Yao JC, Hassan M, Phan A, et al. One hundred years after "carcinoid": epidemiology of and prognostic factors for neuroendocrine tumors in 35,825 cases in the United States. J Clin Oncol. 2008;26:3063-3072.
National Cancer Institute; Reis L, Melbert D, et al. SEER cancer statistics review, 1975-2004: US prevalence counts for 2004. Updated November 15, 2007. Accessed October 31, 2020. https://seer.cancer.gov/archive/csr/1975_2004/results_merged/topic_prevcounts.pdf
Dasari A, Shen C, Halperin D, et al. Trends in the incidence, prevalence, and survival outcomes in patients with neuroendocrine tumors in the United States. JAMA Oncol. 2017;3:1335-1342.
Sandvik OM, Søreide K, Gudlaugsson E, et al. Epidemiology and classification of gastroenteropancreatic neuroendocrine neoplasms using current coding criteria. Br J Surg. 2016;103:226-232.
Lee MR, Harris C, Baeg KJ, et al. Incidence trends of gastroenteropancreatic neuroendocrine tumors in the United States. Clin Gastroenterol Hepatol. 2019;17:2212-2217.
Genus TSE, Bouvier C, Wong KF, et al. Impact of neuroendocrine morphology on cancer outcomes and stage at diagnosis: a UK nationwide cohort study 2013-2015. Br J Cancer. 2019;121:966-972.
Orphan Drug Act, Public Law 97-414, 96 Stat 2049 (1983).
O'Shea T, Druce M. When should genetic testing be performed in patients with neuroendocrine tumours? Rev Endocr Metab Disord. 2017;18:499-515.
Marini F, Falchetti A, Monte FD, et al. Multiple endocrine neoplasia type 1. Orphanet J Rare Dis. 2006;1:38.
Díez M, Teulé A, Salazar R. Gastroenteropancreatic neuroendocrine tumors: diagnosis and treatment. Ann Gastroenterol. 2013;26:29-36.
Cives M, Strosberg JR. Gastroenteropancreatic neuroendocrine tumors. CA Cancer J Clin. 2018;68:471-487.
Bilimoria KY, Talamonti MS, Tomlinson JS, et al. Prognostic score predicting survival after resection of pancreatic neuroendocrine tumors: analysis of 3851 patients. Ann Surg. 2008;247:490-500.
National Comprehensive Cancer Network. NCCN clinical practice guidelines in oncology: neuroendocrine and adrenal tumors. Version 2.2020. Updated July 24, 2020. Accessed October 12, 2020. https://www.nccn.org/professionals/physician_gls/pdf/neuroendocrine.pdf
Granata V, Fusco R, Setola SV, et al. The multidisciplinary team for gastroenteropancreatic neuroendocrine tumours: the radiologist's challenge. Radiol Oncol. 2019;53:373-387.
Dromain C, Baere Td, Lumbroso J, et al. Detection of liver metastases from endocrine tumors: a prospective comparison of somatostatin receptor scintigraphy, computed tomography, and magnetic resonance imaging. J Clin Oncol. 2005;23:70-78.
Sadowski SM, Neychev V, Millo C, et al. Prospective study of 68Ga-DOTATATE positron emission tomography/computed tomography for detecting gastro-entero-pancreatic neuroendocrine tumors and unknown primary sites. J Clin Oncol. 2016;34:588-596.
Mafficini A, Scarpa A. Genetics and epigenetics of gastroenteropancreatic neuroendocrine neoplasms. Endocr Rev. 2019;40:506-536.
Jiao Y, Shi C, Edil BH, et al. DAXX/ATRX, MEN1, and mTOR pathway genes are frequently altered in pancreatic neuroendocrine tumors. Science. 2011;331:1199-1203.
Di Domenico A, Wiedmer T, Marinoni I, et al. Genetic and epigenetic drivers of neuroendocrine tumours (NET). Endocr Relat Cancer. 2017;24:R315-R334.
Scarpa A, Chang DK, Nones K, et al. Whole-genome landscape of pancreatic neuroendocrine tumours. Nature. 2017;543:65-71.
Walter T, van Brakel B, Vercherat C, et al. O6-methylguanine-DNA methyltransferase status in neuroendocrine tumours: prognostic relevance and association with response to alkylating agents. Br J Cancer. 2015;112:523-531.
Klimstra DS, Beltran H, Lilenbaum R, et al. The spectrum of neuroendocrine tumors: histologic classification, unique features and areas of overlap. American Society of Clinical Oncology Educational Book. 2015:92-103.
Kyriakopoulos G, Mavroeidi V, Chatzellis E, et al. Histopathological, immunohistochemical, genetic and molecular markers of neuroendocrine neoplasms. Ann Transl Med. 2018;6:252.
Sappenfield R. Well-differentiated rectal neuroendocrine tumors: analysis of histology, including INSM1 expression, and biologic behavior, involving a large cohort of 94 cases. Presented at: NANETS Multidisciplinary NET Medical Virtual Symposium; October 2-3, 2020. Abstract 181.
Yang Z, Klimstra DS, Hruban RH, et al. Immunohistochemical characterization of the origins of metastatic well-differentiated neuroendocrine tumors to the liver. Am J Surg Pathol. 2017;41:915-922.
Nagtegaal ID, Odze RD, Klimstra D, et al. The 2019 WHO classification of tumours of the digestive system. Histopathology. 2020;76:182-188.
Tang LH, Basturk O, Sue JJ, et al. A practical approach to the classification of WHO grade 3 (G3) well-differentiated neuroendocrine tumor (WD-NET) and poorly differentiated neuroendocrine carcinoma (PD-NEC) of the pancreas. Am J Surg Pathol. 2016;40:1192-1202.
Strosberg J, Weber JM, Feldman M, et al. Prognostic validity of the American Joint Committee on Cancer staging classification for midgut neuroendocrine tumors. J Clin Oncol. 2013;31:420-425.
Jann H, Roll S, Couvelard A, et al. Neuroendocrine tumors of midgut and hindgut origin: tumor-node-metastasis classification determines clinical outcome. Cancer. 2011;117:3332-3341.
Strosberg JR, Cheema A, Weber J, et al. Prognostic validity of a novel American Joint Committee on Cancer staging classification for pancreatic neuroendocrine tumors. J Clin Oncol. 2011;29:3044-3049.
Boninsegna L, Panzuto F, Partelli S, et al. Malignant pancreatic neuroendocrine tumour: lymph node ratio and Ki67 are predictors of recurrence after curative resections. Eur J Cancer. 2012;48:1608-1615.
Modlin IM, Pavel M, Kidd M, et al. Review article: somatostatin analogues in the treatment of gastroenteropancreatic neuroendocrine (carcinoid) tumours. Aliment Pharmacol Ther. 2010;31:169-188.
O'Toole D, Ducreux M, Bommelaer G, et al. Treatment of carcinoid syndrome: a prospective crossover evaluation of lanreotide versus octreotide in terms of efficacy, patient acceptability, and tolerance. Cancer. 2000;88:770-776.
Xermelo (telotristat ethyl) [prescribing information]. Lexicon Pharmaceuticals Inc. Approved 2017. Revised February 2017.
Kulke MH, Horsch D, Caplin ME, et al. Telotristat ethyl, a tryptophan hydroxylase inhibitor for the treatment of carcinoid syndrome. J Clin Oncol. 2017;35:14-23.
Rinke A, Muller HH, Schade-Brittinger C, et al. Placebo-controlled, double-blind, prospective, randomized study on the effect of octreotide LAR in the control of tumor growth in patients with metastatic neuroendocrine midgut tumors: a report from the PROMID study group. J Clin Oncol. 2009;27:4656-4663.
Caplin ME, Pavel M, Cwikla JB, et al. Lanreotide in metastatic enteropancreatic neuroendocrine tumors. N Engl J Med. 2014;371:224-233.
Lesurtel M, Nagorney DM, Mazzaferro V, et al. When should a liver resection be performed in patients with liver metastases from neuroendocrine tumours? A systematic review with practice recommendations. HPB (Oxford). 2015;17:17-22.
Glazer ES, Tseng JF, Al-Refaie W, et al. Long-term survival after surgical management of neuroendocrine hepatic metastases. HPB (Oxford). 2010;12:427-433.
Kwekkeboom DJ, Krenning EP. Peptide receptor radionuclide therapy in the treatment of neuroendocrine tumors. Hematol Oncol Clin North Am. 2016;30:179-191.
Brabander T, van der Zwan WA, Teunissen JJM, et al. Long-term efficacy, survival, and safety of [(177)Lu-DOTA(0),Tyr(3)]octreotate in patients with gastroenteropancreatic and bronchial neuroendocrine tumors. Clin Cancer Res. 2017;23:4617-4624.
LUTATHERA® (lutetium Lu 177 dotatate injection) [prescribing information]. Advanced Accelerator Applications, a subsidiary of Novartis. Approved 2018.
Strosberg J, El-Haddad G, Wolin E, et al. Phase 3 trial of (177)Lu-dotatate for midgut neuroendocrine tumors. N Engl J Med. 2017;376:125-135.
van Essen M, Krenning EP, Kam BL, et al. Salvage therapy with (177)Lu-octreotate in patients with bronchial and gastroenteropancreatic neuroendocrine tumors. J Nucl Med. 2010;51:383-390.
van der Zwan WA, Brabander T, Kam BLR, et al. Salvage peptide receptor radionuclide therapy with [(177)Lu-DOTA,Tyr(3)]octreotate in patients with bronchial and gastroenteropancreatic neuroendocrine tumours. Eur J Nucl Med Mol Imaging. 2019;46:704-717.
AFINITOR® (everolimus) [prescribing information]. Novartis. Approved 2009. Revised February 2020.
Pavel ME, Hainsworth JD, Baudin E, et al. Everolimus plus octreotide long-acting repeatable for the treatment of advanced neuroendocrine tumours associated with carcinoid syndrome (RADIANT-2): a randomised, placebo-controlled, phase 3 study. Lancet. 2011;378:2005-2012.
Pavel ME, Baudin E, Öberg KE, et al. Efficacy of everolimus plus octreotide LAR in patients with advanced neuroendocrine tumor and carcinoid syndrome: final overall survival from the randomized, placebo-controlled phase 3 RADIANT-2 study. Ann Oncol. 2017;28:1569-1575.
Yao JC, Shah MH, Ito T, et al. Everolimus for advanced pancreatic neuroendocrine tumors. N Engl J Med. 2011;364:514-523.
Yao JC, Fazio N, Singh S, et al. Everolimus for the treatment of advanced, non-functional neuroendocrine tumours of the lung or gastrointestinal tract (RADIANT-4): a randomised, placebo-controlled, phase 3 study. Lancet. 2016;387:968-977.
SUTENT® (sunitinib malate) [prescribing information]. Pfizer Inc. Approved 2006. Revised August 2020.
Raymond E, Dahan L, Raoul JL, et al. Sunitinib malate for the treatment of pancreatic neuroendocrine tumors. N Engl J Med. 2011;364:501-513.
Kouvaraki MA, Ajani JA, Hoff P, et al. Fluorouracil, doxorubicin, and streptozocin in the treatment of patients with locally advanced and metastatic pancreatic endocrine carcinomas. J Clin Oncol. 2004;22:4762-4771.
Chan JA, Stuart K, Earle CC, et al. Prospective study of bevacizumab plus temozolomide in patients with advanced neuroendocrine tumors. J Clin Oncol. 2012;30:2963-2968.
Strosberg JR, Fine RL, Choi J, et al. First-line chemotherapy with capecitabine and temozolomide in patients with metastatic pancreatic endocrine carcinomas. Cancer. 2011;117:268-275.
Kunz PL, Catalano PJ, Nimeiri H, et al. A randomized study of temozolomide or temozolomide and capecitabine in patients with advanced pancreatic neuroendocrine tumors: a trial of the ECOG-ACRIN Cancer Research Group (E2211). J Clin Oncol. 2018;36:4004-4004.

As an organization accredited by the ACCME, Medscape, LLC, requires everyone who is in a position to control the content of an education activity to disclose all relevant financial relationships with any commercial interest. The ACCME defines "relevant financial relationships" as financial relationships in any amount, occurring within the past 12 months, including financial relationships of a spouse or life partner, that could create a conflict of interest.

Medscape, LLC, encourages Authors to identify investigational products or off-label uses of products regulated by the US Food and Drug Administration, at first mention and where appropriate in the content.

Faculty

Jean-Yves Scoazec, MD

Department Head
Laboratory Medicine and Pathology
Gustave Roussy Cancer Center
Villejuif, France

Disclosures

Disclosure: Jean-Yves Scoazec, MD, has disclosed no relevant financial relationships.

Raj Srirajaskanthan, MD, FRCP

Consultant Gastroenterologist
King's College Hospital
London, United Kingdom

Disclosures

Disclosure: Raj Srirajaskanthan, MD, FRCP, has disclosed the following relevant financial relationships:
Served as an advisor or consultant for: AAA (Advanced Accelerator Applications); Ipsen Pharmaceuticals
Served as a speaker or a member of a speakers bureau for: AAA; Ipsen

Jonathan Strosberg, MD

Professor, Department of Gastrointestinal Oncology, Moffitt Cancer Center, Tampa, Florida

Disclosures

Disclosure: Jonathan Strosberg, MD, has disclosed the following relevant financial relationships:
Serve(d) as a consultant for: Novartis
Serve(d) as a speaker or a member of a speakers bureau for: Ipsen; Lexicon

Editor

Megan Whitney, DMD

Medical Education Director, Medscape, LLC

Disclosures

Disclosure: Megan Whitney, DMD, has disclosed no relevant financial relationships.

CME Reviewer

Hazel Dennison, DNP, RN, FNP, CHCP, CPHQ, CNE

Associate Director, Accreditation and Compliance, Medscape, LLC

Disclosures

Disclosure: Hazel Dennison, DNP, RN, FNP, CHCP, CPHQ, CNE, has disclosed no relevant financial relationships.

Medscape, LLC staff have disclosed that they have no relevant financial relationships.

CME / ABIM MOC

ABCs of GEP-NETs: Overview of Neuroendocrine Tumors

Authors: Jean-Yves Scoazec, MD; Raj Srirajaskanthan, MD, FRCP; Jonathan Strosberg, MD
CME / ABIM MOC Released: 12/7/2020
THIS ACTIVITY HAS EXPIRED FOR CREDIT
Valid for credit through: 12/7/2021

Start Activity

Target Audience and Goal Statement

This activity is intended for oncologists, gastroenterologists, and pathologists, as well as endocrinologists, radiologists, and nuclear medicine specialists.

The goal of this activity is to provide foundational education on gastroenteropancreatic neuroendocrine tumors (GEP-NETs) to better equip community physicians for their management throughout the course of the patient journey.

Upon completion of this activity, participants will:

Have increased knowledge regarding the
- Characteristics of GEP-NETs
- Testing methodologies used to diagnose and monitor GEP-NETs
- Guidelines for the management of GEP-NETs

Disclosures

Faculty

Jean-Yves Scoazec, MD

Department Head
Laboratory Medicine and Pathology
Gustave Roussy Cancer Center
Villejuif, France

Disclosures

Disclosure: Jean-Yves Scoazec, MD, has disclosed no relevant financial relationships.

Raj Srirajaskanthan, MD, FRCP

Consultant Gastroenterologist
King's College Hospital
London, United Kingdom

Disclosures

Disclosure: Raj Srirajaskanthan, MD, FRCP, has disclosed the following relevant financial relationships:
Served as an advisor or consultant for: AAA (Advanced Accelerator Applications); Ipsen Pharmaceuticals
Served as a speaker or a member of a speakers bureau for: AAA; Ipsen

Jonathan Strosberg, MD

Professor, Department of Gastrointestinal Oncology, Moffitt Cancer Center, Tampa, Florida

Disclosures

Disclosure: Jonathan Strosberg, MD, has disclosed the following relevant financial relationships:
Serve(d) as a consultant for: Novartis
Serve(d) as a speaker or a member of a speakers bureau for: Ipsen; Lexicon

Editor

Megan Whitney, DMD

Medical Education Director, Medscape, LLC

Disclosures

Disclosure: Megan Whitney, DMD, has disclosed no relevant financial relationships.

CME Reviewer

Hazel Dennison, DNP, RN, FNP, CHCP, CPHQ, CNE

Associate Director, Accreditation and Compliance, Medscape, LLC

Disclosures

Disclosure: Hazel Dennison, DNP, RN, FNP, CHCP, CPHQ, CNE, has disclosed no relevant financial relationships.

Medscape, LLC staff have disclosed that they have no relevant financial relationships.

Accreditation Statements

In support of improving patient care, Medscape, LLC is jointly accredited by the Accreditation Council for Continuing Medical Education (ACCME), the Accreditation Council for Pharmacy Education (ACPE), and the American Nurses Credentialing Center (ANCC), to provide continuing education for the healthcare team.

For Physicians

Medscape, LLC designates this enduring material for a maximum of 1.0 AMA PRA Category 1 Credit(s)™ . Physicians should claim only the credit commensurate with the extent of their participation in the activity.

Successful completion of this CME activity, which includes participation in the evaluation component, enables the participant to earn up to 1.0 MOC points in the American Board of Internal Medicine's (ABIM) Maintenance of Certification (MOC) program. Participants will earn MOC points equivalent to the amount of CME credits claimed for the activity. It is the CME activity provider's responsibility to submit participant completion information to ACCME for the purpose of granting ABIM MOC credit. Aggregate participant data will be shared with commercial supporters of this activity.

Contact This Provider

For questions regarding the content of this activity, contact the accredited provider for this CME/CE activity noted above. For technical assistance, contact [email protected]

Instructions for Participation and Credit

There are no fees for participating in or receiving credit for this online educational activity. For information on applicability and acceptance of continuing education credit for this activity, please consult your professional licensing board.

This activity is designed to be completed within the time designated on the title page; physicians should claim only those credits that reflect the time actually spent in the activity. To successfully earn credit, participants must complete the activity online during the valid credit period that is noted on the title page. To receive AMA PRA Category 1 Credit™, you must receive a minimum score of 75% on the post-test.

Follow these steps to earn CME/CE credit*:

Read the target audience, learning objectives, and author disclosures.
Study the educational content online or printed out.
Online, choose the best answer to each test question. To receive a certificate, you must receive a passing score as designated at the top of the test. We encourage you to complete the Activity Evaluation to provide feedback for future programming.

You may now view or print the certificate from your CME/CE Tracker. You may print the certificate but you cannot alter it. Credits will be tallied in your CME/CE Tracker and archived for 6 years; at any point within this time period you can print out the tally as well as the certificates from the CME/CE Tracker.

*The credit that you receive is based on your user profile.

From Medscape Education Oncology

CME / ABIM MOC

ABCs of GEP-NETs: Overview of Neuroendocrine Tumors

Authors: Jean-Yves Scoazec, MD; Raj Srirajaskanthan, MD, FRCP; Jonathan Strosberg, MDFaculty and Disclosures

THIS ACTIVITY HAS EXPIRED FOR CREDIT

CME / ABIM MOC Released: 12/7/2020

Valid for credit through: 12/7/2021

processing....

¹¹¹In = indium-111
¹⁷⁷Lu = lutetium-177
5-HIAA = 5-hydroxyindoleacetic acid
⁶⁸Ga = gallium-68
⁹⁰Y = yttrium-90
AJCC = American Joint Committee on Cancer
ATRX = α-thalassemia mental retardation syndrome
CDKN1B = cyclin-dependent kinase inhibitor 1B
CDX2 = caudal type homeobox 2
CgA = chromogranin A
CT = computed tomography
DAXX = death domain-associated protein
EUS = endoscopic ultrasound
GEP = gastroenteropancreatic
GEP-NET = gastroenteropancreatic neuroendocrine tumor
GI = gastrointestinal
GI-NET = gastrointestinal neuroendocrine tumor
INSM1 = insulinoma-associated protein 1
ISL1 = insulin gene enhancer protein 1
LAR = long acting release
MEN-1 = multiple endocrine neoplasia type 1
MGMT = O-6-methylguanine-DNA methyltransferase
mPFS = median progression-free survival
MRI = magnetic resonance imaging
mTOR = mammalian target of rapamycin
NCCN = National Comprehensive Cancer Network
NEC = neuroendocrine carcinoma
NEN = neuroendocrine neoplasm
NET = neuroendocrine tumor
ORR = objective response rate
OS = overall survival
PDX1 = insulin promoter factor 1
PET = positron emission tomography
PFS = progression-free survival
pNEC = pancreatic neuroendocrine carcinoma
pNET = pancreatic neuroendocrine tumor
PRRT = peptide receptor radionuclide therapy
RB1 = retinoblastoma protein
SEER = Surveillance, Epidemiology, and End Results
SMAD4 = mothers against decapentaplegic homolog 4
SRS = somatostatin receptor scintigraphy
SSA = somatostatin analogue
SSTR = somatostatin receptor
SSTR2 = somatostatin receptor 2
TNM = tumor, node, metastasis
TP53 = tumor protein p53
TSC1 = tuberous sclerosis 1
TSC2 = tuberous sclerosis 2
TTP = time to progression
UICC = Union for International Cancer Control
VHL = Von Hippel Lindau syndrome
VIP = vasoactive intestinal peptide
WHO = World Health Organization

« Return to: ABCs of GEP-NETs: Overview of Neuroendocrine Tumors

CME / ABIM MOC Information

Print
Like Tweet

References

Faculty and Disclosures

Faculty

Jean-Yves Scoazec, MD

Raj Srirajaskanthan, MD, FRCP

Jonathan Strosberg, MD

Editor

Megan Whitney, DMD

CME Reviewer

Hazel Dennison, DNP, RN, FNP, CHCP, CPHQ, CNE

CME / ABIM MOC

ABCs of GEP-NETs: Overview of Neuroendocrine Tumors

Target Audience and Goal Statement

Disclosures

Faculty

Jean-Yves Scoazec, MD

Raj Srirajaskanthan, MD, FRCP

Jonathan Strosberg, MD

Editor

Megan Whitney, DMD

CME Reviewer

Hazel Dennison, DNP, RN, FNP, CHCP, CPHQ, CNE

Accreditation Statements

For Physicians

Instructions for Participation and Credit

ABCs of GEP-NETs: Overview of Neuroendocrine Tumors

Jean-Yves Scoazec, MD

Jean-Yves Scoazec, MD