Journal article
The NANETS Consensus Guideline for the Diagnosis and Management of Neuroendocrine Tumors Well-Differentiated Neuroendocrine Tumors of the Jejunum, Ileum, Appendix, and Cecum
Pancreas, Vol.39(6), pp.753-766
08/01/2010
DOI: 10.1097/MPA.0b013e3181ebb2a5
PMID: 20664473
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.
Details
- Title: Subtitle
- The NANETS Consensus Guideline for the Diagnosis and Management of Neuroendocrine Tumors Well-Differentiated Neuroendocrine Tumors of the Jejunum, Ileum, Appendix, and Cecum
- Creators
- J. Philip Boudreaux - Louisiana State UniversityDavid S. Klimstra - Memorial Sloan Kettering Cancer CenterManal M. Hassan - GI Medical OncologyEugene A. Woltering - Louisiana State UniversityRobert T. Jensen - National Institutes of Health, BethesdaStanley J. Goldsmith - Cornell UniversityCharles Nutting - Swedish Medical CenterDavid L. Bushnell - University of IowaMartyn E. Caplin - University College LondonJames C. Yao - GI Medical OncologyNorth American Neuroendocrine Tumor Society (NANETS)
- Resource Type
- Journal article
- Publication Details
- Pancreas, Vol.39(6), pp.753-766
- Publisher
- Lippincott Williams & Wilkins
- DOI
- 10.1097/MPA.0b013e3181ebb2a5
- PMID
- 20664473
- ISSN
- 0885-3177
- eISSN
- 1536-4828
- Number of pages
- 14
- Grant note
- ZIADK053200 / NATIONAL INSTITUTE OF DIABETES AND DIGESTIVE AND KIDNEY DISEASES; United States Department of Health & Human Services; National Institutes of Health (NIH) - USA; NIH National Institute of Diabetes & Digestive & Kidney Diseases (NIDDK)
- Language
- English
- Date published
- 08/01/2010
- Academic Unit
- Radiology
- Record Identifier
- 9984318803702771
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