Gastroenteropancreatic neuroendocrine tumours

Gastroenteropancreatic (GEP) neuroendocrine tumours (NETs) are fairly rare neoplasms that present many clinical challenges. They secrete peptides and neuroamines that cause distinct clinical syndromes, including carcinoid syndrome. However, many are clinically silent until late presentation with mas...

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Published in	The lancet oncology Vol. 9; no. 1; pp. 61 - 72
Main Authors	Modlin, Irvin M, Oberg, Kjell, Chung, Daniel C, Jensen, Robert T, de Herder, Wouter W, Thakker, Rajesh V, Caplin, Martyn, Delle Fave, Gianfranco, Kaltsas, Greg A, Krenning, Eric P, Moss, Steven F, Nilsson, Ola, Rindi, Guido, Salazar, Ramon, Ruszniewski, Philippe, Sundin, Anders
Format	Journal Article
Language	English
Published	England Elsevier Ltd 2008 Elsevier Limited
Subjects	Animals Biology Biomarkers, Tumor Black people Cancer therapies Carcinoma, Neuroendocrine - classification Carcinoma, Neuroendocrine - metabolism Carcinoma, Neuroendocrine - pathology Disease Epidemiology Ethnicity Gastrointestinal Neoplasms - metabolism Gastrointestinal Neoplasms - physiopathology Gastrointestinal Neoplasms - therapy Hematology, Oncology and Palliative Medicine Humans Kinases MEDICIN MEDICINE Molecular Biology Neuroendocrine tumors Pancreatic Neoplasms - metabolism Pancreatic Neoplasms - physiopathology Pancreatic Neoplasms - therapy Patients Peptides Protein-Tyrosine Kinases - antagonists & inhibitors Small intestine Somatostatin - analogs & derivatives Somatostatin - metabolism Somatostatin - therapeutic use White people United States > US
Online Access	Get full text
ISSN	1470-2045 1474-5488 1474-5488
DOI	10.1016/S1470-2045(07)70410-2

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Abstract	Gastroenteropancreatic (GEP) neuroendocrine tumours (NETs) are fairly rare neoplasms that present many clinical challenges. They secrete peptides and neuroamines that cause distinct clinical syndromes, including carcinoid syndrome. However, many are clinically silent until late presentation with mass effects. Investigation and management should be highly individualised for a patient, taking into consideration the likely natural history of the tumour and general health of the patient. Management strategies include surgery for cure (which is achieved rarely) or for cytoreduction, radiological intervention (by chemoembolisation and radiofrequency ablation), chemotherapy, and somatostatin analogues to control symptoms that result from release of peptides and neuroamines. New biological agents and somatostatin-tagged radionuclides are under investigation. The complexity, heterogeneity, and rarity of GEP NETs have contributed to a paucity of relevant randomised trials and little or no survival increase over the past 30 years. To improve outcome from GEP NETs, a better understanding of their biology is needed, with emphasis on molecular genetics and disease modeling. More-reliable serum markers, better tumour localisation and identification of small lesions, and histological grading systems and classifications with prognostic application are needed. Comparison between treatments is currently very difficult. Progress is unlikely to occur without development of centers of excellence, with dedicated combined clinical teams to coordinate multicentre studies, maintain clinical and tissue databases, and refine molecularly targeted therapeutics.
AbstractList	Gastroenteropancreatic (GEP) neuroendocrine tumours (NETs) are fairly rare neoplasms that present many clinical challenges. They secrete peptides and neuroamines that cause distinct clinical syndromes, including carcinoid syndrome. However, many are clinically silent until late presentation with mass effects. Investigation and management should be highly individualised for a patient, taking into consideration the likely natural history of the tumour and general health of the patient. Management strategies include surgery for cure (which is achieved rarely) or for cytoreduction, radiological intervention (by chemoembolisation and radiofrequency ablation), chemotherapy, and somatostatin analogues to control symptoms that result from release of peptides and neuroamines. New biological agents and somatostatin-tagged radionuclides are under investigation. The complexity, heterogeneity, and rarity of GEP NETs have contributed to a paucity of relevant randomised trials and little or no survival increase over the past 30 years. To improve outcome from GEP NETs, a better understanding of their biology is needed, with emphasis on molecular genetics and disease modeling. More-reliable serum markers, better tumour localisation and identification of small lesions, and histological grading systems and classifications with prognostic application are needed. Comparison between treatments is currently very difficult. Progress is unlikely to occur without development of centers of excellence, with dedicated combined clinical teams to coordinate multicentre studies, maintain clinical and tissue databases, and refine molecularly targeted therapeutics. Gastroenteropancreatic (GEP) neuroendocrine tumours (NETs) are fairly rare neoplasms that present many clinical challenges. They secrete peptides and neuroamines that cause distinct clinical syndromes, including carcinoid syndrome. However, many are clinically silent until late presentation with mass effects. Investigation and management should be highly individualised for a patient, taking into consideration the likely natural history of the tumour and general health of the patient. Management strategies include surgery for cure (which is achieved rarely) or for cytoreduction, radiological intervention (by chemoembolisation and radiofrequency ablation), chemotherapy, and somatostatin analogues to control symptoms that result from release of peptides and neuroamines. New biological agents and somatostatin-tagged radionuclides are under investigation. The complexity, heterogeneity, and rarity of GEP NETs have contributed to a paucity of relevant randomised trials and little or no survival increase over the past 30 years. To improve outcome from GEP NETs, a better understanding of their biology is needed, with emphasis on molecular genetics and disease modeling. More-reliable serum markers, better tumour localisation and identification of small lesions, and histological grading systems and classifications with prognostic application are needed. Comparison between treatments is currently very difficult. Progress is unlikely to occur without development of centers of excellence, with dedicated combined clinical teams to coordinate multicentre studies, maintain clinical and tissue databases, and refine molecularly targeted therapeutics.Gastroenteropancreatic (GEP) neuroendocrine tumours (NETs) are fairly rare neoplasms that present many clinical challenges. They secrete peptides and neuroamines that cause distinct clinical syndromes, including carcinoid syndrome. However, many are clinically silent until late presentation with mass effects. Investigation and management should be highly individualised for a patient, taking into consideration the likely natural history of the tumour and general health of the patient. Management strategies include surgery for cure (which is achieved rarely) or for cytoreduction, radiological intervention (by chemoembolisation and radiofrequency ablation), chemotherapy, and somatostatin analogues to control symptoms that result from release of peptides and neuroamines. New biological agents and somatostatin-tagged radionuclides are under investigation. The complexity, heterogeneity, and rarity of GEP NETs have contributed to a paucity of relevant randomised trials and little or no survival increase over the past 30 years. To improve outcome from GEP NETs, a better understanding of their biology is needed, with emphasis on molecular genetics and disease modeling. More-reliable serum markers, better tumour localisation and identification of small lesions, and histological grading systems and classifications with prognostic application are needed. Comparison between treatments is currently very difficult. Progress is unlikely to occur without development of centers of excellence, with dedicated combined clinical teams to coordinate multicentre studies, maintain clinical and tissue databases, and refine molecularly targeted therapeutics. Summary Gastroenteropancreatic (GEP) neuroendocrine tumours (NETs) are fairly rare neoplasms that present many clinical challenges. They secrete peptides and neuroamines that cause distinct clinical syndromes, including carcinoid syndrome. However, many are clinically silent until late presentation with mass effects. Investigation and management should be highly individualised for a patient, taking into consideration the likely natural history of the tumour and general health of the patient. Management strategies include surgery for cure (which is achieved rarely) or for cytoreduction, radiological intervention (by chemoembolisation and radiofrequency ablation), chemotherapy, and somatostatin analogues to control symptoms that result from release of peptides and neuroamines. New biological agents and somatostatin-tagged radionuclides are under investigation. The complexity, heterogeneity, and rarity of GEP NETs have contributed to a paucity of relevant randomised trials and little or no survival increase over the past 30 years. To improve outcome from GEP NETs, a better understanding of their biology is needed, with emphasis on molecular genetics and disease modeling. More-reliable serum markers, better tumour localisation and identification of small lesions, and histological grading systems and classifications with prognostic application are needed. Comparison between treatments is currently very difficult. Progress is unlikely to occur without development of centers of excellence, with dedicated combined clinical teams to coordinate multicentre studies, maintain clinical and tissue databases, and refine molecularly targeted therapeutics.
Author	Oberg, Kjell Caplin, Martyn Krenning, Eric P Ruszniewski, Philippe Jensen, Robert T Moss, Steven F Rindi, Guido Modlin, Irvin M Nilsson, Ola Kaltsas, Greg A Chung, Daniel C Salazar, Ramon de Herder, Wouter W Thakker, Rajesh V Delle Fave, Gianfranco Sundin, Anders
Author_xml	– sequence: 1 givenname: Irvin M surname: Modlin fullname: Modlin, Irvin M email: imodlin@optonline.net organization: Department of Gastroenterological Surgery, Yale University, New Haven, CT, USA – sequence: 2 givenname: Kjell surname: Oberg fullname: Oberg, Kjell organization: Endocrine Oncology Unit, Department of Internal Medicine, University Hospital Uppsala, Sweden – sequence: 3 givenname: Daniel C surname: Chung fullname: Chung, Daniel C organization: Massachusetts General Hospital, Harvard University, Boston, MA, USA – sequence: 4 givenname: Robert T surname: Jensen fullname: Jensen, Robert T organization: Digestive Diseases Branch, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD, USA – sequence: 5 givenname: Wouter W surname: de Herder fullname: de Herder, Wouter W organization: Department of Internal Medicine III, University Hospital Rotterdam, Erasmus Medical Centre, Rotterdam, Netherlands – sequence: 6 givenname: Rajesh V surname: Thakker fullname: Thakker, Rajesh V organization: Academic Endocrine Unit, Nuffield Department of Clinical Medicine, University of Oxford, Oxford, UK – sequence: 7 givenname: Martyn surname: Caplin fullname: Caplin, Martyn organization: Neuroendocrine Tumour Unit, Centre for Gastroenterology, Royal Free Hospital, London, UK – sequence: 8 givenname: Gianfranco surname: Delle Fave fullname: Delle Fave, Gianfranco organization: Department of Digestive and Liver Disease, University of La Sapienza, Rome, Italy – sequence: 9 givenname: Greg A surname: Kaltsas fullname: Kaltsas, Greg A organization: Department of Endocrinology, George Genimatas Hospital, Athens, Greece – sequence: 10 givenname: Eric P surname: Krenning fullname: Krenning, Eric P organization: Department of Nuclear Medicine, Erasmus Medical Centre, Rotterdam, Netherlands – sequence: 11 givenname: Steven F surname: Moss fullname: Moss, Steven F organization: Division of Gastroenterology, Department of Medicine, Rhode Island Hospital, Brown University, Providence, RI, USA – sequence: 12 givenname: Ola surname: Nilsson fullname: Nilsson, Ola organization: Lundberg Laboratory for Cancer Research at the Department of Pathology, Sahlgrenska University Hospital, Gothenburg, Sweden – sequence: 13 givenname: Guido surname: Rindi fullname: Rindi, Guido organization: Department of Pathology and Laboratory Medicine, University of Parma, Parma, Italy – sequence: 14 givenname: Ramon surname: Salazar fullname: Salazar, Ramon organization: Department of Medical Oncology, Catalan Institute of Oncology, Barcelona, Spain – sequence: 15 givenname: Philippe surname: Ruszniewski fullname: Ruszniewski, Philippe organization: Department of Gastroenterology, Beaujon Hospital, Clichy, France – sequence: 16 givenname: Anders surname: Sundin fullname: Sundin, Anders organization: Centre for Medical Imaging, Department of Radiology, Uppsala University, Uppsala, Sweden
BackLink	https://www.ncbi.nlm.nih.gov/pubmed/18177818$$D View this record in MEDLINE/PubMed https://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-105420$$DView record from Swedish Publication Index
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Snippet	Gastroenteropancreatic (GEP) neuroendocrine tumours (NETs) are fairly rare neoplasms that present many clinical challenges. They secrete peptides and... Summary Gastroenteropancreatic (GEP) neuroendocrine tumours (NETs) are fairly rare neoplasms that present many clinical challenges. They secrete peptides and...
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SubjectTerms	Animals Biology Biomarkers, Tumor Black people Cancer therapies Carcinoma, Neuroendocrine - classification Carcinoma, Neuroendocrine - metabolism Carcinoma, Neuroendocrine - pathology Disease Epidemiology Ethnicity Gastrointestinal Neoplasms - metabolism Gastrointestinal Neoplasms - physiopathology Gastrointestinal Neoplasms - therapy Hematology, Oncology and Palliative Medicine Humans Kinases MEDICIN MEDICINE Molecular Biology Neuroendocrine tumors Pancreatic Neoplasms - metabolism Pancreatic Neoplasms - physiopathology Pancreatic Neoplasms - therapy Patients Peptides Protein-Tyrosine Kinases - antagonists & inhibitors Small intestine Somatostatin - analogs & derivatives Somatostatin - metabolism Somatostatin - therapeutic use White people
Title	Gastroenteropancreatic neuroendocrine tumours
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