Index. Note: Page numbers of article titles are in boldface type.

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1 Note: Page numbers of article titles are in boldface type. A Abdominal pain, in neuroendocrine tumor syndromes, 20, Acromegaly, as neuroendocrine tumor presentation, 38 ACTH suppression studies, ACTH-secreting tumors, 26, 29 30, 33, 38 in children and young adults, surgery for, 165 Activities of daily living, neuroendocrine tumor syndromes and, QOL measurement of, Adenocarcinomas, chromogranin A elevation with, Adiponectin, obesity and, 83 Adiposity. See also Obesity. visceral, cardiometabolic risk syndrome and, 82 Adrenal tumors, chromogranin A and, 121 in children and young adults, 66 Amines, neuroendocrine tumors and, chromogranin A secretion and, 112, 114, 120 current recommendations for, 207, quantitative and qualitative analysis of, evolutionary advances in, 135, 143 glossary for, 147 history of, methodological differences in, 136 serotonin and tryptophan techniques, , 146 standardization in laboratories, Angiography, of neuroendocrine tumors, 49, 51 current recommendations for, 208, Antibodies, insulin-binding, 143 to chromogranin A, immunoassays of, immunohistochemical staining and, 117 Appendiceal neuroendocrine tumors, in children and young adults, 69, surgery for, 168 Appetite, bariatric surgery and, 85 obesity and, Asymptomatic primary neuroendocrine tumors, surgery indications for, 167 B Bariatric surgery, metabolic and cardiovascular consequences of, CMRS benefits of weight loss and, CMRS-specific, compared to non-surgical therapies, Endocrinol Metab Clin N Am 40 (2011) doi: /s (11) /11/$ see front matter ª 2011 Elsevier Inc. All rights reserved. endo.theclinics.com

2 234 Bariatric (continued) herbal therapy and, 84 lifestyle interventions and, metabolic-specific, obesity and metabolic health in, obesity epidemic perspectives in, obesity pathophysiology in, 83 obesity treatment goals and, pharmacologic therapy and, 84 summary overview of, 90 techniques for, Barium studies. See Upper GI series. Bioassays, in neuroendocrine tumor diagnosis, , Biochemical markers, of cardiometabolic risk syndrome, 82 of neuroendocrine tumors, as diagnostic, 38 39, 43 45, , 117 as prognostic, blood and urine, chromogranin A relevance as, See also Chromogranin A (CgA). classification according to use, current recommendations for, of Inter Science Institute Gastrointestinal Council, of New Orleans Louisiana Neuroendocrine Tumor group, for bone metastasis diagnosis, 46 for cardiac involvement diagnosis, 46 hormonal, in children and young adults, preoperative evaluation of, 226 QOL measurement and, of paragangliomas, 30, 41 of pheochromocytomas, 30, 41 Biopsy, for liver metastasis, with neuroendocrine tumors, 51 Body fat mass, after weight loss, 82 Body mass index (BMI), obesity and, 81 Bone metastasis, of neuroendocrine tumors, biochemical markers for, 46 imaging of, in children and young adults, Breast carcinoma, chromogranin A elevation with, in children and young adults, 65 66, 68 diagnosis and therapy for, 70, 72 Bronchial carcinoid tumors, imaging of, 157 in children and young adults, 69 70, 72 Bronchoconstriction, in neuroendocrine tumor syndromes, 20, 26 C Calcitonin-secreting tumors, surgery for, 165 Calcium stimulation test, for hypoglycemia, after bariatric surgery, 89 Cancer clinical trials, patient-reported QOL outcomes in, 99 Cancer registry, U.S., of GEP-NETs. See Surveillance, Epidemiology and End Results (SEER) program.

3 Cancers, endocrine/neuroendocrine. See also specific anatomy or type, e.g., Lung carcinoma. in children and young adults, classification of, 65 67, diagnosis and therapy for, epidemiology of, future directions for, non-neuroendocrine, chromogranin A elevation with, Capsule endoscopy, for neuroendocrine tumor diagnosis, 52, 208, Carcinoembryonic antigen (CEA), in neuroendocrine tumor diagnosis, chromogranin A vs., Carcinoid crisis, prevention during surgery, for GEP-NETs, 169, Carcinoid syndrome/tumors, bronchial, imaging of, 157 in children and young adults, 69 70, 72 classification of, 206 clinical presentations of, 20, 26 diagnosis of, 23 24, 27, radioimmunoassays for, 137 gastric types of, 164. See also Gastric neuroendocrine tumors. in children and young adults, surgery for, 164, 166, 168 imaging of, 20, 26, 36, 46 47, in children and young adults, 65 66, 68 bronchial, 69 70, 72 gastric, natural history of, prevalence of, 19, 21 QOL measurement with, , 107 surgery for, gastric, 164, 166, 168 targeted radiopeptide therapy for, 195 Carcinomas. See Cancer entries; specific type. Cardiac involvement, with neuroendocrine tumors, biochemical markers of, 46 Cardiometabolic risk syndrome (CMRS), bariatric surgery and, dyslipidemia and, 86 hypertension and, 86 NAFLD and, PCOS and, 87 T2DM complications and, 87 benefits of weight loss on, definition of, obesity and, pathophysiology of, 83 Cardiovascular consequences, of bariatric surgery, cardiometabolic risk syndrome as, benefits of weight loss on, dyslipidemia and, 86 hypertension and, 86 NAFLD and, PCOS and, 87 T2DM complications and, 87 compared to non-surgical therapies, herbal therapy and,

4 236 Cardiovascular (continued) lifestyle interventions and, metabolic-specific, obesity and metabolic health in, obesity epidemic perspectives in, obesity pathophysiology in, 83 obesity treatment goals and, pharmacologic therapy and, 84 summary overview of, 90 of neuroendocrine tumor syndromes, QOL measurement with, Carney complex, 65 C-cell hyperplasia, 42 Cecal neuroendocrine tumors, surgery for, 168 Cervical carcinoma, small cell, in children and young adults, 66, 69, 71, 74 Chemoembolization, for neuroendocrine tumors, per Inter Science Institute Gastrointestinal Council, 224 per New Orleans Louisiana Neuroendocrine Tumor group, 225 Chemotherapy, for neuroendocrine tumors, chemoembolization combined with, current recommendations on, 216, 219, 229 per Inter Science Institute Gastrointestinal Council, 220 per New Orleans Louisiana Neuroendocrine Tumor group, 221 radionuclide therapies combined with, 219, rare functioning pancreatic, 165 Children and young adults, neuroendocrine tumors in, appendiceal, jejunal, and ileal, 69, breast carcinoma as, 65 66, 70, 72 bronchial carcinoid, 69 70, 72 classification of, colon and rectal, 69, diagnosis and therapy for, distribution of, epidemiology of, future directions for, gastric carcinoid, 65 66, 68, hindgut, 66, 69, 71, incidence of, late diagnosis of, lung carcinoma as, 65 66, 68 70, 72 midgut, 66, 69 70, molecular genetics of, 70 71, 75 neural crest, 65 68, neuroblastoma as, 65 67, ovary and cervix, 65 66, 69, 71, 74 pancreatic, 66 67, 71, 73 paraganglioma as, 74 pathology report data sets for, pheochromocytoma as, 65 67, 74 prevalence of, 68 summary overview of, 65, survival of, 67

5 thyroid carcinoma as, 65 66, 70, 72 Chlorozotocin, for neuroendocrine tumors, 216 Cholecystectomy, for neuroendocrine tumors, 166, 226 Cholelithiasis, in neuroendocrine tumor syndromes, 20, 226 DVT, steatorrhea, neurofibromatosis with, 20 somatostatinoma and, 34 Chromatography, in neuroendocrine tumor diagnosis, Chromogranin A (CgA), as gastroenteropancreatic neuroendocrine tumor marker, as diagnostic, , rationale for, as prognostic, cleavage to other peptides and, 114 clinical utility of, as tumor burden measurement, as tumor marker, comparison to alternative tumor markers, concentration fluctuations and, 121 relapse identification as, 124 response to treatment correlations as, 124 sensitivity and specificity of, granin family and, 112, 114, measurement of, immunoassays for, immunohistochemical staining for, 117 platforms for blood, 116 platforms for tissue, 117 neuroendocrine cell biology and, 112 neuroendocrine cell types and secretion of, normal cellular biology vs., pathologic elevation and, iatrogenic causes of, 119 malignant but non-neuroendocrine causes of, nonmalignant causes of, peptide fragments and, physiologic role of, summary overview of, 125 in neuroendocrine tumor diagnosis, 21 23, for children and young adults, pancreatic, QOL measurement with, , 106 tumor type specificity, 43 45, 206 in neuroendocrine tumor prognosis, 45, Chromogranin A (CgA)-secretogranin (Sg) proteins, as neuroendocrine tumor marker, Chromogranin B (CgB), in neuroendocrine tumor diagnosis, Chronic conditions, aggravated by obesity, chromogranin A elevation with, [ 11 C]5-HTP positron emission tomography (HTP-PET), of neuroendocrine tumors, 48 Classification system, for neuroendocrine tumors, 27 28, 66 current recommendations for, 206, 222 gastroenteropancreatic, 2,

6 238 Classification (continued ) ICD-O-3 codes, 4 5 TRT patient selection based on, Clinical trials, patient-reported QOL outcomes in, with neuroendocrine tumors, 99 CMRS. See Cardiometabolic risk syndrome (CMRS). Colon neuroendocrine tumors, chromogranin A elevation with, 119 in children and young adults, 69, surgery for, 168 Colorimetry, in neuroendocrine tumor diagnosis, 136, 144 Combination chemotherapy, for neuroendocrine tumors, 229 chemoembolization in, pharmacologic agents and radionuclide therapies in, 219, pharmacologic agents in, 216, Computed tomography (CT), of neuroendocrine tumors, 36, 47, 50 51, cross-sectional imaging techniques for, current recommendations for, 208, in children and young adults, 69 pancreatic, 49 Constipation, in neuroendocrine tumor syndromes, QOL measurement with, 100 Cough, in neuroendocrine tumor syndromes. See also Bronchoconstriction. QOL measurement with, 100 C-reactive protein (CRP), bariatric surgery and, 89 obesity and, 82 Cushing syndrome, 30, 38 Cytoreduction, for neuroendocrine tumors, , 229 rare functioning pancreatic, 165 Cytotoxic therapy. See Chemotherapy. D Debulking, surgical, for neuroendocrine tumors, , 223, Dementia, in neuroendocrine tumor syndromes, 20 Depression, with neuroendocrine tumor syndromes, QOL measurement of, Dermatitis, in neuroendocrine tumor syndromes, 20, 26 QOL measurement with, 100 Diabetes mellitus, in neuroendocrine tumor syndromes, 20, 34 obesity and. See Type 2 diabetes mellitus (T2DM). somatostatinoma and, 34 Diagnosis, of neuroendocrine tumors, algorithm for, 38 39, impediments to, techniques for, 2, 19, 27. See also specific presentation or syndrome, e.g., Flushing. Diarrhea, in neuroendocrine tumor syndromes, abdominal pain and dyspepsia with, 20, QOL measurement with, 100 steatorrhea with, 20, 34 secretory, 25

7 239 somatostatinoma and, 34 Diet modifications, for obesity, 82 83, 86 post-gastric bypass, 89 Diffuse neuroendocrine system (DNES), 112 chromogranin A secretion and, GEP-NETs originating from, Disease-specific questionnaires, for QOL measurement, with neuroendocrine tumors, description of, , 107 development of, 100 study methods for completing, results, scales and scores, statistical analysis, 102 summary discussion on, F-18 Dopamine positron emission tomography, of neuroendocrine tumors, 156, Dosimetry, in targeted radiopeptide therapy, for neuroendocrine tumors, , DOTANOC positron emission tomography, 177 LU-DOTATOC, in targeted radiopeptide therapy, 188 of neuroendocrine tumors, 156 DOTATATE positron emission tomography, of neuroendocrine tumors, 156, 158 DOTATOC, radiolabeled, in targeted radiopeptide therapy. See 177 LU-DOTATOC; 90 Y-DOTATOC. DOTATOC positron emission tomography, of neuroendocrine tumors, 52, 156, Doxorubicin, for neuroendocrine tumors, 216, radionuclide therapies combined with, Dumping syndrome, in neuroendocrine tumor syndromes, 26 late-phase, after bariatric surgery, 88 Duodenal neuroendocrine tumors, surgery for, poorly differentiated, 166 DVT, in neuroendocrine tumor syndromes, 20 steatorrhea, cholelithiasis, neurofibromatosis with, 20 Dyslipidemia, bariatric surgery and, 86 cardiometabolic risk syndrome and, 82 Dyspepsia, in neuroendocrine tumor syndromes, 20, ulcer with, 20, 26 E Electron chemical (EC) detector, HPLC, in neuroendocrine tumor diagnosis, 138, 142, 144 Electron impact ionization (EI), in radioimmunoassays, 140 Electrospray ionization (ESI), in radioimmunoassays, 141 Embolic therapy, for neuroendocrine tumors, per Inter Science Institute Gastrointestinal Council, 224 per New Orleans Louisiana Neuroendocrine Tumor group, 225 End Results Group (ERG), GEP-NETs data from, 2 Endocrine disorders, radioimmunoassays as catalyst for study of, 147

8 240 Endocrine (continued ) somatostatinoma association with, 35 Endocrine tumors. See also Neuroendocrine tumors (NETs). in children and young adults, Endoscopic ultrasound (EUS), for neuroendocrine tumor diagnosis, 46 47, 51 current recommendations for, 208, gastric carcinoid, 164 in MEN-1 syndrome, 165 pancreatic, 49 50, 165 rectal carcinoid, 168 Endoscopy, for neuroendocrine tumor diagnosis, 36, 51 capsule, 52 current recommendations for, 208, Enteroscopy, for neuroendocrine tumor diagnosis, 208, Enzyme-linked immunosorbent assay (ELISA), for chromogranin A measurement, 116 EORTC QLQ-C30 GI.NET-21 questionnaire, description of, 100, 107 development of, study methods for completing, results, scales and scores, statistical analysis, 102 summary discussion on, European Organization for Research and Treatment of Cancer (EORTC), patient-recorded outcome measurements, in cancer clinical trials, 99. See also EORTC QLQ-C30 GI.NET-21 questionnaire. Ewing sarcoma, in children and young adults, Excision, of gastroenteropancreatic neuroendocrine tumors, 163, 223, See also Surgery. Extent of disease. See Tumor burden. External beam radiation, for neuroendocrine tumors, radio-sensitizers for, 197 F Familial endocrine tumors, in children and young adults, Fatigue, in neuroendocrine tumor syndromes, QOL measurement with, 100 Fatty acid metabolism, obesity and, FDA-positron emission tomography (FDA-PET), of neuroendocrine tumors, 50 FDG-positron emission tomography (FDG-PET), of neuroendocrine tumors, 48, 50, 52, 156, 158, 195 current recommendations for, FDOPA-positron emission tomography (FDOPA-PET), of neuroendocrine tumors, 50, 156, HIAA. See 5-Hydroxyindoleacetic acid (5-HIAA). Fluorometry, in neuroendocrine tumor diagnosis, , 141, Fluorouracil, for neuroendocrine tumors, 216, radionuclide therapies combined with, Flushing, in neuroendocrine tumor syndromes, 20, differential diagnosis of, 23

9 241 QOL measurement with, Foregut, poorly differentiated neuroendocrine tumors of, surgery for, 166 Free fatty acids (FFAs), obesity and, 81, 83 G Ga-68 DOTATOC positron emission tomography, of neuroendocrine tumors, 156, Gallbladder disease. See Cholelithiasis. Ganglion tumors, peripheral, in children and young adults, Gas chromatography (GC), in neuroendocrine tumor diagnosis, 139, 143 Gas chromatography/mass spectrometry (GC/MS), in neuroendocrine tumor diagnosis, , 142, 145 Gastric banding, laparoscopic adjustable, 84 Gastric bypass. See Bariatric surgery. Gastric neuroendocrine tumors. See also specific anatomy. biomarkers of, clinical syndrome presentations of, diagnosis of, 27 imaging of, 36, 46 47, immunohistochemistry of, 28 in children and young adults, 65 66, 68, molecular genetics of, 28, 75 pathology of, prevalence of, 19, 21 special pathologic consideration of, 28 surgery for, See also Surgery. Gastrin, in neuroendocrine tumor diagnosis, 20, 27, 38 39, 164 for children and young adults, 71 Gastrinoma, 29, 32, 113, 123 somatostatin analogs for, 216 surgery for, 165 Gastroenteropancreatic neuroendocrine tumors (GEP-NETs), biochemical diagnosis of, cellular origins of, , 164 chromogranin A relevance to, as diagnostic, , as prognostic, cleavage to other peptides and, 114 clinical utility of, as tumor burden measurement, as tumor marker, comparison to alternative tumor markers, concentration fluctuations and, 121 relapse identification as, 124 response to treatment correlations as, 124 sensitivity and specificity of, granin family and, 112, 114 measurement of, immunoassays for, platforms for blood, 116 platforms for tissue, 117

10 242 Gastroenteropancreatic (continued ) neuroendocrine cell biology and, 112 neuroendocrine cell types and secretion of, normal cellular biology vs., pathologic elevation and, iatrogenic causes of, 119 malignant but non-neuroendocrine causes of, nonmalignant causes of, peptide fragments and, physiologic role of, summary overview of, 125 classification of, 2, 195 diagnostic techniques for, 2 epidemiology of, 1 18 SEER discussion in, SEER incidence in, 2 SEER methods in, 2 5 SEER results in, 5 15 summary overview of, 1 2, 16 etiologies of, 1 frequency by primary sites, 5 7 characteristics of common, 11 geographic distribution of, 2 3, 16 incidence of, 2 age-adjusted, 5, 8 9, 12 changes in age-adjusted, 5, 10 diagnostic stage and grade influence on, 11 13, mean age-adjusted, 5, 11 persistent increase in, peptide receptor radionuclide therapy for, affinity profiles for sst 1 sst 5 receptors of series of somatostatin analogs, 177 options to improve, quality of life with, 179 retreatment with, summary overview of, , 182 survival data comparisons, 180 timing of, 180 tumor response to different radiolabeled somatostatin analogs, 175 with [ 111 Indium-diethylenetriamine pentaacetic acid 0 ] octreotide, 174 with [ 177 Lutetium-dota 0, tyr 3 ] octreotate, comparison to other PRRT, 177, with [ 90 Yttrium-dota 0, tyrosine 3 ] octreotide, 174, 176 prevalence of, vs. neuroendocrine tumors, 19, 21 somatostatin receptor radionuclide therapy for, See also Peptide receptor radionuclide therapy (PRRT). surgery for, appendiceal tumors, 168 asymptomatic primary tumors, 167 carcinoid crisis with, 169, carcinoid tumors, 164, 166, 168 colonic tumors, 168

11 243 foregut poorly differentiated tumors, 166 hepatic metastases indications in, 168 jejunoileum tumors, 166 liver transplantation as, 169, lymphatic mapping and, 167 MEN-1 syndrome tumors, midgut tumors, 166 pancreatic tumors, poorly differentiated, 166 rare functioning, 165 rectal tumors, 168 summary overview of, , 170 survival rates with, 2, annual percentage change in, 4 5, 13, 15 chromogranin A prediction of, 123 geographic associations of, 16 peptide receptor radionuclide therapy comparisons, 180 percentage change in, 5, 14 skewing based on classification/grading systems, year rates, 11 12, Gastrointestinal symptoms, in neuroendocrine tumor syndromes. See also specific symptom, e.g., Diarrhea. QOL measurement with, Gastroplasty, vertical banded, Genetic testing, for neuroendocrine tumors, Genetics, molecular, of neuroendocrine tumorigenesis, 28 in children and young adults, 70 71, 75 of pheochromocytomas, 40 of chromogranin A secretion, 114 GEP-NETs. See Gastroenteropancreatic neuroendocrine tumors (GEP-NETs). Ghrelin, bariatric surgery and, 85 obesity and, 82 Gigantism, as neuroendocrine tumor presentation, 38 GLP-1, bariatric surgery effect on, 88 Glucagonoma, 29, 33, 113 somatostatin analogs for, 216 surgery for, 165 Grading system, for neuroendocrine tumors, 5, 27 28, 66, 222 gastric, 11 13, TRT patient selection based on, Granin family, chromogranin A secretion and, 112, 114, Growth hormone releasing factor secreting tumors (GRFomas), 29, 37 in children and young adults, H Health status (HQOL), patient-reported outcomes of, with neuroendocrine tumors, 99 Hematological toxicity, of targeted radiopeptide therapy, for neuroendocrine tumors, 193 Hepatic metastases, of neuroendocrine tumors. See Liver metastases.

12 244 Hepatocellular cancer (HCC), chromogranin A elevation with, 119 Herbal therapy, for weight loss, 84 High-performance liquid chromatography (HPLC), in neuroendocrine tumor diagnosis, , Hindgut tumors, neuroendocrine, in children and young adults, 66, 69, 71, surgery for, 168 History taking, for neuroendocrine tumor surgery, 226 Hormonal gradients, assessment for neuroendocrine tumors, 147 current recommendations for, 206, in children and young adults, pancreatic, 50 Hormones, neuroendocrine tumor syndromes associated with, See also specific hormone, e.g., Chromogranin A (CgA). in children and young adults, radioimmunoassays of, 147 HPLC-electron chemical (EC) detector, in neuroendocrine tumor diagnosis, 138, 142, 144 HPLC-fluoresence, in neuroendocrine tumor diagnosis, , 144 HQOL (health status), patient-reported outcomes of, with neuroendocrine tumors, 99 [ 11 C]5-HTP positron emission tomography (HTP-PET), of neuroendocrine tumors, 48, 52 5-Hydroxyindoleacetic acid (5-HIAA), in neuroendocrine tumor diagnosis, 23 24, 26 27, 29 algorithm, chromogranin A vs., current recommendations for, QOL measurement with, , 106 radioimmunoassays of, , 141, 143 Hypertension, bariatric surgery and, 86 cardiometabolic risk syndrome and, 82 Hypochlorhydria, in neuroendocrine tumor syndromes, 35 somatostatinoma and, 34 Hypoglycemia, after bariatric surgery, clinical syndrome of, evaluation of, 89 pathophysiology of, prognosis for, 90 treatment of, 89 in neuroendocrine tumor syndromes, 20, 26, 34 somatostatinoma and, 34 I ICD-O-3 morphology codes, for neuroendocrine tumors, 4 5 Ileal neuroendocrine tumors, in children and young adults, 69, surgery for, 166 lymphatic mapping during, 167 Ileocecal valve, intraoperative sparing of, with neuroendocrine tumor resection, 167 Imaging studies, of neuroendocrine tumors, See also specific technique. asymptomatic primary, 167

13 245 carcinoid, 20, 26, 36, 46 47, computed tomography as, , 159 cross-sectional techniques for, current recommendations for, of Inter Science Institute Gastrointestinal Council, 214 of New Orleans Louisiana Neuroendocrine Tumor group, 215 DOPA-positron emission tomography as, 156, 159 DOTANOC positron emission tomography as, 156 DOTATATE positron emission tomography as, 156, 158 F-18 dopamine positron emission tomography as, 156, FDG-positron emission tomography as, 156, Ga-68 DOTATOC positron emission tomography as, 156, in children and young adults, 69 In-111 pentetreotide scintigraphy as, 155, See also Octreoscan. magnetic resonance imaging as, , 159 MIBG scintigraphy as, 47, 50, 52 midgut, 47, 52 nuclear techniques for, pancreatic, paragangliomas, pheochromocytoma, positron emission tomography as, 156, preoperative recommendations for, 226, 229 somatostatinoma, SPECT/CT as, 154, summary overview of, 51 52, 153, 159 tumor-specific techniques, Immunoassays, for chromogranin A measurement, comparison to alternative tumor markers, radiolabeled, for neuroendocrine tumors, See also Radioimmunoassays. Immunohistochemistry, in neuroendocrine tumor diagnosis, 19, 28, chromogranin A and, 117 current recommendations for, 206 for children and young adults, in somatostatinoma diagnosis, 34, In-111 pentetreotide scintigraphy, for neuroendocrine tumor diagnosis, 69, 155, , 167. See also Octreoscan. Incretins, bariatric surgery effect on, Indium-DTPA (diethylenetriamine pentaacetic acid 0 ) octreotide scintigraphy, for neuroendocrine tumors, 47 49, In-DOTABASS, in targeted radiopeptide therapy, In-octreotide, in targeted radiopeptide therapy, 188 clinical studies of, diagnostic use vs., future developments vs., prognostic use vs., In-octreotide scintigraphy, See also Somatostatin receptor scintigraphy (SRS). Insulin production, bariatric surgery effect on, 85, Insulin resistance, obesity and, Insulin-binding antibodies, 143

14 246 Insulinoma, 29, 31, 38, 113 post-gastric bypass, 89 somatostatin analogs for, 216 surgery for, Inter Science Institute Gastrointestinal Council recommendations, on neuroendocrine tumors, biochemical markers, chemotherapy, 220 imaging studies, 214 surgery, 227 symptomatic treatment, 217 Interferon-a, for neuroendocrine tumors, 219 Internal radiotherapy, for neuroendocrine tumors, See also Targeted radiopeptide therapy (TRT). clinical studies of, current recommendations for, 219, 222, dosing considerations of, side effects and toxicity of, International Classification of Diseases for Oncology, Third Edition (ICD-O-3) codes, for neuroendocrine tumors, 4 5 Intraoperative ultrasonography (IUS), for pancreatic neuroendocrine tumor localization, 49 50, 165 Irritable bowel syndrome (IBS), neuroendocrine tumor vs., Islet cell tumors, pancreatic. See also specific tumor, e.g., Gastrinoma. classification of, 206 current treatment recommendations for, in children and young adults, 66 67, 71, 73 surgery for, Islet cells, pancreatic, bariatric surgery effect on, 85, Isotopes, in targeted radiopeptide therapy, clinical studies of, current recommendations for, 219, 222, future developments of, J Jejunal neuroendocrine tumors, in children and young adults, 69, surgery for, 166 K Ki-67, in neuroendocrine tumor diagnosis, 28, 206 L Lanreotide, for symptomatic neuroendocrine tumors, 209, in targeted radiopeptide therapy, 188 clinical studies of, current recommendations for, Laparoscopic adjustable gastric banding (LAGB), 84 Laxative abuse, 25 Leptin, obesity and, 83

15 247 Lifestyle interventions, for weight loss, Liquid chromatography (LC), high-performance, in neuroendocrine tumor diagnosis, , ultra high-performance, in neuroendocrine tumor diagnosis, 138, 141 Liquid chromatography/mass spectrometry (LC/MS), in neuroendocrine tumor diagnosis, , 145 Liver metastases, of neuroendocrine tumors, chromogranin A prediction of, 123 imaging of, 47 48, 51, , in children and young adults, liver transplantation for, 169, surgery for, 166, 168 targeted radiopeptide therapy and, 190, Liver resection, for metastatic GEP-NETs, 168 Liver toxicity, of targeted radiopeptide therapy, for neuroendocrine tumors, 194 Liver transplantation, for unresectable metastatic GEP-NETs, 169, LU-DOTATATE, for GEP-NETs, comparison to other PRRT, 177, survival with, 180 in targeted radiopeptide therapy, 188 clinical studies of, current recommendations for, future developments of, 197 prognosis with, 196 toxicity of, LU-DOTATOC, in targeted radiopeptide therapy, 188 clinical studies of, Lung carcinoma, imaging of, 157 in children and young adults, 65 66, 68 diagnosis and therapy for, 69 70, 72 small cell, chromogranin A and, Lymph node dissection, in surgery, for GEP-NETs, 166, 226 Lymphatic mapping, in surgery, for GEP-NETs, 167, 229 M Magnetic resonance imaging (MRI), of neuroendocrine tumors, 47, 50, cross-sectional imaging techniques for, current recommendations for, , in children and young adults, 69 pancreatic, 49 Malignancy(ies), chromogranin A elevation with, as neuroendocrine tumor marker, with non-neuroendocrine disorders, with neuroendocrine tumors. See also specific anatomy or type, e.g., Medullary carcinoma of the thyroid (MCT). in children and young adults, 66, 68 surgery for, , 229. See also Surgery. with somatostatinoma, 35 Mass spectrometry (MS), in neuroendocrine tumor diagnosis, gas chromatography with, , 142, 145

16 248 Mass (continued) liquid chromatography with, , 145 Medullary carcinoma of the breast, in children and young adults, diagnosis and therapy for, 70, 72 Medullary carcinoma of the thyroid (MCT), 42 chromogranin A and, 120 in children and young adults, 65 66, 70, 72 Melanomas, in children and young adults, Melatonin, in neuroendocrine tumor diagnosis, Mendelian inheritance, of endocrine tumors, 65 Merkel cell carcinoma of the skin, chromogranin A and, Metabolic health effect, of bariatric surgery, CMRS benefits of weight loss and, CMRS-specific vs., compared to non-surgical therapies, herbal therapy and, 84 hypoglycemia as, clinical syndrome of, evaluation of, 89 pathophysiology of, prognosis for, 90 treatment of, 89 lifestyle interventions and, obesity and, obesity epidemic perspectives in, obesity pathophysiology in, 83 obesity treatment goals and, pharmacologic therapy and, 84 summary overview of, 90 Metastatic disease, in neuroendocrine tumor syndromes, biopsy for, 51 bone. See Bone metastasis. chromogranin A prediction of, 123 imaging studies of, 47 48, , current recommendations for, , in children and young adults, liver. See Liver metastases. lymphatic mapping of, 167 QOL measurement with, , 106 targeted radiopeptide therapy and, 190, with rare functioning pancreatic tumors, I MIBG (metaiodobenzylguanidine) radionucleotide therapy, for neuroendocrine tumors, current recommendations for, 222, I MIBG (metaiodobenzylguanidine) scintigraphy, of neuroendocrine tumors, 47, 154 carcinoid, I MIBG (metaiodobenzylguanidine) scintigraphy, of neuroendocrine tumors, 47, 50, 52, Midgut tumors, neuroendocrine, blood and urine biomarkers of, imaging of, 47, 52, 157 current recommendations for, 208, for initial staging, 47

17 249 in children and young adults, 66, 69 70, special pathologic consideration among, 28 surgery for, 166 Molecular genetics, of neuroendocrine tumorigenesis, 28 in children and young adults, 70 71, 75 of pheochromocytomas, 40 Morphology codes, ICD-O-3, for neuroendocrine tumors, 4 5 mrna, chromogranin A secretion and, 114 Multiple endocrine neoplasia type 1 (MEN-1) syndrome, 31 32, 37 38, 40 chromogranin A elevation with, 121, 123 in children and young adults, 65 66, somatostatinoma association with, 35 surgery for neuroendocrine tumors with, Multiple endocrine neoplasia type 2 (MEN-2) syndrome, 35, 40, 43 N NAFLD. See Nonalcoholic fatty liver disease (NAFLD). NANETS algorithm, for neuroendocrine tumor evaluation, 38 39, National Cancer Institutes (NCIs), U.S., GEP-NETs studies of, 2. See also Surveillance, Epidemiology and End Results (SEER) program. HQOL outcome studies of, with neuroendocrine tumors, 99 Negative chemical ionization (NCI), in radioimmunoassays, NETs. See Neuroendocrine tumors (NETs). Neural crest tumors, in children and young adults, 65 68, Neuroblastoma, in children and young adults, 65 67, Neuroendocrine cells, chromogranin A immunohistochemical staining in, 117 chromogranin A secretion and, , 120 GEP-NETs originating from, , 164 normal biology of, 112 Neuroendocrine tumors (NETs). See also specific anatomy or tumor, e.g., Gastric neuroendocrine tumors. acromegaly presentation with, 38 biochemical markers of, 19. See also specific hormone. as diagnostic, 43 45, , 117 as prognostic, blood and urine, classification according to use, current recommendations for, of Inter Science Institute Gastrointestinal Council, of New Orleans Louisiana Neuroendocrine Tumor group, for bone metastasis diagnosis, 46 for cardiac involvement diagnosis, 46 hormonal, in children and young adults, QOL measurement and, C-cell hyperplasia as, 42 chemotherapy for, 216, 219, 229 chemoembolization combined with, per Inter Science Institute Gastrointestinal Council, 220 per New Orleans Louisiana Neuroendocrine Tumor group, 221

18 250 Neuroendocrine (continued ) radionuclide therapies combined with, 219, classification, grading, and staging system for, 27 28, 66 current recommendations for, 206 TRT patient selection based on, current recommendations for, biochemical markers, , chemoembolization, chemotherapy, 216, , 229 imaging, , radionuclide therapies, 219, 222, summary overview of, 206 surgery, 223, symptomatic treatment, 209, tumor classification, 206 Cushing syndrome and, 38 description of, 19 diagnostic algorithm for, 38 39, diagnostic impediments of, diagnostic techniques for, 2, 19, 27. See also specific presentation or syndrome, e.g., Flushing. gastroenteropancreatic. See Gastroenteropancreatic neuroendocrine tumors (GEP-NETs). gigantism presentation with, 38 imaging studies of, See also specific technique. asymptomatic primary, 167 computed tomography as, , 159 cross-sectional techniques for, current recommendations for, of Inter Science Institute Gastrointestinal Council, 214 of New Orleans Louisiana Neuroendocrine Tumor group, 215 DOPA-positron emission tomography as, 156, 159 DOTANOC positron emission tomography as, 156 DOTATATE positron emission tomography as, 156, 158 F-18 dopamine positron emission tomography as, 156, FDG-positron emission tomography as, 156, Ga-68 DOTATOC positron emission tomography as, 156, gastric carcinoid, In-111 pentetreotide scintigraphy as, 155, See also Octreoscan. magnetic resonance imaging as, , 159 MIBG scintigraphy as, 47, 50, 52 midgut, 47, 52 nuclear techniques for, pancreatic, paragangliomas, pheochromocytoma, positron emission tomography as, 156, preoperative recommendations for, 226, 229 somatostatinoma, SPECT/CT as, 154, summary overview of, 51 52, 153, 159

19 tumor-specific techniques, in children and young adults, See also specific anatomy or type, e.g., Breast carcinoma. classification of, diagnosis and therapy for, distribution of, epidemiology of, future directions for, incidence of, late diagnosis of, prevalence of, 68 summary overview of, 65, survival of, 67 incidence of, annual, 19 over time, 19, 21 International Classification of Diseases codes for, 4 5 MEN syndromes associated with. See Multiple endocrine neoplasia type 1 (MEN-1) syndrome; Multiple endocrine neoplasia type 2 (MEN-2) syndrome. molecular genetics of, 28, 40, 70 71, 75 new and emerging syndromes due to, abdominal pain as, 20, clinical presentations per tumor type/site and hormones, dementia as, 20 dermatitis as, 20, 26 diarrhea as, abdominal pain and dyspepsia with, 20, steatorrhea with, 20, 34 dyspepsia as, 20, ulcer with, 20, 26 flushing as, 20, differential diagnosis of, 23 gastric, See also Gastric neuroendocrine tumors. hypoglycemia as, 20, 26, 34 imaging studies of, pancreatic, See also Pancreatic neuroendocrine tumors (PNETs). summary overview of, 19, 52 ulcer as, dyspepsia with, 20, 26 wheezing as, 20, 26, 69 pathology of, 27 midgut considerations, 28 primary, asymptomatic, surgery indications for, 167 gastroenteropancreatic, 5 7, 11 prognostic factors/markers of, biochemical, chromogranin A as, 45, somatostatin receptor scintigraphy and, 195 with targeted radiopeptide therapy, 195 quality of life with, chromogranin A correlation to, 121 concerns regarding, introduction to, patient-reported outcomes of, 99 two disease-specific questionnaires for, description of, ,

20 252 Neuroendocrine (continued ) development of, 100 study methods for completing, results, scales and scores, statistical analysis, 102 summary discussion on, radioimmunoassays for, evolutionary advances in, 135, 143, 147 glossary for, 147 history of, methodological differences in, 136 sensitivity and specificity of, , 145 serotonin and tryptophan techniques, , 146 standardization in laboratories, surgery for, 223, 226 debulking, , 223, gastric, per Inter Science Institute Gastrointestinal Council, 227 per New Orleans Louisiana Neuroendocrine Tumor group, 228 preoperative and perioperative recommendations for, 226, 229 primary asymptomatic, 167 survival rates with, based on symptomatic treatment, 209 in children and young adults, 67 median duration based on tumor extent, 22 TRT response and, symptomatic treatment of, 209, 216 per Inter Science Institute Gastrointestinal Council, 217 per New Orleans Louisiana Neuroendocrine Tumor group, 218 targeted radiopeptide therapy for, clinical studies of, 190 using 111 In-octreotide, 190 using 177 LU-DOTATATE, using 177 LU-DOTATOC, using 90 Y-DOTA-lanreotide, using 90 Y-DOTATATE, using 90 Y-DOTATOC, current recommendations for, 219, 222, dosimetric considerations in, , future developments in, gastroenteropancreatic, See also Peptide receptor radionuclide therapy (PRRT). in children and young adults, 72, patient selection for, radionuclides used in, nuclear properties of, 188 side effects of, summary overview of, , 197 toxicity of, hematological, 193 liver, 194 reduction strategies for, 197

21 253 renal, Neurofibromastosis, in children and young adults, in neuroendocrine tumor syndromes, DVT, steatorrhea, cholelithiasis with, 20 Neurokinin A (NKA), in neuroendocrine tumor diagnosis, for children and young adults, 71 in neuroendocrine tumor prognosis, 46 Neuron-specific enolase (NSE), in neuroendocrine tumor diagnosis, 21, 29, chromogranin A vs., current recommendations for, 206 New Orleans Louisiana Neuroendocrine Tumor group recommendations, on neuroendocrine tumors, biochemical markers, chemotherapy, 221 imaging studies, 215 surgery, 228 symptomatic treatment, 218 Nisidioblastosis, 73 Nonalcoholic fatty liver disease (NAFLD), bariatric surgery effect on, obesity and, 82 Nonalcoholic steatohepatitis (NASH), obesity and, 82 Nonmalignant pathologies, chromogranin A elevation with, Norfolk QOL-NET questionnaire, description of, , 107 development of, 100 study methods for completing, results, scales and scores, statistical analysis, 102 summary discussion on, Nuclear imaging, of neuroendocrine tumors, See also specific technique, e.g., Magnetic resonance imaging (MRI). Nutritional therapy, for obesity, 82 83, 86 O Obesity, epidemic perspectives of, metabolic health effects of, pathophysiology of, 83 treatment goals for, 83 84, 86 Octreoscan, for neuroendocrine tumor diagnosis, 47 48, 51 52, 155 asymptomatic, 167 carcinoid, current recommendations for, 208, development of, in children and young adults, 69 pancreatic, 49 Octreotide, for carcinoid crisis prevention, during surgery, 169, for symptomatic neuroendocrine tumors, 209, long-acting, 209, Octreotide radiopharmaceuticals, for neuroendocrine tumors, , See also Targeted radiopeptide therapy (TRT). current recommendations for, 219, 222, gastroenteropancreatic. See Peptide receptor radionuclide therapy (PRRT).

22 254 Octreotide (continued ) in children and young adults, Orlistat, for obesity, 84 Ovarian carcinoma, in children and young adults, small cell, 66, 69, 71, 74 P Pancreastatin, in neuroendocrine tumor diagnosis, 21 22, 208 for children and young adults, in neuroendocrine tumor prognosis, Pancreatic carcinoma, chromogranin A elevation with, 119 Pancreatic islet cell tumors. See also specific tumor, e.g., Gastrinoma. current treatment recommendations for, in children and young adults, 66 67, 71, 73 surgery for, Pancreatic islet cells, bariatric surgery effect on, 85, Pancreatic neuroendocrine tumors (PNETs). See also specific tumor. biochemical assessment and monitoring of, chemotherapy for, 216, 219 chromogranin A as marker of, 121 clinical syndrome presentations of, 20, gastrinoma as, 29, 32 gastroenteric. See Gastroenteropancreatic neuroendocrine tumors (GEP-NETs). glucagonoma as, 29, 33 imaging of, conventional, 49 endoscopic ultrasonography, functional localization with, 50 general, intraoperative localization with, 50 scintigraphy, 49 in children and young adults, 66 67, 71, 73 incidence of, 29 insulinoma as, 29, 31 miscellaneous, nonfunctional, 37 prevalence of, 19, 21 rare functional, 29, 37 surgery for, 165 somatostatinoma, 29, surgery for, poorly differentiated, 166 VIPomas as, 29, 33 Pancreatic polypeptide (PP), in neuroendocrine tumor diagnosis, 25 26, 37, chromogranin A vs., 113, 123 Paragangliomas, biochemical markers of, 30, 41 imaging of, in children and young adults, 74

23 Patient-reported outcomes (PROs), of QOL, in clinical trials on neuroendocrine tumors, 99 PCOS. See Polycystic ovary syndrome (PCOS). Pellagra, in neuroendocrine tumor syndromes, 26 Peptic ulcer, in neuroendocrine tumor syndromes, dyspepsia with, 20, 26 Peptide fragments, of chromogranin A, as tumor marker, current recommendations for, immunohistochemical investigations of, 117 physiology of, Peptide radioreceptor nuclide therapy (PRRNT), for neuroendocrine tumors. See also Targeted radiopeptide therapy (TRT). current recommendations for, 219, 222, gastroenteropancreatic. See Peptide receptor radionuclide therapy (PRRT). in children and young adults, 72, Peptide receptor radionuclide therapy (PRRT), for gastroenteropancreatic neuroendocrine tumors, affinity profiles for sst 1 sst 5 receptors of series of somatostatin analogs, 177 options to improve, quality of life with, 179 retreatment with, summary overview of, , 182 survival data comparisons, 180 timing of, 180 tumor response to different radiolabeled somatostatin analogs, 175 with [ 111 Indium-diethylenetriamine pentaacetic acid 0 ] octreotide, 174 with [ 177 Lutetium-dota 0, tyr 3 ] octreotate, comparison to other PRRT, 177, survival with, 180 with [ 90 Yttrium-dota 0, tyrosine 3 ] octreotide, 174, 176 for neuroendocrine tumors, See also Targeted radiopeptide therapy (TRT). Peptides, neuroendocrine tumors and, chromogranin A cleavage to, 114 chromogranin A secretion and, 112, 114, 120 current recommendations for, , quantitative and qualitative analysis of, evolutionary advances in, 135, 143 glossary for, 147 history of, methodological differences in, 136 serotonin and tryptophan techniques, , 146 standardization in laboratories, Peptide-secreting tumors, diagnosis of, pancreatic polypeptide in, 25 26, 37, chromogranin A vs., 113, 123 plasma peptides in, for children and young adults, 69 71, 75 PTH-related peptide in, 30, surgery for, 165 Perioperative recommendations, for neuroendocrine tumor surgery, 226, 229 octreotide therapy as, 169, Peripheral ganglion tumors, in children and young adults, Pharmacologic therapy, for weight loss, 84 in combination chemotherapy, for neuroendocrine tumors, 216, radionuclide therapies with, 219,

24 256 Pharmacologic (continued) radiolabeled. See Octreotide radiopharmaceuticals. Pheochromocytomas, biochemical markers of, 30, 41 chromogranin A and, epidemiology of, 40 imaging of, in children and young adults, 65 67, 74 molecular genetics of, 40 pathology of, 40 signs and symptoms of, 40 Physical examination, for neuroendocrine tumor surgery, 226 Physical functioning, with neuroendocrine tumor syndromes, chromogranin A correlation to, 121 QOL measurement of, Pituitary adenomas, chromogranin A and, 121 in children and young adults, Plasma peptides, in neuroendocrine tumor diagnosis. See also specific peptide, e.g., Pancreatic polypeptide (PP). for children and young adults, 69 71, 75 Polycystic ovary syndrome (PCOS), bariatric surgery effect on, 87 obesity and, 82, 84 Positron emission tomography (PET), for neuroendocrine tumor diagnosis, 47 48, 50, 52, 156 carcinoid, current recommendations for, 208, in children and young adults, 69 pancreatic localization with, 50 tracers for somatostatin receptor imaging with, 173, See also specific tracer, e.g., Ga-68 DOTATOC positron emission tomography. Preoperative evaluations, for neuroendocrine tumor surgery, 226, 229 Primary neuroendocrine tumors, asymptomatic, surgery indications for, 167 gastroenteropancreatic, 5 7, 11 of small bowel, 167 Prognostic factors/markers, of metabolic health effect, after bariatric surgery, 90 of neuroendocrine tumors, biochemical, chromogranin A as, 45, somatostatin receptor scintigraphy and, 195 with targeted radiopeptide therapy, 195 Prostatic carcinoma, chromogranin A elevation with, 119 Proteins, chromogranin A secretion and, 112, 114 Provocative tests, for carcinoid syndrome, 24 for pancreatic neuroendocrine tumors, Psychosocial functioning, with neuroendocrine tumor syndromes, QOL measurement of, PTH-related peptide (PTH-rp), in neuroendocrine tumor syndromes, 30, Q Quality of life (QOL), with neuroendocrine tumors, chromogranin A correlation to, 121

25 257 concerns regarding, disease-specific questionnaires for measurement of, description of, , 107 development of, 100 study methods for completing, results, scales and scores, statistical analysis, 102 summary discussion on, introduction to, patient-reported outcomes of, 99 with peptide receptor radionuclide therapy, for GEP-NETs, 179 R Radioenzymatic assays, in neuroendocrine tumor diagnosis, 143, 145 Radiofrequency ablation (RFA), for neuroendocrine tumors, rare functioning pancreatic, 165 Radiographic studies. See Imaging studies. Radioimmunoassays, of neuroendocrine tumors, evolutionary advances in, 135, 143, 147 glossary for, 147 history of, methodological differences in, 136 sensitivity and specificity of, , 145 serotonin and tryptophan techniques, , 146 standardization in laboratories, Radiolabeled studies, of neuroendocrine tumors, imaging. See Scintigraphy. plasma, serum, and CSF. See Radioimmunoassays. Radionuclide therapy, for neuroendocrine tumors, chemotherapy combined with, 219, per Inter Science Institute Gastrointestinal Council, 224 per New Orleans Louisiana Neuroendocrine Tumor group, 225 somatostatin receptor targeted, for gastroenteropancreatic neuroendocrine tumors, See also Peptide receptor radionuclide therapy (PRRT). for neuroendocrine tumors, See also Targeted radiopeptide therapy (TRT). Radioreceptor nuclide therapy. See Peptide radioreceptor nuclide therapy (PRRNT). Radiotherapy, targeted, with radiolabeled somatostatin analogs, See also Targeted radiopeptide therapy (TRT). Rash, in neuroendocrine tumor syndromes. See Dermatitis. Rectal neuroendocrine tumors, chromogranin A elevation with, 119 in children and young adults, 69, surgery for, 168 Relapse, of GEP-NETs, chromogranin A identification of, 124 Renal toxicity, of targeted radiopeptide therapy, for neuroendocrine tumors, Resection, of gastroenteropancreatic neuroendocrine tumors, 163, 223, See also Surgery. Respiratory symptoms, in neuroendocrine tumor syndromes, QOL measurement with,

26 258 Retreatment, with peptide receptor radionuclide therapy, for GEP-NETs, Roux-en-Y gastric bypass (RYGBP), laparoscopic, S Schwannomas, in children and young adults, Scintigraphy, for neuroendocrine tumor diagnosis, 47 48, 50 51, 155. See also specific technique, e.g., Somatostatin receptor scintigraphy (SRS). in children and young adults, 69 pancreatic, 49 Secretory diarrhea, 25 SEER. See Surveillance, Epidemiology and End Results (SEER) program. Sensitivity and specificity, of elevated chromogranin A, in neuroendocrine tumor diagnosis, comparison to alternative tumor markers, concentration fluctuations, 121 in neuroendocrine tumors prognosis, in neuroendocrine tumors treatment, as relapse identifier, 124 as response measurement, 124 Serotonin, in neuroendocrine tumor diagnosis, current recommendations for, 207 for children and young adults, QOL measurement with, , 106 radioimmunoassays of, , 146 metabolic pathways for, 146 Sibutramine, for obesity, 84 Signs and symptoms, in neuroendocrine tumor syndromes, absence of, 20 surgery indications with, 167 QOL measurement with, Single ion monitoring (SIM), in radioimmunoassays, Single-photon emission CT (SPECT), of neuroendocrine tumors, 48 49, 154 carcinoid, in children and young adults, 69 Skin carcinoma, Merkel cell, chromogranin A and, Small bowel, primary neuroendocrine tumors of, 167 Small cell carcinoma, of lungs, chromogranin A and, of ovaries and cervix, in children and young adults, 66, 69, 71, 74 Somatostatin analogs, for symptomatic neuroendocrine tumors, 209, current recommendations for, 219, 222, long-acting, 209, in peptide receptor radionuclide therapy, current recommendations for, 219, 222, for GEP-NETs. See also Peptide receptor radionuclide therapy (PRRT). affinity profiles for sst 1 sst 5 receptors of series of, 177 tumor response to, 175 for neuroendocrine tumors, See also Targeted radiopeptide therapy (TRT). future developments of, Somatostatin receptor radionuclide therapy, for gastroenteropancreatic neuroendocrine tumors, See also Peptide receptor radionuclide therapy (PRRT).