NCCN Neuroendocrine Tumors Guidelines Are Divided Into 6 Endocrine Systems, Which Produce and Secrete Regulatory Hormones
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NCCN Clinical Practice Guidelines in Oncology™ Neuroendocrine Tumors V.1.2007 Continue www.nccn.org Guidelines Index ® Practice Guidelines Neuroendocrine TOC NCCN in Oncology – v.1.2007 Neuroendocrine Tumors MS, References NCCN Neuroendocrine Tumors Panel Members * Orlo H. Clark, MD/Chair ¶ John F. Gibbs, MD ¶ Thomas W. Ratliff, MD † UCSF Comprehensive Cancer Center Roswell Park Cancer Institute St. Jude Children's Research Hospital/University of Tennessee Jaffer Ajani, MD †¤ Martin J. Heslin, MD ¶ Cancer Institute The University of Texas M. D. University of Alabama at Anderson Cancer Center Birmingham Comprehensive Leonard Saltz, MD † Cancer Center Memorial Sloan-Kettering Cancer Al B. Benson, III, MD † Center Robert H. Lurie Comprehensive Fouad Kandeel, MD Cancer Center of Northwestern City of Hope Cancer Center David E. Schteingart, MD ð University University of Michigan Anne Kessinger, MD † Comprehensive Cancer Center David Byrd, MD ¶ UNMC Eppley Cancer Center at The Fred Hutchinson Cancer Research Nebraska Medical Center Manisha H. Shah, MD † Center/Seattle Cancer Care Alliance Arthur G. James Cancer Hospital & Matthew H. Kulke, MD † Richard J. Solove Research Gerard M. Doherty, MD ¶ Dana-Farber/Partners CancerCare Institute at The Ohio State University of Michigan Comprehensive University Cancer Center Larry Kvols, MD † H. Lee Moffitt Cancer Center & Stephen Shibata, MD † Paul F. Engstrom, MD † Research Institute at the University City of Hope Cancer Center Fox Chase Cancer Center of South Florida David S. Ettinger, MD † John A. Olson, Jr., MD, PhD ¶ The Sidney Kimmel Comprehensive Duke Comprehensive Cancer Cancer Center at Johns Hopkins Center * Writing Committee Member ¶ Surgery/Surgical oncology † Medical oncology ¤ Gastroenterology ð Endocrinology Continue Version 1.2007, 03/14/07 © 2007 National Comprehensive Cancer Network, Inc. All rights reserved. These guidelines and this illustration may not be reproduced in any form without the express written permission of NCCN. Guidelines Index ® Practice Guidelines Neuroendocrine TOC NCCN in Oncology – v.1.2007 Neuroendocrine Tumors MS, References Table of Contents NCCN Neuroendocrine Tumors Panel Members For help using these Neuroendocrine Tumors, Initial Workup (NE-1) documents, please click here Multiple Endocrine Neoplasia, Type 1 (NE-2) Multiple Endocrine Neoplasia, Type 2 (MEN2-1) Manuscript Carcinoid Tumors (NE-2) References Islet Cell Tumors (NE-2) Clinical Trials: The NCCN Pheochromocytoma (PHEO-1) believes that the best management Poorly Differentiated (High Grade or Anaplastic)/ for any cancer patient is in a clinical trial. Participation in clinical trials is Small Cell/Atypical Lung Carcinoids (ANAP-1) especially encouraged. Guidelines Index To find clinical trials online at NCCN Print the Neuroendocrine Tumors Guideline member institutions, click here: nccn.org/clinical_trials/physician.html NCCN Categories of Consensus: All recommendations are Category 2A unless otherwise specified. See NCCN Categories of Consensus Summary of Guidelines Updates These guidelines are a statement of consensus of the authors regarding their views of currently accepted approaches to treatment. Any clinician seeking to apply or consult these 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 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. These guidelines are copyrighted by National Comprehensive Cancer Network. All rights reserved. These guidelines and the illustrations herein may not be reproduced in any form without the express written permission of NCCN. ©2007. Version 1.2007, 03/14/07 © 2007 National Comprehensive Cancer Network, Inc. All rights reserved. These guidelines and this illustration may not be reproduced in any form without the express written permission of NCCN. Guidelines Index ® Practice Guidelines Neuroendocrine TOC NCCN in Oncology – v.1.2007 Neuroendocrine Tumors MS, References Summary of the Guidelines updates Summary of changes in the 2007 version of the Neuroendocrine Tumors Guidelines from the 2.2006 version include: · Lipomas and skin angiomas was added as a clinical presentation under Multiple Endocrine Neoplasia, Type 1 (NE-2). Multiple Endocrine Neoplasia, Type 1 · Observewas added as an option in the treatment of patients with u nresectable or extrahepatic metastases (MEN1-8 ). · For long term surveillance of MEN, Type 1, the time interval was removed from Octreoscan ().MEN1-9 Multiple Endocrine Neoplasia, Type 2 · “Genetic testing for germline mutations of the RET proto-oncogene” is recommended by the panel for all patients with medullary thyroid cancer(MEN2-2 ). · Hyperparathyroid: The panel recommended to evaluate for pheochromocytoma prior to an invasive procedure (MEN2-2 ). Carcinoid Tumors · Significant tumor burden was added as a category for metastatic disease (CARC-4 ). · Regional/mesenteric lymph nodes was added as acategory for metastatic disease (CARC-4 ). Islet Cell Tumors · For the treatment of bone metastases, after radiation therapy, bisphosphonates (category 2A for symptomatic, category 2B for asymptomatic) was added as a treatment option ().ISLT-4 · Footnote "d" was modified as “For patients with positive octreoscan, elevated biomarkers or secreting tumors”(ISLT-4 ). · Anti-proliferative effect is not well established in nonfunctional hormonal was removed as footnote from this page (ISLT-4 ). Pheochromocytoma · For resectable pheochromocytoma, ‘when safe and feasible’ was added to laparoscopically preferred ().PHEO-2 Version 1.2007, 03/14/07 © 2007 National Comprehensive Cancer Network, Inc. All rights reserved. These guidelines and this illustration may not be reproduced in any form without the express written permission of NCCN. UPDATES Guidelines Index ® Practice Guidelines Neuroendocrine TOC NCCN in Oncology – v.1.2007 Neuroendocrine Tumors MS, References CLINICAL DIAGNOSIS INITIAL WORKUPa See Primary Poorly differentiated Treatment (ANAP-1) Suspected/known Grade of c Well, moderately neuroendocrine FNAb differentiation differentiated tumor based on: or · b or Clinical Biopsy resection Localizing studies: manifestations based on location Specialized stainsd · Octreotide scan · Laboratory of lesion as or · CT, ultrasound, See Diagnosis studies clinically Laboratory studiesd endoscopic and Clinical · Presentation Imaging studies appropriate depending on tumor ultrasound, or MRI: · (NE-2) Family history suspected tumor directed · Bone scan if symptoms aSequence may vary. bRule out functioning adrenal neoplasms prior to biopsy. cWell, moderately, or poorly differentiated. Solcia E, Rindi G, Paolotti D, La Rosa S, Capella C, Fiocca R. Clinicopathological profile as a basis for classification of the endocrine tumors of the gastroenteropancreatic tract. Ann Oncol. 1999;10 Suppl 2:S9-15. dSee Stains and Laboratory Studies indicated in the Workup of Neuroendocrine Tumors (NE-A). Note: All recommendations are category 2A unless otherwise indicated. Clinical Trials: NCCN believes that the best management of any cancer patient is in a clinical trial. Participation in clinical trials is especially encouraged. Version 1.2007, 03/14/07 © 2007 National Comprehensive Cancer Network, Inc. All rights reserved. These guidelines and this illustration may not be reproduced in any form without the express written permission of NCCN. NE-1 Guidelines Index ® Practice Guidelines Neuroendocrine TOC NCCN in Oncology – v.1.2007 Neuroendocrine Tumors MS, References DIAGNOSIS AND CLINICAL PRESENTATIONS Multiple endocrine neoplasia, type 1 Clinical presentations: · Hyperparathyroidism (See MEN1-2) · Gastrinoma(See MEN1-1), Insulinoma, Glucagonoma (See MEN1-2) · VIPoma, Pancreatic polypeptidoma, Somatostatinoma, Nonfunctioning tumor (See MEN1-3) · Pituitary (See MEN1-4) · Adrenal gland tumor (See MEN1-7) · Liver metastases (See MEN1-8) · Thymus, Bronchial Carcinoid (See CARC-1) · Lipomas, skin angiomas Multiple endocrine neoplasia, type 2/medullary thyroid carcinoma (See MEN2-1) Carcinoid tumor Clinical presentations: · Thymus, Bronchial (See CARC-1) · Gastric (See CARC-2) · Appendix, Small bowel, Colon, Rectal (See CARC-3) · Recurrent or Metastatic disease (See CARC-4) Islet cell tumors Clinical presentations: · Gastrinoma (See ISLT-1) · Insulinoma, Glucagonoma, VIPoma (See ISLT-2) · Pancreatic polypeptidoma, Somatostatinoma (See ISLT-2) · Nonfunctioning pancreatic tumors (See ISLT-2) · Surveillance and recurrence (See ISLT-3) Pheochromocytoma/paraganglioma (See PHEO-1) Poorly differentiated/small cell/atypical lung carcinoid (See ANAP-1) Small cell carcinoma of the lung (See Small Cell Lung Cancer Guidelines) Merkel cell carcinoma (See Merkel Cell Carcinoma Guidelines) Note: All recommendations are category 2A unless otherwise indicated. Clinical Trials: NCCN believes that the best management of any cancer patient is in a clinical trial. Participation in clinical trials is especially encouraged. Version 1.2007, 03/14/07 © 2007 National Comprehensive Cancer Network, Inc. All rights reserved. These guidelines and this illustration may not be reproduced in any form without the express written permission of NCCN. NE-2 Neuroendocrine Tumors Guidelines Index ® Practice Guidelines Neuroendocrine TOC NCCN in Oncology – v.1.2007 Multiple Endocrine Neoplasia, Type 1 MS, References PARATHYROID, PITUITARY,