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1、Neuroendocrine and Adrenal Tumors Version 4 2018 January 07 2019 NCCN org NCCN Guidelines for Patients available at www nccn org patients NCCN Clinical Practice Guidelines in Oncology NCCN Guidelines Continue Version 4 2018 01 07 19 National Comprehensive Cancer Network Inc 2019 All rights reserved
2、The NCCN Guidelines and this illustration may not be reproduced in any form without the express written permission of NCCN Manisha H Shah MD Chair The Ohio State University Comprehensive Cancer Center James Cancer Hospital and Solove Research Institute Matthew H Kulke MD Chair Dana Farber Brigham an
3、d Women s Cancer Center Whitney S Goldner MD Vice Chair Fred CT with IV contrast when possible Footnote revised If disease progression treatment with octreotide or lanreotide should be continued in patients with functional tumors and may be used in combination with any of the subsequent options For
4、details on the administration of octreotide or lanreotide with 177Lu dotatate see NE E Footnote added where 177Lu dotatate is recommended See Principles of Peptide Receptor Radionuclide Therapy PRRT with lutetium 177 Lu dotatate 177Lu Dotatate NE E NET 8 Added to primary therapy with octreotide or l
5、anreotide if somatostatin receptor positive and or hormonal symptoms Also on NET 9 Added the following option for patients with clinically significant tumor burden and low grade typical bronchopulmonary thymus tumors or those with evidence of progression Consider PRRT with 177Lu dotatate if somatost
6、atin receptor positive and progression on octreotide lanreotide Also on NET 9 for intermediate grade atypical bronchopulmonary thymus tumors Moved treatment recommendations for those with intermediate grade atypical disease and those with multiple lung nodules or tumorletes and evidence of DIPNECH t
7、o NET 9 NET 10 Added the following option for patients with unresectable locoregional advanced disease of the GI tract and or distant metastases if disease progression following therapy with octreotide or lanreotide PRRT with 177Lu dotatate if somatostatin receptor positive category 1 for mid gut tu
8、mors Removed consider from the following options for those with disease progression following therapy with octreotide or lanreotide Hepatic directed therapy for hepatic predominant disease Interferon alfa 2b category 3 Cytotoxic chemotherapy category 3 if no other options feasible Added footnote hh
9、Treatment with octreotide or lanreotide will likely only benefit those patients who are somatostatin receptor positive Also on NET 11 NET 11 For those with poorly controlled carcinoid syndrome revised last option Consider other systemic therapy based on disease site PanNET 1 Footnote f revised Obser
10、vation can be considered for small 2 cm or incomplete resection the following option has been added Consider somatostatin receptor based imaging if equivocal CT findings ie gallium 68 dotatate PET CT preferred or somatostatin receptor scintigraphy NET 3 Small completely resected incidental tumors Ne
11、w pathways have been added for negative margins versus indeterminate margins For indeterminate margins Endoscopy has been added to assess for residual disease for those with indeterminate margins and low grade disease Refer to treatment pathway for all other rectal tumors if positive margins or inte
12、rmediate grade disease All other rectal tumors Evaluation options revised EndoRectal MRI or EUS endorectal ultrasound Surveillance EUS changed to Endorectal ultrasound NET 4 Footnote k revised Serum gastrin can be falsely elevated with proton pump inhibitor PPI use To confirm diagnosis it should ide
13、ally be checked NET 5 Added corresponding stage of disease to each pathway For locoregional disease following incomplete resection and or positive margins Options for low grade disease were revised Consider observation or consider RT category 3 systemic therapy Options for intermediate grade disease
14、 revised Consider observation or consider RT systemic therapy cisplatin etoposide or carboplatin etoposide For locoregional unresectable disease new pathways added for low grade and intermediate grade tumors Options for low grade include Consider observation or consider systemic therapy or consider
15、RT category 3 systemic therapy Options for intermediate grade include Consider RT systemic therapy or consider systemic therapy Footnote r added There is a gap issue and therapeutic challenge in managing patients who fall into this category due to a lack of data However the panel suggests use of the
16、se options in select cases or as needed NET 6 Under evaluation Added Other biochemical evaluation as clinically indicated Removed chromogranin A category 3 Clarified which pathways to follow for localized locoregional resectable locoregional unresectable and metastatic disease Adjuvant therapy options revised for Locoregional resectable disease intermediate grade Locoregional unresectable disease low grade Locoregional unresectable disease intermediate grade Footnote u added Bronchopulmonary neu
