NCCN Guidelines®) Neuroendocrine and Adrenal Tumors Version 2.2018 — May 4, 2018

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NCCN Guidelines®) Neuroendocrine and Adrenal Tumors Version 2.2018 — May 4, 2018 NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Neuroendocrine and Adrenal Tumors Version 2.2018 — May 4, 2018 NCCN.org Continue Version 2.2018, 05/04/18 © National Comprehensive Cancer Network, Inc. 2018, All rights reserved. The NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN®. Printed by Diane Silverman on 7/10/2018 12:28:42 AM. For personal use only. Not approved for distribution. Copyright © 2018 National Comprehensive Cancer Network, Inc., All Rights Reserved. NCCN Guidelines Version 2.2018 NCCN Guidelines Index Table of Contents Neuroendocrine and Adrenal Tumors Discussion * Manisha H. Shah, MD/Chair † Thorvardur R. Halfdanarson, MD Þ † Leonard Saltz, MD † Þ The Ohio State University Comprehensive Mayo Clinic Cancer Center Memorial Sloan Kettering Cancer Center Cancer Center - James Cancer Hospital Daniel Halperin, MD † Julie Ann Sosa, MD ¶ ð and Solove Research Institute The University of Texas Duke Cancer Institute Matthew H. Kulke, MD/Chair † MD Anderson Cancer Center Jonathan R. Strosberg, MD † Dana-Farber/Brigham and Women’s Jin He, MD, PhD ¶ Moftt Cancer Center Cancer Center The Sidney Kimmel Comprehensive Cancer Craig A. Sussman, MD Þ * Whitney S. Goldner, MD/Vice Chair ð Center at Johns Hopkins Vanderbilt-Ingram Cancer Center Fred & Pamela Bufett Cancer Center Martin J. Heslin, MD ¶ Nikolaos A. Trikalinos, MD † Al B. Benson, III, MD † University of Alabama at Birmingham Siteman Cancer Center at Barnes-Jewish Robert H. Lurie Comprehensive Cancer Comprehensive Cancer Center Hospital and Washington University Center of Northwestern University Gregory P. Kalemkerian, MD/Liaison † School of Medicine Emily Bergsland, MD † University of Michigan Nataliya A. Uboha, MD, PhD † UCSF Helen Diller Family Comprehensive Cancer Center University of Wisconsin Comprehensive Cancer Center Fouad Kandeel, MD, PhD ð Carbone Cancer Center Jordan D. Berlin, MD † City of Hope Comprehensive Cancer Center Jonathan Whisenant, MD † Vanderbilt-Ingram Cancer Center Sajid A. Khan, MD ¶ Huntsman Cancer Institute Lawrence S. Blaszkowsky, MD † Yale Cancer Center/Smilow at the University of Utah Massachusetts General Hospital Cancer Hospital Terence Wong, MD ɸ Cancer Center Wajih Zaheer Kidwai, MD † Duke Cancer Institute Jennifer Chan, MD † Yale Cancer Center/Smilow James C. Yao, MD † Dana-Farber/Brigham and Women’s Cancer Hospital The University of Texas Cancer Center Pamela L. Kunz, MD † MD Anderson Cancer Center Jennifer Eads, MD † Þ Stanford Cancer Institute Case Comprehensive Cancer Center/ Boris W. Kuvshinof, II, MD, MBA ¶ University Hospitals Seidman Cancer Center NCCN Roswell Park Comprehensive and Cleveland Clinic Taussig Cancer Institute Jennifer Burns Cancer Center Ndiya Ogba, PhD Paul F. Engstrom, MD † Christopher Lieu, MD † Griselda Zuccarino-Catania, PhD Fox Chase Cancer Center University of Colorado Cancer Center Paul Fanta, MD † Venu G. Pillarisetty, MD ¶ ¶ Surgery/Surgical oncology UC San Diego Moores Cancer Center Fred Hutchinson Cancer Research Center/ † Medical oncology Thomas Giordano, MD, PhD ≠ Seattle Cancer Care Alliance ð Endocrinology University of Michigan ≠ Pathology Comprehensive Cancer Center Þ Internal medicine Continue f Nuclear medicine NCCN Guidelines Panel Disclosures * Discussion Section Writing Committee Version 2.2018, 05/04/18 © National Comprehensive Cancer Network, Inc. 2018, All rights reserved. The NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN®. Printed by Diane Silverman on 7/10/2018 12:28:42 AM. For personal use only. Not approved for distribution. Copyright © 2018 National Comprehensive Cancer Network, Inc., All Rights Reserved. NCCN Guidelines Version 2.2018 NCCN Guidelines Index Table of Contents Neuroendocrine and Adrenal Tumors Discussion NCCN Neuroendocrine Tumors Panel Members Clinical Trials: NCCN believes that Summary of the Guidelines Updates the best management for any patient with cancer is in a clinical trial. Clinical Presentations and Diagnosis (CP-1) Participation in clinical trials is Neuroendocrine Tumors of the Gastrointestinal Tract, Lung, and Thymus (Carcinoid Tumors) (NET-1) especially encouraged. To fnd clinical trials online at NCCN Neuroendocrine Tumors of the Pancreas (PanNET-1) Member Institutions, click here: Neuroendocrine Tumors of Unknown Primary (NUP-1) nccn.org/clinical_trials/physician.html. Adrenal Gland Tumors (AGT-1) NCCN Categories of Evidence and Consensus: All recommendations Pheochromocytoma/Paraganglioma (PHEO-1) are category 2A unless otherwise indicated. Poorly Diferentiated Neuroendocrine Carcinoma/Large or Small Cell (other than lung) (PDNEC-1) See NCCN Categories of Evidence Multiple Endocrine Neoplasia, Type 1 (MEN1-1) and Consensus. Multiple Endocrine Neoplasia, Type 2 (MEN2-1) Principles of Pathology for Diagnosis and Reporting of Neuroendocrine Tumors (NE-A) Principles of Biochemical Testing (NE-B) Surgical Principles for Management of Neuroendocrine Tumors (NE-C) Principles of Systemic Anti-Tumor Therapy (NE-D) Principles of Peptide Receptor Radionuclide Therapy (PRRT) with 177Lu-Dotatate (NE-E) Staging (ST-1) The NCCN Guidelines® are a statement of evidence and consensus of the authors regarding their views of currently accepted approaches to treatment. Any clinician seeking to apply or consult the NCCN Guidelines is expected to use independent medical judgment in the context of individual clinical circumstances to determine any patient’s care or treatment. The National Comprehensive Cancer Network® (NCCN®) makes no representations or warranties of any kind regarding their content, use or application and disclaims any responsibility for their application or use in any way. The NCCN Guidelines are copyrighted by National Comprehensive Cancer Network®. All rights reserved. The NCCN Guidelines and the illustrations herein may not be reproduced in any form without the express written permission of NCCN. ©2018. Version 2.2018, 05/04/18 © National Comprehensive Cancer Network, Inc. 2018, All rights reserved. The NCCN Guidelines® and this illustration may not be reproduced in any form without the express written permission of NCCN®. Printed by Diane Silverman on 7/10/2018 12:28:42 AM. For personal use only. Not approved for distribution. Copyright © 2018 National Comprehensive Cancer Network, Inc., All Rights Reserved. NCCN Guidelines Version 2.2018 NCCN Guidelines Index Table of Contents Neuroendocrine and Adrenal Tumors Discussion Updates in Version 2.2018 of the NCCN Guidelines for Neuroendocrine Tumors from Version 1.2018 include: Global NET-10 • Removed "if equivocal CT fndings" where somatostatin • Added the following option for patients with unresectable locoregional receptor-based imaging is recommended. advanced disease of the GI tract and/or distant metastases, if disease • Footnote revised: "Gallium-68 dotatate (68Ga-dotatate) progression following therapy with octreotide or lanreotide: "PRRT with 177Lu- PET/CT is more sensitive than somatostatin receptor dotatate, if somatostatin receptor positive (category 1 for mid-gut tumors)." scintigraphy for determining somatostatin receptor status. • Removed "consider" from the following options for those with disease PET/CT of skull base to mid-thigh; CT with IV contrast progression following therapy with octreotide or lanreotide: "Hepatic directed when possible..." therapy for hepatic-predominant disease; Interferon alfa-2b (category 3); • Footnote revised: "If disease progression, treatment with Cytotoxic chemotherapy (category 3), if no other options feasible." octreotide or lanreotide should be continued in patients • Added footnote "hh": "Treatment with octreotide or lanreotide will likely only with functional tumors and may be used in combination beneft those patients who are somatostatin receptor positive." (Also on NET- with any of the subsequent options. For details on the 11) 177 administration of octreotide or lanreotide with Lu- NET-11 dotatate, see NE-E." • For those with poorly controlled carcinoid syndrome, revised last option: 177 • Footnote added where Lu-dotatate is recommended : "Consider other systemic therapy based on disease site." "See Principles of Peptide Receptor Radionuclide Therapy (PRRT) with lutetium 177 Lu-dotatate (177Lu-Dotatate) (NE- PanNET-1 E)." • Footnote "f" revised: "Observation can be considered for small (<1 cm) low- grade, incidently discovered tumors. and low-grade tumors. Decision based..." NET-8 • Added to primary therapy with octreotide or lanreotide: PanNET-7 "(if somatostatin receptor positive and/or hormonal • Added the following option for patients with unresectable locoregional advanced pancreatic NET and/or distant metastases, if disease progression symptoms)." (Also on NET-9) 177 • Added the following option for patients with clinically following therapy with octreotide or lanreotide: "PRRT with Lu-dotatate, if signifcant tumor burden and low grade (typical) somatostatin receptor positive." bronchopulmonary/thymus tumors, or those with evidence PHEO-2 of progression: "Consider PRRT with 177Lu-dotatate • Added the following options for locally unresectable pheochromocytoma/ (if somatostatin receptor positive and progression on paraganglioma or those with distant metastases: "PRRT with 177Lu-dotatate (if octreotide/lanreotide)." (Also on NET-9 for intermediate somatostatin receptor positive)." grade [atypical] bronchopulmonary/thymus tumors) • Moved surveillance recommendations onto PHEO-3.
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