10% of NEN patients with recent data suggesting a higher frequency in pancreatic NENs or paraganglioma/pheochromocytoma (PPGL). However, identification of VUS can complicate interpretation of germline results, particularly when diverse populations are under study and the optimal gene panel size for testing remains unclear.Methods: A single-center retrospective cha...