The NANETS consensus guideline for the diagnosis and management of neuroendocrine tumors: Well-differentiated neuroendocrine tumors of the jejunum, ileum, appendix, and cecum Journal Article
| Authors: | Boudreaux, J. P.; Klimstra, D. S.; Hassan, M. M.; Woltering, E. A.; Jensen, R. T.; Goldsmith, S. J.; Nutting, C.; Bushnell, D. L.; Caplin, M. E.; Yao, J. C. |
| Article Title: | The NANETS consensus guideline for the diagnosis and management of neuroendocrine tumors: Well-differentiated neuroendocrine tumors of the jejunum, ileum, appendix, and cecum |
| Abstract: | Well-differentiated neuroendocrine tumors (NETs) of the jejunum, ileum, and appendix are also collectively known as midgut carcinoids. Similar to NETs in general, the diagnosed incidence of the midgut NETs is on the rise. Their presenting symptoms vary depending on stage and primary site. Local-regional NETs often present with vague and nonspecific symptoms. Classic carcinoid syndrome is more likely to appear in patients with advanced disease. Local-regional NETs of the small bowel should be resected whenever possible. With the exception of small well-differentiated NETs of the appendix, NETs of the midgut have substantial risk of relapse after resection and need to be followed for at least 7 years.Metastatic/advanced NETs of the midgut are incurable. Optimal management requires a multidisciplinary approach. Somatostatin analogs are effective in the management of carcinoid syndrome. Octreotide long-acting release has also recently been shown to delay disease progression. Liver-directed therapy and surgical debulking can improve quality of life in selected patients. Pivotal phase 3 studies with bevacizumab targeting vascular endothelial growth factor and everolimus targeting mTOR (mammalian target of rapamycin) are ongoing and may lead to improved outcome. Further studies of novel approaches such as peptide receptor radiotherapy are also warranted. Copyright © 2010 by Lippincott Williams & Wilkins. |
| Keywords: | cancer chemotherapy; cancer survival; treatment response; cancer surgery; surgical technique; unclassified drug; overall survival; clinical feature; drug activity; drug tolerability; fatigue; histopathology; review; bevacizumab; doxorubicin; fluorouracil; sunitinib; diarrhea; dose response; hypertension; side effect; drug targeting; alpha interferon; cancer staging; molecular genetics; nuclear magnetic resonance imaging; outcome assessment; positron emission tomography; cancer diagnosis; cancer grading; sensitivity and specificity; anorexia; clinical practice; ki 67 antigen; tumor localization; bolus injection; metastasis; progression free survival; computer assisted tomography; anemia; tumor volume; protein targeting; leukopenia; thrombocytopenia; clinical assessment; differential diagnosis; incidence; prevalence; weight reduction; bradycardia; practice guideline; drug effect; vasculotropin inhibitor; risk factor; temsirolimus; flushing; loading drug dose; neuroendocrine tumor; imaging system; hypotension; survival time; immunocytochemistry; cardiovascular disease; mammalian target of rapamycin; fluorodeoxyglucose f 18; appendix; medical society; drug infusion; vitamin supplementation; cancer registry; world health organization; perioperative period; octreotide; malabsorption; cancer epidemiology; preoperative treatment; corticosteroid; intestinal neoplasms; chromogranin; tumor classification; everolimus; dopamine; angiopeptin; scintigraphy; cecum; ileum; jejunum; bone marrow toxicity; somatostatin; neuroendocrine tumors; bronchospasm; antihistaminic agent; vasomotor disorder; vasopressin; streptozocin; synaptophysin; 5 hydroxyindoleacetic acid; kreon; long acting drug; nicotinamide; pancrelipase; phenylephrine; recombinant alpha2a interferon; recombinant alpha2b interferon; sorafinib; antibody labeling; carcinoid syndrome; midgut; nicotinic acid deficiency; pellagra; wheezing |
| Journal Title: | Pancreas |
| Volume: | 39 |
| Issue: | 6 |
| ISSN: | 0885-3177 |
| Publisher: | Lippincott Williams & Wilkins |
| Date Published: | 2010-08-01 |
| Start Page: | 753 |
| End Page: | 766 |
| Language: | English |
| DOI: | 10.1097/MPA.0b013e3181ebb2a5 |
| PUBMED: | 20664473 |
| PROVIDER: | scopus |
| DOI/URL: | |
| Notes: | --- - "Cited By (since 1996): 2" - "Export Date: 20 April 2011" - "CODEN: PANCE" - "Source: Scopus" |
