Note: Page numbers of article titles are in boldface type. A Abdominal pain, in neuroendocrine tumor syndromes, 20, 25 26 Acromegaly, as neuroendocrine tumor presentation, 38 ACTH suppression studies, 38 39 ACTH-secreting tumors, 26, 29 30, 33, 38 in children and young adults, 69 70 surgery for, 165 Activities of daily living, neuroendocrine tumor syndromes and, QOL measurement of, 100 107 Adenocarcinomas, chromogranin A elevation with, 119 120 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, 210 213 quantitative and qualitative analysis of, 135 151 evolutionary advances in, 135, 143 glossary for, 147 history of, 143 147 methodological differences in, 136 serotonin and tryptophan techniques, 136 143, 146 standardization in laboratories, 135 136 Angiography, of neuroendocrine tumors, 49, 51 current recommendations for, 208, 214 215 Antibodies, insulin-binding, 143 to chromogranin A, immunoassays of, 115 116 immunohistochemical staining and, 117 Appendiceal neuroendocrine tumors, in children and young adults, 69, 72 73 surgery for, 168 Appetite, bariatric surgery and, 85 obesity and, 82 83 Asymptomatic primary neuroendocrine tumors, surgery indications for, 167 B Bariatric surgery, metabolic and cardiovascular consequences of, 81 96 CMRS benefits of weight loss and, 85 86 CMRS-specific, 86 87 compared to non-surgical therapies, 84 85 Endocrinol Metab Clin N Am 40 (2011) 233 263 doi:10.1016/s0889-8529(11)00021-1 0889-8529/11/$ see front matter ª 2011 Elsevier Inc. All rights reserved. endo.theclinics.com
234 Bariatric (continued) herbal therapy and, 84 lifestyle interventions and, 84 85 metabolic-specific, 87 90 obesity and metabolic health in, 81 82 obesity epidemic perspectives in, 82 83 obesity pathophysiology in, 83 obesity treatment goals and, 83 84 pharmacologic therapy and, 84 summary overview of, 90 techniques for, 84 85 Barium studies. See Upper GI series. Bioassays, in neuroendocrine tumor diagnosis, 136 137, 143 144 Biochemical markers, of cardiometabolic risk syndrome, 82 of neuroendocrine tumors, as diagnostic, 38 39, 43 45, 111 112, 117 as prognostic, 45 46 blood and urine, 28 29 chromogranin A relevance as, 111 134. See also Chromogranin A (CgA). classification according to use, 43 46 current recommendations for, 207 208 of Inter Science Institute Gastrointestinal Council, 210 211 of New Orleans Louisiana Neuroendocrine Tumor group, 212 213 for bone metastasis diagnosis, 46 for cardiac involvement diagnosis, 46 hormonal, 19 23 in children and young adults, 69 71 preoperative evaluation of, 226 QOL measurement and, 101 107 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, 153 154 in children and young adults, 67 69 Breast carcinoma, chromogranin A elevation with, 119 120 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.
Cancers, endocrine/neuroendocrine. See also specific anatomy or type, e.g., Lung carcinoma. in children and young adults, classification of, 65 67, 76 77 diagnosis and therapy for, 69 75 epidemiology of, 67 69 future directions for, 75 76 non-neuroendocrine, chromogranin A elevation with, 118 120 Capsule endoscopy, for neuroendocrine tumor diagnosis, 52, 208, 214 215 Carcinoembryonic antigen (CEA), in neuroendocrine tumor diagnosis, chromogranin A vs., 121 123 Carcinoid crisis, prevention during surgery, for GEP-NETs, 169, 227 228 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, 38 39 radioimmunoassays for, 137 gastric types of, 164. See also Gastric neuroendocrine tumors. in children and young adults, 70 74 surgery for, 164, 166, 168 imaging of, 20, 26, 36, 46 47, 156 159 in children and young adults, 65 66, 68 bronchial, 69 70, 72 gastric, 70 74 natural history of, 21 22 prevalence of, 19, 21 QOL measurement with, 100 103, 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, 86 87 dyslipidemia and, 86 hypertension and, 86 NAFLD and, 86 87 PCOS and, 87 T2DM complications and, 87 benefits of weight loss on, 85 86 definition of, 81 82 obesity and, 81 82 pathophysiology of, 83 Cardiovascular consequences, of bariatric surgery, 81 96 cardiometabolic risk syndrome as, 86 87 benefits of weight loss on, 85 86 dyslipidemia and, 86 hypertension and, 86 NAFLD and, 86 87 PCOS and, 87 T2DM complications and, 87 compared to non-surgical therapies, 84 85 herbal therapy and, 84 235
236 Cardiovascular (continued) lifestyle interventions and, 84 85 metabolic-specific, 87 90 obesity and metabolic health in, 81 82 obesity epidemic perspectives in, 82 83 obesity pathophysiology in, 83 obesity treatment goals and, 83 84 pharmacologic therapy and, 84 summary overview of, 90 of neuroendocrine tumor syndromes, QOL measurement with, 100 107 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, 222 223 per Inter Science Institute Gastrointestinal Council, 224 per New Orleans Louisiana Neuroendocrine Tumor group, 225 Chemotherapy, for neuroendocrine tumors, chemoembolization combined with, 222 223 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, 224 225 rare functioning pancreatic, 165 Children and young adults, neuroendocrine tumors in, 65 80 appendiceal, jejunal, and ileal, 69, 72 73 breast carcinoma as, 65 66, 70, 72 bronchial carcinoid, 69 70, 72 classification of, 65 67 colon and rectal, 69, 73 74 diagnosis and therapy for, 69 75 distribution of, 68 69 epidemiology of, 67 69 future directions for, 75 76 gastric carcinoid, 65 66, 68, 70 74 hindgut, 66, 69, 71, 73 74 incidence of, 67 68 late diagnosis of, 67 68 lung carcinoma as, 65 66, 68 70, 72 midgut, 66, 69 70, 72 73 molecular genetics of, 70 71, 75 neural crest, 65 68, 74 75 neuroblastoma as, 65 67, 74 75 ovary and cervix, 65 66, 69, 71, 74 pancreatic, 66 67, 71, 73 paraganglioma as, 74 pathology report data sets for, 69 71 pheochromocytoma as, 65 67, 74 prevalence of, 68 summary overview of, 65, 76 77 survival of, 67
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, 137 145 Chromogranin A (CgA), as gastroenteropancreatic neuroendocrine tumor marker, 111 134 as diagnostic, 111 112, 121 123 rationale for, 120 121 as prognostic, 123 124 cleavage to other peptides and, 114 clinical utility of, 120 125 as tumor burden measurement, 123 124 as tumor marker, 120 121 comparison to alternative tumor markers, 121 123 concentration fluctuations and, 121 relapse identification as, 124 response to treatment correlations as, 124 sensitivity and specificity of, 121 125 granin family and, 112, 114, 207 208 measurement of, immunoassays for, 115 116 immunohistochemical staining for, 117 platforms for blood, 116 platforms for tissue, 117 neuroendocrine cell biology and, 112 neuroendocrine cell types and secretion of, 112 114 normal cellular biology vs., 112 116 pathologic elevation and, 117 120 iatrogenic causes of, 119 malignant but non-neuroendocrine causes of, 118 120 nonmalignant causes of, 117 119 peptide fragments and, 114 116 physiologic role of, 114 116 summary overview of, 125 in neuroendocrine tumor diagnosis, 21 23, 26 29 for children and young adults, 70 71 pancreatic, 37 39 QOL measurement with, 101 103, 106 tumor type specificity, 43 45, 206 in neuroendocrine tumor prognosis, 45, 123 124 Chromogranin A (CgA)-secretogranin (Sg) proteins, as neuroendocrine tumor marker, 207 208 Chromogranin B (CgB), in neuroendocrine tumor diagnosis, 43 45 Chronic conditions, aggravated by obesity, 82 83 chromogranin A elevation with, 117 119 [ 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, 195 237
238 Classification (continued ) ICD-O-3 codes, 4 5 TRT patient selection based on, 194 196 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, 73 74 surgery for, 168 Colorimetry, in neuroendocrine tumor diagnosis, 136, 144 Combination chemotherapy, for neuroendocrine tumors, 229 chemoembolization in, 222 223 pharmacologic agents and radionuclide therapies in, 219, 224 225 pharmacologic agents in, 216, 219 221 Computed tomography (CT), of neuroendocrine tumors, 36, 47, 50 51, 153 154 cross-sectional imaging techniques for, 47 48 current recommendations for, 208, 214 215 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, 223 225, 229 rare functioning pancreatic, 165 Cytotoxic therapy. See Chemotherapy. D Debulking, surgical, for neuroendocrine tumors, 165 166, 223, 226 228 Dementia, in neuroendocrine tumor syndromes, 20 Depression, with neuroendocrine tumor syndromes, QOL measurement of, 101 107 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, 51 52 impediments to, 21 22 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, 25 26 QOL measurement with, 100 steatorrhea with, 20, 34 secretory, 25
239 somatostatinoma and, 34 Diet modifications, for obesity, 82 83, 86 post-gastric bypass, 89 Diffuse neuroendocrine system (DNES), 112 chromogranin A secretion and, 112 114 GEP-NETs originating from, 112 113 Disease-specific questionnaires, for QOL measurement, with neuroendocrine tumors, description of, 100 101, 107 development of, 100 study methods for completing, 101 104 results, 102 105 scales and scores, 101 102 statistical analysis, 102 summary discussion on, 104 107 F-18 Dopamine positron emission tomography, of neuroendocrine tumors, 156, 158 159 Dosimetry, in targeted radiopeptide therapy, for neuroendocrine tumors, 189 190, 196 197 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, 158 159 Doxorubicin, for neuroendocrine tumors, 216, 219 221 radionuclide therapies combined with, 224 225 Dumping syndrome, in neuroendocrine tumor syndromes, 26 late-phase, after bariatric surgery, 88 Duodenal neuroendocrine tumors, surgery for, 165 166 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, 25 26 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, 222 223 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
240 Endocrine (continued ) somatostatinoma association with, 35 Endocrine tumors. See also Neuroendocrine tumors (NETs). in children and young adults, 65 67 Endoscopic ultrasound (EUS), for neuroendocrine tumor diagnosis, 46 47, 51 current recommendations for, 208, 214 215 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, 214 215 Enteroscopy, for neuroendocrine tumor diagnosis, 208, 214 215 Enzyme-linked immunosorbent assay (ELISA), for chromogranin A measurement, 116 EORTC QLQ-C30 GI.NET-21 questionnaire, description of, 100, 107 development of, 99 100 study methods for completing, 101 104 results, 102 105 scales and scores, 101 102 statistical analysis, 102 summary discussion on, 104 107 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, 65 67 Excision, of gastroenteropancreatic neuroendocrine tumors, 163, 223, 226 228. See also Surgery. Extent of disease. See Tumor burden. External beam radiation, for neuroendocrine tumors, 224 225 radio-sensitizers for, 197 F Familial endocrine tumors, in children and young adults, 65 67 Fatigue, in neuroendocrine tumor syndromes, QOL measurement with, 100 Fatty acid metabolism, obesity and, 82 83 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, 214 215 FDOPA-positron emission tomography (FDOPA-PET), of neuroendocrine tumors, 50, 156, 158 159 5-HIAA. See 5-Hydroxyindoleacetic acid (5-HIAA). Fluorometry, in neuroendocrine tumor diagnosis, 136 139, 141, 144 5-Fluorouracil, for neuroendocrine tumors, 216, 219 221 radionuclide therapies combined with, 224 225 Flushing, in neuroendocrine tumor syndromes, 20, 22 25 differential diagnosis of, 23
241 QOL measurement with, 100 107 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, 158 159 Gallbladder disease. See Cholelithiasis. Ganglion tumors, peripheral, in children and young adults, 65 66 Gas chromatography (GC), in neuroendocrine tumor diagnosis, 139, 143 Gas chromatography/mass spectrometry (GC/MS), in neuroendocrine tumor diagnosis, 139 140, 142, 145 Gastric banding, laparoscopic adjustable, 84 Gastric bypass. See Bariatric surgery. Gastric neuroendocrine tumors. See also specific anatomy. biomarkers of, 28 29 clinical syndrome presentations of, 20 26 diagnosis of, 27 imaging of, 36, 46 47, 157 159 immunohistochemistry of, 28 in children and young adults, 65 66, 68, 72 74 molecular genetics of, 28, 75 pathology of, 27 28 prevalence of, 19, 21 special pathologic consideration of, 28 surgery for, 163 170. 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, 111 112 cellular origins of, 112 113, 164 chromogranin A relevance to, 111 134 as diagnostic, 111 112, 121 123 as prognostic, 123 124 cleavage to other peptides and, 114 clinical utility of, 120 125 as tumor burden measurement, 123 124 as tumor marker, 120 121 comparison to alternative tumor markers, 121 123 concentration fluctuations and, 121 relapse identification as, 124 response to treatment correlations as, 124 sensitivity and specificity of, 121 125 granin family and, 112, 114 measurement of, immunoassays for, 115 116 platforms for blood, 116 platforms for tissue, 117
242 Gastroenteropancreatic (continued ) neuroendocrine cell biology and, 112 neuroendocrine cell types and secretion of, 112 114 normal cellular biology vs., 112 116 pathologic elevation and, 117 120 iatrogenic causes of, 119 malignant but non-neuroendocrine causes of, 118 120 nonmalignant causes of, 117 119 peptide fragments and, 114 116 physiologic role of, 114 116 summary overview of, 125 classification of, 2, 195 diagnostic techniques for, 2 epidemiology of, 1 18 SEER discussion in, 13 16 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, 15 16 mean age-adjusted, 5, 11 persistent increase in, 13 14 peptide receptor radionuclide therapy for, 173 185 affinity profiles for sst 1 sst 5 receptors of series of somatostatin analogs, 177 options to improve, 181 182 quality of life with, 179 retreatment with, 180 181 summary overview of, 173 174, 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, 176 178 comparison to other PRRT, 177, 179 180 with [ 90 Yttrium-dota 0, tyrosine 3 ] octreotide, 174, 176 prevalence of, vs. neuroendocrine tumors, 19, 21 somatostatin receptor radionuclide therapy for, 173 185. See also Peptide receptor radionuclide therapy (PRRT). surgery for, 163 171 appendiceal tumors, 168 asymptomatic primary tumors, 167 carcinoid crisis with, 169, 227 228 carcinoid tumors, 164, 166, 168 colonic tumors, 168
243 foregut poorly differentiated tumors, 166 hepatic metastases indications in, 168 jejunoileum tumors, 166 liver transplantation as, 169, 226 228 lymphatic mapping and, 167 MEN-1 syndrome tumors, 165 166 midgut tumors, 166 pancreatic tumors, 164 165 poorly differentiated, 166 rare functioning, 165 rectal tumors, 168 summary overview of, 163 164, 170 survival rates with, 2, 11 12 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, 15 16 5-year rates, 11 12, 14 15 Gastrointestinal symptoms, in neuroendocrine tumor syndromes. See also specific symptom, e.g., Diarrhea. QOL measurement with, 100 107 Gastroplasty, vertical banded, 84 85 Genetic testing, for neuroendocrine tumors, 38 39 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, 15 16 TRT patient selection based on, 194 196 Granin family, chromogranin A secretion and, 112, 114, 207 208 Growth hormone releasing factor secreting tumors (GRFomas), 29, 37 in children and young adults, 69 70 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.
244 Hepatocellular cancer (HCC), chromogranin A elevation with, 119 Herbal therapy, for weight loss, 84 High-performance liquid chromatography (HPLC), in neuroendocrine tumor diagnosis, 137 139, 141 142 Hindgut tumors, neuroendocrine, in children and young adults, 66, 69, 71, 73 74 surgery for, 168 History taking, for neuroendocrine tumor surgery, 226 Hormonal gradients, assessment for neuroendocrine tumors, 147 current recommendations for, 206, 210 213 in children and young adults, 69 71 pancreatic, 50 Hormones, neuroendocrine tumor syndromes associated with, 19 23. See also specific hormone, e.g., Chromogranin A (CgA). in children and young adults, 69 71 radioimmunoassays of, 147 HPLC-electron chemical (EC) detector, in neuroendocrine tumor diagnosis, 138, 142, 144 HPLC-fluoresence, in neuroendocrine tumor diagnosis, 138 139, 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, 38 39 chromogranin A vs., 121 123 current recommendations for, 207 208 QOL measurement with, 101 103, 106 radioimmunoassays of, 136 138, 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, 87 90 clinical syndrome of, 87 88 evaluation of, 89 pathophysiology of, 88 89 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, 72 73 surgery for, 166 lymphatic mapping during, 167 Ileocecal valve, intraoperative sparing of, with neuroendocrine tumor resection, 167 Imaging studies, of neuroendocrine tumors, 153 162. See also specific technique. asymptomatic primary, 167
245 carcinoid, 20, 26, 36, 46 47, 156 159 computed tomography as, 153 154, 159 cross-sectional techniques for, 47 48 current recommendations for, 208 209 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, 158 159 FDG-positron emission tomography as, 156, 158 159 Ga-68 DOTATOC positron emission tomography as, 156, 158 159 in children and young adults, 69 In-111 pentetreotide scintigraphy as, 155, 157 158. See also Octreoscan. magnetic resonance imaging as, 153 154, 159 MIBG scintigraphy as, 47, 50, 52 midgut, 47, 52 nuclear techniques for, 47 48 pancreatic, 48 50 paragangliomas, 50 51 pheochromocytoma, 50 51 positron emission tomography as, 156, 158 159 preoperative recommendations for, 226, 229 somatostatinoma, 36 37 SPECT/CT as, 154, 157 158 summary overview of, 51 52, 153, 159 tumor-specific techniques, 46 52 Immunoassays, for chromogranin A measurement, 115 116 comparison to alternative tumor markers, 121 123 radiolabeled, for neuroendocrine tumors, 135 151. See also Radioimmunoassays. Immunohistochemistry, in neuroendocrine tumor diagnosis, 19, 28, 38 39 chromogranin A and, 117 current recommendations for, 206 for children and young adults, 69 71 in somatostatinoma diagnosis, 34, 36 37 In-111 pentetreotide scintigraphy, for neuroendocrine tumor diagnosis, 69, 155, 157 158, 167. See also Octreoscan. Incretins, bariatric surgery effect on, 88 111 Indium-DTPA (diethylenetriamine pentaacetic acid 0 ) octreotide scintigraphy, for neuroendocrine tumors, 47 49, 174 111 In-DOTABASS, in targeted radiopeptide therapy, 196 111 In-octreotide, in targeted radiopeptide therapy, 188 clinical studies of, 190 191 diagnostic use vs., 187 188 future developments vs., 196 197 prognostic use vs., 195 111 In-octreotide scintigraphy, 187 188. See also Somatostatin receptor scintigraphy (SRS). Insulin production, bariatric surgery effect on, 85, 88 89 Insulin resistance, obesity and, 82 83 Insulin-binding antibodies, 143
246 Insulinoma, 29, 31, 38, 113 post-gastric bypass, 89 somatostatin analogs for, 216 surgery for, 164 165 Inter Science Institute Gastrointestinal Council recommendations, on neuroendocrine tumors, biochemical markers, 210 211 chemotherapy, 220 imaging studies, 214 surgery, 227 symptomatic treatment, 217 Interferon-a, for neuroendocrine tumors, 219 Internal radiotherapy, for neuroendocrine tumors, 188 189. See also Targeted radiopeptide therapy (TRT). clinical studies of, 190 192 current recommendations for, 219, 222, 224 225 dosing considerations of, 189 190 side effects and toxicity of, 192 194 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., 21 22 Islet cell tumors, pancreatic. See also specific tumor, e.g., Gastrinoma. classification of, 206 current treatment recommendations for, 209 229 in children and young adults, 66 67, 71, 73 surgery for, 164 165 Islet cells, pancreatic, bariatric surgery effect on, 85, 88 89 Isotopes, in targeted radiopeptide therapy, 188 189 clinical studies of, 190 192 current recommendations for, 219, 222, 224 225 future developments of, 196 197 J Jejunal neuroendocrine tumors, in children and young adults, 69, 72 73 surgery for, 166 K Ki-67, in neuroendocrine tumor diagnosis, 28, 206 L Lanreotide, for symptomatic neuroendocrine tumors, 209, 217 218 in targeted radiopeptide therapy, 188 clinical studies of, 190 192 current recommendations for, 224 225 Laparoscopic adjustable gastric banding (LAGB), 84 Laxative abuse, 25 Leptin, obesity and, 83
247 Lifestyle interventions, for weight loss, 84 85 Liquid chromatography (LC), 143 145 high-performance, in neuroendocrine tumor diagnosis, 137 139, 141 142 ultra high-performance, in neuroendocrine tumor diagnosis, 138, 141 Liquid chromatography/mass spectrometry (LC/MS), in neuroendocrine tumor diagnosis, 140 141, 145 Liver metastases, of neuroendocrine tumors, 20 22 chromogranin A prediction of, 123 imaging of, 47 48, 51, 153 154, 157 159 in children and young adults, 67 69 liver transplantation for, 169, 226 228 surgery for, 166, 168 targeted radiopeptide therapy and, 190, 194 195 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, 226 228 177 LU-DOTATATE, for GEP-NETs, 176 178 comparison to other PRRT, 177, 179 180 survival with, 180 in targeted radiopeptide therapy, 188 clinical studies of, 191 192 current recommendations for, 224 225 future developments of, 197 prognosis with, 196 toxicity of, 194 177 LU-DOTATOC, in targeted radiopeptide therapy, 188 clinical studies of, 191 192 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, 120 121 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, 153 154 cross-sectional imaging techniques for, 47 48 current recommendations for, 208 209, 214 215 in children and young adults, 69 pancreatic, 49 Malignancy(ies), chromogranin A elevation with, as neuroendocrine tumor marker, 120 121 with non-neuroendocrine disorders, 118 120 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, 165 166, 229. See also Surgery. with somatostatinoma, 35 Mass spectrometry (MS), in neuroendocrine tumor diagnosis, 139 142 gas chromatography with, 139 140, 142, 145
248 Mass (continued) liquid chromatography with, 140 141, 145 Medullary carcinoma of the breast, in children and young adults, 65 66 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, 65 66 Melatonin, in neuroendocrine tumor diagnosis, 141 143 Mendelian inheritance, of endocrine tumors, 65 Merkel cell carcinoma of the skin, chromogranin A and, 120 121 Metabolic health effect, of bariatric surgery, 81 96 CMRS benefits of weight loss and, 85 86 CMRS-specific vs., 86 87 compared to non-surgical therapies, 84 85 herbal therapy and, 84 hypoglycemia as, 87 90 clinical syndrome of, 87 88 evaluation of, 89 pathophysiology of, 88 89 prognosis for, 90 treatment of, 89 lifestyle interventions and, 84 85 obesity and, 81 82 obesity epidemic perspectives in, 82 83 obesity pathophysiology in, 83 obesity treatment goals and, 83 84 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, 153 154, 157 159 current recommendations for, 208 209, 214 215 in children and young adults, 67 69 liver. See Liver metastases. lymphatic mapping of, 167 QOL measurement with, 101 103, 106 targeted radiopeptide therapy and, 190, 194 195 with rare functioning pancreatic tumors, 165 131 I MIBG (metaiodobenzylguanidine) radionucleotide therapy, for neuroendocrine tumors, current recommendations for, 222, 224 225 131 I MIBG (metaiodobenzylguanidine) scintigraphy, of neuroendocrine tumors, 47, 154 carcinoid, 157 158 123 I MIBG (metaiodobenzylguanidine) scintigraphy, of neuroendocrine tumors, 47, 50, 52, 154 155 Midgut tumors, neuroendocrine, blood and urine biomarkers of, 28 29 imaging of, 47, 52, 157 current recommendations for, 208, 214 215 for initial staging, 47
249 in children and young adults, 66, 69 70, 72 73 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, 73 75 somatostatinoma association with, 35 surgery for neuroendocrine tumors with, 165 166 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, 51 52 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, 140 141 NETs. See Neuroendocrine tumors (NETs). Neural crest tumors, in children and young adults, 65 68, 74 75 Neuroblastoma, in children and young adults, 65 67, 74 75 Neuroendocrine cells, chromogranin A immunohistochemical staining in, 117 chromogranin A secretion and, 112 114, 120 GEP-NETs originating from, 112 113, 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, 111 112, 117 as prognostic, 45 46 blood and urine, 28 29 classification according to use, 43 46 current recommendations for, 207 208 of Inter Science Institute Gastrointestinal Council, 210 211 of New Orleans Louisiana Neuroendocrine Tumor group, 212 213 for bone metastasis diagnosis, 46 for cardiac involvement diagnosis, 46 hormonal, 19 23 in children and young adults, 69 71 QOL measurement and, 101 107 C-cell hyperplasia as, 42 chemotherapy for, 216, 219, 229 chemoembolization combined with, 222 223 per Inter Science Institute Gastrointestinal Council, 220 per New Orleans Louisiana Neuroendocrine Tumor group, 221
250 Neuroendocrine (continued ) radionuclide therapies combined with, 219, 224 225 classification, grading, and staging system for, 27 28, 66 current recommendations for, 206 TRT patient selection based on, 194 196 current recommendations for, 205 231 biochemical markers, 207 208, 210 213 chemoembolization, 222 225 chemotherapy, 216, 219 221, 229 imaging, 208 209, 214 215 radionuclide therapies, 219, 222, 224 225 summary overview of, 206 surgery, 223, 226 229 symptomatic treatment, 209, 216 218 tumor classification, 206 Cushing syndrome and, 38 description of, 19 diagnostic algorithm for, 38 39, 51 52 diagnostic impediments of, 21 22 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, 153 162. See also specific technique. asymptomatic primary, 167 computed tomography as, 153 154, 159 cross-sectional techniques for, 47 48 current recommendations for, 208 209 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, 158 159 FDG-positron emission tomography as, 156, 158 159 Ga-68 DOTATOC positron emission tomography as, 156, 158 159 gastric carcinoid, 46 47 In-111 pentetreotide scintigraphy as, 155, 157 158. See also Octreoscan. magnetic resonance imaging as, 153 154, 159 MIBG scintigraphy as, 47, 50, 52 midgut, 47, 52 nuclear techniques for, 47 48 pancreatic, 48 50 paragangliomas, 50 51 pheochromocytoma, 50 51 positron emission tomography as, 156, 158 159 preoperative recommendations for, 226, 229 somatostatinoma, 36 37 SPECT/CT as, 154, 157 158 summary overview of, 51 52, 153, 159
tumor-specific techniques, 46 52 in children and young adults, 65 80. See also specific anatomy or type, e.g., Breast carcinoma. classification of, 65 67 diagnosis and therapy for, 69 75 distribution of, 68 69 epidemiology of, 67 69 future directions for, 75 76 incidence of, 67 68 late diagnosis of, 67 68 prevalence of, 68 summary overview of, 65, 76 77 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, 19 63 abdominal pain as, 20, 25 26 clinical presentations per tumor type/site and hormones, 19 20 dementia as, 20 dermatitis as, 20, 26 diarrhea as, abdominal pain and dyspepsia with, 20, 25 26 steatorrhea with, 20, 34 dyspepsia as, 20, 25 26 ulcer with, 20, 26 flushing as, 20, 22 25 differential diagnosis of, 23 gastric, 19 29. See also Gastric neuroendocrine tumors. hypoglycemia as, 20, 26, 34 imaging studies of, 46 52 pancreatic, 29 43. 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, 45 46 chromogranin A as, 45, 123 124 somatostatin receptor scintigraphy and, 195 with targeted radiopeptide therapy, 195 quality of life with, 97 109 chromogranin A correlation to, 121 concerns regarding, 97 98 introduction to, 98 99 patient-reported outcomes of, 99 two disease-specific questionnaires for, description of, 100 101, 107 251
252 Neuroendocrine (continued ) development of, 100 study methods for completing, 101 104 results, 102 105 scales and scores, 101 102 statistical analysis, 102 summary discussion on, 104 107 radioimmunoassays for, 135 151 evolutionary advances in, 135, 143, 147 glossary for, 147 history of, 143 147 methodological differences in, 136 sensitivity and specificity of, 141 143, 145 serotonin and tryptophan techniques, 136 143, 146 standardization in laboratories, 135 136 surgery for, 223, 226 debulking, 165 166, 223, 226 228 gastric, 163 170 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, 194 196 symptomatic treatment of, 209, 216 per Inter Science Institute Gastrointestinal Council, 217 per New Orleans Louisiana Neuroendocrine Tumor group, 218 targeted radiopeptide therapy for, 187 204 clinical studies of, 190 using 111 In-octreotide, 190 using 177 LU-DOTATATE, 191 192 using 177 LU-DOTATOC, 191 192 using 90 Y-DOTA-lanreotide, 190 192 using 90 Y-DOTATATE, 190 192 using 90 Y-DOTATOC, 190 192 current recommendations for, 219, 222, 224 225 dosimetric considerations in, 189 190, 196 197 future developments in, 196 197 gastroenteropancreatic, 173 185. See also Peptide receptor radionuclide therapy (PRRT). in children and young adults, 72, 74 76 patient selection for, 194 196 radionuclides used in, 188 189 nuclear properties of, 188 side effects of, 192 193 summary overview of, 187 188, 197 toxicity of, hematological, 193 liver, 194 reduction strategies for, 197
253 renal, 193 194 Neurofibromastosis, in children and young adults, 65 66 in neuroendocrine tumor syndromes, DVT, steatorrhea, cholelithiasis with, 20 Neurokinin A (NKA), in neuroendocrine tumor diagnosis, 21 22 for children and young adults, 71 in neuroendocrine tumor prognosis, 46 Neuron-specific enolase (NSE), in neuroendocrine tumor diagnosis, 21, 29, 44 45 chromogranin A vs., 121 123 current recommendations for, 206 New Orleans Louisiana Neuroendocrine Tumor group recommendations, on neuroendocrine tumors, biochemical markers, 212 213 chemotherapy, 221 imaging studies, 215 surgery, 228 symptomatic treatment, 218 Nisidioblastosis, 73 Nonalcoholic fatty liver disease (NAFLD), bariatric surgery effect on, 86 87 obesity and, 82 Nonalcoholic steatohepatitis (NASH), obesity and, 82 Nonmalignant pathologies, chromogranin A elevation with, 117 119 Norfolk QOL-NET questionnaire, description of, 100 101, 107 development of, 100 study methods for completing, 101 104 results, 102 105 scales and scores, 101 102 statistical analysis, 102 summary discussion on, 104 107 Nuclear imaging, of neuroendocrine tumors, 47 48. See also specific technique, e.g., Magnetic resonance imaging (MRI). Nutritional therapy, for obesity, 82 83, 86 O Obesity, epidemic perspectives of, 82 83 metabolic health effects of, 81 82 pathophysiology of, 83 treatment goals for, 83 84, 86 Octreoscan, for neuroendocrine tumor diagnosis, 47 48, 51 52, 155 asymptomatic, 167 carcinoid, 157 158 current recommendations for, 208, 214 215 development of, 187 188 in children and young adults, 69 pancreatic, 49 Octreotide, for carcinoid crisis prevention, during surgery, 169, 227 229 for symptomatic neuroendocrine tumors, 209, 217 218 long-acting, 209, 216 218 Octreotide radiopharmaceuticals, for neuroendocrine tumors, 187 191, 196 197. See also Targeted radiopeptide therapy (TRT). current recommendations for, 219, 222, 224 225 gastroenteropancreatic. See Peptide receptor radionuclide therapy (PRRT).
254 Octreotide (continued ) in children and young adults, 75 76 Orlistat, for obesity, 84 Ovarian carcinoma, in children and young adults, 65 66 small cell, 66, 69, 71, 74 P Pancreastatin, in neuroendocrine tumor diagnosis, 21 22, 208 for children and young adults, 70 71 in neuroendocrine tumor prognosis, 45 46 Pancreatic carcinoma, chromogranin A elevation with, 119 Pancreatic islet cell tumors. See also specific tumor, e.g., Gastrinoma. current treatment recommendations for, 209 229 in children and young adults, 66 67, 71, 73 surgery for, 164 165 Pancreatic islet cells, bariatric surgery effect on, 85, 88 89 Pancreatic neuroendocrine tumors (PNETs). See also specific tumor. biochemical assessment and monitoring of, 38 39 chemotherapy for, 216, 219 chromogranin A as marker of, 121 clinical syndrome presentations of, 20, 29 30 gastrinoma as, 29, 32 gastroenteric. See Gastroenteropancreatic neuroendocrine tumors (GEP-NETs). glucagonoma as, 29, 33 imaging of, 48 50 conventional, 49 endoscopic ultrasonography, 49 50 functional localization with, 50 general, 48 49 intraoperative localization with, 50 scintigraphy, 49 in children and young adults, 66 67, 71, 73 incidence of, 29 insulinoma as, 29, 31 miscellaneous, 37 38 nonfunctional, 37 prevalence of, 19, 21 rare functional, 29, 37 surgery for, 165 somatostatinoma, 29, 33 37 surgery for, 164 165 poorly differentiated, 166 VIPomas as, 29, 33 Pancreatic polypeptide (PP), in neuroendocrine tumor diagnosis, 25 26, 37, 44 45 chromogranin A vs., 113, 123 Paragangliomas, 41 42 biochemical markers of, 30, 41 imaging of, 50 51 in children and young adults, 74
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, 120 121 current recommendations for, 207 208 immunohistochemical investigations of, 117 physiology of, 114 116 Peptide radioreceptor nuclide therapy (PRRNT), for neuroendocrine tumors. See also Targeted radiopeptide therapy (TRT). current recommendations for, 219, 222, 224 225 gastroenteropancreatic. See Peptide receptor radionuclide therapy (PRRT). in children and young adults, 72, 74 76 Peptide receptor radionuclide therapy (PRRT), for gastroenteropancreatic neuroendocrine tumors, 173 185 affinity profiles for sst 1 sst 5 receptors of series of somatostatin analogs, 177 options to improve, 181 182 quality of life with, 179 retreatment with, 180 181 summary overview of, 173 174, 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, 176 178 comparison to other PRRT, 177, 179 180 survival with, 180 with [ 90 Yttrium-dota 0, tyrosine 3 ] octreotide, 174, 176 for neuroendocrine tumors, 187 204. 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, 207 208, 210 213 quantitative and qualitative analysis of, 135 151 evolutionary advances in, 135, 143 glossary for, 147 history of, 143 147 methodological differences in, 136 serotonin and tryptophan techniques, 136 143, 146 standardization in laboratories, 135 136 Peptide-secreting tumors, diagnosis of, pancreatic polypeptide in, 25 26, 37, 44 45 chromogranin A vs., 113, 123 plasma peptides in, for children and young adults, 69 71, 75 PTH-related peptide in, 30, 37 39 surgery for, 165 Perioperative recommendations, for neuroendocrine tumor surgery, 226, 229 octreotide therapy as, 169, 227 229 Peripheral ganglion tumors, in children and young adults, 65 66 Pharmacologic therapy, for weight loss, 84 in combination chemotherapy, for neuroendocrine tumors, 216, 219 221 radionuclide therapies with, 219, 224 225 255
256 Pharmacologic (continued) radiolabeled. See Octreotide radiopharmaceuticals. Pheochromocytomas, 40 42 biochemical markers of, 30, 41 chromogranin A and, 120 121 epidemiology of, 40 imaging of, 50 51 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, 100 107 Pituitary adenomas, chromogranin A and, 121 in children and young adults, 65 66 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, 158 159 current recommendations for, 208, 214 215 in children and young adults, 69 pancreatic localization with, 50 tracers for somatostatin receptor imaging with, 173, 187 188. 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, 45 46 chromogranin A as, 45, 123 124 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, 38 39 Psychosocial functioning, with neuroendocrine tumor syndromes, QOL measurement of, 100 107 PTH-related peptide (PTH-rp), in neuroendocrine tumor syndromes, 30, 37 39 Q Quality of life (QOL), with neuroendocrine tumors, 97 109 chromogranin A correlation to, 121
257 concerns regarding, 97 98 disease-specific questionnaires for measurement of, description of, 100 101, 107 development of, 100 study methods for completing, 101 104 results, 102 105 scales and scores, 101 102 statistical analysis, 102 summary discussion on, 104 107 introduction to, 98 99 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, 224 225 rare functioning pancreatic, 165 Radiographic studies. See Imaging studies. Radioimmunoassays, of neuroendocrine tumors, 135 151 evolutionary advances in, 135, 143, 147 glossary for, 147 history of, 143 147 methodological differences in, 136 sensitivity and specificity of, 141 143, 145 serotonin and tryptophan techniques, 136 143, 146 standardization in laboratories, 135 136 Radiolabeled studies, of neuroendocrine tumors, imaging. See Scintigraphy. plasma, serum, and CSF. See Radioimmunoassays. Radionuclide therapy, for neuroendocrine tumors, chemotherapy combined with, 219, 224 225 per Inter Science Institute Gastrointestinal Council, 224 per New Orleans Louisiana Neuroendocrine Tumor group, 225 somatostatin receptor targeted, for gastroenteropancreatic neuroendocrine tumors, 173 185. See also Peptide receptor radionuclide therapy (PRRT). for neuroendocrine tumors, 187 204. See also Targeted radiopeptide therapy (TRT). Radioreceptor nuclide therapy. See Peptide radioreceptor nuclide therapy (PRRNT). Radiotherapy, targeted, with radiolabeled somatostatin analogs, 187 204. 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, 73 74 surgery for, 168 Relapse, of GEP-NETs, chromogranin A identification of, 124 Renal toxicity, of targeted radiopeptide therapy, for neuroendocrine tumors, 193 194 Resection, of gastroenteropancreatic neuroendocrine tumors, 163, 223, 226 228. See also Surgery. Respiratory symptoms, in neuroendocrine tumor syndromes, QOL measurement with, 100 107
258 Retreatment, with peptide receptor radionuclide therapy, for GEP-NETs, 180 181 Roux-en-Y gastric bypass (RYGBP), laparoscopic, 84 85 S Schwannomas, in children and young adults, 65 66 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, 121 125 in neuroendocrine tumor diagnosis, 121 123 comparison to alternative tumor markers, 121 123 concentration fluctuations, 121 in neuroendocrine tumors prognosis, 123 124 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, 70 71 QOL measurement with, 101 104, 106 radioimmunoassays of, 136 143, 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, 100 107 Single ion monitoring (SIM), in radioimmunoassays, 140 141 Single-photon emission CT (SPECT), of neuroendocrine tumors, 48 49, 154 carcinoid, 157 158 in children and young adults, 69 Skin carcinoma, Merkel cell, chromogranin A and, 120 121 Small bowel, primary neuroendocrine tumors of, 167 Small cell carcinoma, of lungs, chromogranin A and, 120 121 of ovaries and cervix, in children and young adults, 66, 69, 71, 74 Somatostatin analogs, for symptomatic neuroendocrine tumors, 209, 217 218 current recommendations for, 219, 222, 224 225 long-acting, 209, 216 218 in peptide receptor radionuclide therapy, current recommendations for, 219, 222, 224 225 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, 187 188. See also Targeted radiopeptide therapy (TRT). future developments of, 196 197 Somatostatin receptor radionuclide therapy, for gastroenteropancreatic neuroendocrine tumors, 173 185. See also Peptide receptor radionuclide therapy (PRRT).