Searchable abstracts of presentations at key conferences in endocrinology
Previous issue | Volume 98 | NANETS2023



Clinical – Surgery/Applied Pathology

ea0098c43 | Clinical – Surgery/Applied Pathology | NANETS2023

Accuracy of DOTATATE PET imaging in the preoperative planning of small bowel neuroendocrine tumor resection

Zhang, MD Chi , Gudmundsdottir, MD Hallbera , Takahashi, MD, PhD Hiroaki , Day, MS Courtney , Glasgow, MHA Amy , Wasif, MD, MPH Nabil , Starlinger, MD Patrick , Warner, MD Susanne , Grotz, MD Travis , Smoot, MD Rory , Truty, MD Mark , Cleary, MD Sean , Kendrick, MD Michael , Nagorney, MD David , Navin, MB, BCh Patrick , Halfdanarson, MD Thorvardur R. , Thiels, DO, MBA Cornelius

Background: Small bowel neuroendocrine tumors (sbNETs) incidence is on the rise. Given that sbNETS are often multifocal with high risk of regional nodal metastasis, open resection with lymphadenectomy is the gold standard approach as it offers manual palpation of the entire small bowel. We evaluated the accuracy of preoperative DOTATATE PET/CT in estimating the number of lesions and the presence of nodal disease compared to postoperative pathology findings. We hypothesize that...

ea0098c44 | Clinical – Surgery/Applied Pathology | NANETS2023

Real-time prospective DAXX/ATRX testing of 186 consecutive surgically resected pancreatic neuroendocrine tumors reveals loss of expression correlates with distant metastasis

D Bell, Katelyn Smith Phoenix , Paniccia Alessandro , K Lee Kenneth , F Pingpank James , Hogg Melissa , J Zeh Herbert , N Nikiforov Marina , D Singhi Aatur , H Zureikat Amer

Background: Pancreatic neuroendocrine tumors (PanNETs) are a heterogeneous group of neoplasms with increasing incidence and unpredictable behavior. Whole-exome sequencing studies of metastatic PanNETs have found recurrent genomic alterations in DAXX and ATRX, which correlate with corresponding loss of protein expression. Loss of DAXX/ATRX in PanNETs is reported to be associated with several, adverse clinicopathologic features and poor patient disease-free survival (DFS). Howev...

ea0098c45 | Clinical – Surgery/Applied Pathology | NANETS2023

Grade progression in gastrointestinal neuroendocrine tumors

Hill-Fung Lau Bryan , Moon Farhana , Joseph Nancy , Nakakura Eric , Khuong Le Bryan , Wang Stephanie , Zhang Li , Bergsland Emily

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...

ea0098c46 | Clinical – Surgery/Applied Pathology | NANETS2023

Peptide receptor radionuclide therapy improves survival in patients who progress after resection of gastroenteropancreatic neuroendocrine tumors

Borbon Luis C. , Sherman Scott K. , Breheny Patrick , Ear Po H. , Menda Yusuf , Chandrasekharan Chandrikha , Bellizzi Andrew M. , O'Dorisio Thomas M. , Dillon Joseph S. , Howe James R

Background: Peptide Receptor Radionuclide Therapy (PRRT) is effective for gastroenteropancreatic (GEP) neuroendocrine tumors (NETs) and received FDA approval after demonstrating improved progression-free-survival (PFS) in advanced midgut NETs. We investigated a two-decade experience with PRRT, hypothesizing that PRRT confers PFS and overall-survival (OS) advantages in patients who progress after surgery.Methods: A single-institutional NET database was re...

ea0098c47 | Clinical – Surgery/Applied Pathology | NANETS2023

Surgical cytoreduction vs systemic therapy in patients with metastatic gastroenteropancreatic neuroendocrine tumors (GEP-NETS): NCDB analysis

Mohamed A. , Fasih A. , Ocuin L.M. , Winter J. , Ammori J. , Hardacre J. , Mahipal A. , Bajor D. , Chakrabarti S. , Asa S.L. , Selfridge J.E. , Tirumani S.H. , Henke L.E. , Hoehn R.S

Background: The role of surgical cytoreduction for metastatic gastroenteropancreatic neuroendocrine tumors (GEP-NETs) is considered an area of debate due to lack of prospective data. While retrospective and single institution analyses demonstrated higher survival rates associated with surgical cytoreduction, there was no comparison with systemic therapies alone in these studies. The aim of this analysis of the National Cancer Database (NCDB) was to evaluate the survival benefi...

ea0098c48 | Clinical – Surgery/Applied Pathology | NANETS2023

Predictors of low-to-high grade progression in pancreatic neuroendocrine tumors

Wang Stephanie J. , Moon Farhana , Joseph Nancy , Nakakura Eric , Khuong Le Bryan , Zhang Li , Bergsland Emily

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...

ea0098c49 | Clinical – Surgery/Applied Pathology | NANETS2023

Post-procedure outcomes of liver-directed therapy of neuroendocrine liver metastases

Meloche-Dumas Leamarie , Mercier Frederic , Barabash Victoria , Law Calvin , Singh Simron , Myrehaug Sten , Chan Wing , Hallet Julie

Background: While there have been major advances in the care of neuroendocrine tumors (NETs), there is still no widely adopted therapeutic sequencing in metastatic NETs. The roles and benefits of locoregional treatments need reassessment, in order to define a modern therapeutic algorithm. We examined contemporary short-term outcomes of liver-directed therapy for metastatic NETs.Methods: We conducted a population-based retrospective cohort study of patien...

ea0098c50 | Clinical – Surgery/Applied Pathology | NANETS2023

Phenotype Genotype Correlation in Multiple Endocrine Neoplasia Type 1

Worthy Charlita , Vikram Nayan U. , Tora Rana , Welch James , Ninan Anisha , Bliss Lynn , Cochran Craig , S Weinstein Lee , Simonds William F. , Blau Jenny E. , Agarwal Sunita K. , Jha Smita

Background: The presence of a genotype-phenotype correlation in patients with MEN1 remains controversial with conflicting data from different centers. Furthermore, about 10-30% patients have genotype-negative (GN)-MEN1. Here, we evaluate the presence of genotype-phenotype correlation in our cohort of comprehensively phenotyped patients with MEN1. In addition, we compare the phenotype of GN-MEN1 and genotype-positive (GP)-MEN1 patients and investigate somatic mosaicism as a cau...

ea0098c51 | Clinical – Surgery/Applied Pathology | NANETS2023

A prospective phase ii single-arm trial on neoadjuvant peptide receptor radionuclide therapy (PRRT) with 177Lu-DOTATATE followed by surgery for pancreatic neuroendocrine tumors (NeoLuPaNET)

Partelli Stefano , Landoni Luca , Bartolomei Mirco , Zerbi Alessandro , Maria Grana Chiara , Boggi Ugo , Butturini Giovanni , Casadei Riccardo , Bassi Claudio , Falconi Massimo

Background: Surgical resection of Nonfunctioning Pancreatic Neuroendocrine Tumor (NF-PanNET) is curative in most of the cases. Neoadjuvant treatments in patients with resectable NF-PanNET at high-risk of recurrence have never been investigated. Aim of this study was to test the safety and efficacy of neoadjuvant PRRT with 177Lu-DOTATATE followed by surgery in patients with resectable high-risk NF-PanNET.Methods: This was a multi-center single-...

ea0098c52 | Clinical – Surgery/Applied Pathology | NANETS2023

Translational potential of a receptor-targeted contrast agent for fluorescence-guided applications in pancreatic neuroendocrine tumors

AghaAmiri Solmaz , Estrella Jeannelyn S. , Hurd Mark W. , Hernandez Vargas Servando , Ghosh Sukhen C. , Azhdarinia Ali , Ikoma Naruhiko

Background: Accurate intraoperative localization of primary and metastatic pancreatic neuroendocrine tumors (pNETs) is challenging, and molecularly driven fluorescence-guided surgery could significantly improve surgical outcomes with given the overexpression of somatostatin receptor subtype-2 (SSTR2). We developed an optimized SSTR2-targeted fluorescent agent, MMC(FNIR-Tag)-TOC, which expressed excellent targeting specificity and pharmacokinetics, as the lead compound for clin...

ea0098c54 | Clinical – Surgery/Applied Pathology | NANETS2023

Tissue section thickness of Ki67 immunohistochemistry does not have a correlative effect on pancreatic neuroendocrine tumor grading

Ng Moreen , Sinard John , Kunstman John , Kunz Pam , Misdraji Joseph

Background: Despite the widespread use of immunohistochemistry (IHC), there are debates on how pre-analytic variables influence diagnostic results, including the effect of varying tissue section thickness. Scant literature suggests that tissue thickness can affect stain interpretation, including nuclear stains that rely on assigning tumor cells to the positive category, such as Ki-67, and raise the question of whether tissue section thickness should be standardized for some di...

ea0098c55 | Clinical – Surgery/Applied Pathology | NANETS2023

Association of long-term PPI use with low-risk gastric neuroendocrine tumor

Al-Toubah MPH Taymeyah , Pelle MD Eleonora , Strosberg MD Jonathan

Background: Gastric neuroendocrine tumors are rare neoplasms, comprising approximately 2% of all gastric tumors, and develop from enterochromaffin-like (ECL) cells in the gastric mucosa. Type I and II develop due to hypergastrinemia and ECL cell hyperplasia; type III typically occur sporadically, tend to be more aggressive, present metastatically in > 50% of cases, have normal fasting gastrin levels, and vary histopathologically from well- to poorly differentiated tumors. ...

ea0098c56 | Clinical – Surgery/Applied Pathology | NANETS2023

Comparative gene expression and pathway analysis of neuroendocrine neoplasms in relation to clinical outcomes and tumor location

Laderian Bahar , Saberzadeh Ardestani Bahar , Chen Yanwen , Mohamed Amr , Ni Ying , Mundi Prabhjot

Background: Neuroendocrine malignancies are heterogeneous cancers with varied clinical outcomes. The molecular landscape driving this heterogeneity has not yet been fully characterized. We aimed to investigate the gene expression profiles of neuroendocrine neoplasms, in relationship to baseline clinicopathological features and clinical outcomes, to elucidate underlying biology and potential therapeutic targets.Methods: Patients with neuroendocrine tumors...

ea0098c57 | Clinical – Surgery/Applied Pathology | NANETS2023

Perioperative management of carcinoid crisis: protocol for a modified Delphi international expert consensus statement with patient engagement

Hallet Julie , Alam Fahad , Rocha Flavio , Del Rivero Jaydira , Partelli Stefano , Koea Jonathan , Ladowski Stephanie , Saliba Chadi , Pommier Rodney

Background: Patients with neuroendocrine tumors (NETs) are at risk of carcinoid crisis when undergoing operations, which is associated with adverse post-operative outcomes. There is no contemporary guideline regarding the perioperative management of patients at risk of carcinoid crisis. Moreover, recent evidence has challenged traditional management with somatostatin analogs preparation and avoidance of beta-adrenergic medication. Therefore, specific guidance is needed to prov...

ea0098c58 | Clinical – Surgery/Applied Pathology | NANETS2023

Do Pancreatic Well-differentiated Neuroendocrine Tumor (NET) Progress to Poorly-differentiated Neuroendocrine Carcinoma (NEC)?

Joseph Nancy , Umetsu Sarah , Kakar Sanjay , Wang Stephanie J. , Nakakura Eric , Paciorek Alan , Khuong Le Bryan , Moon Farhana , Bergsland Emily

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...

ea0098c59 | Clinical – Surgery/Applied Pathology | NANETS2023

High grade medullary thyroid carcinoma predicts greater lymph node burden in the ipsilateral lateral neck

Nigam Aradhya , Xu Bin , Spanheimer Philip M. , Ganly Ian , Michael Tuttle R. , Wong Richard J. , Shaha Ashok R. , Ghossein Ronald A. , Untch Brian R.

Background: The International Medullary Thyroid Carcinoma Grading System (IMTCGS) is a newly established grading system for medullary thyroid carcinoma and is predictive of disease-specific outcomes. When compared to low-grade tumor patients, patients with high-grade tumors have worse locoregional recurrence rates and overall survival. We aimed to investigate how tumor grade impacts neck lymph node burden and post-resection recurrence patterns in MTC. Me...