Gastroenteropancreatic neuroendocrine tumours

• 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
• ISSN: 1470-2045; 1474-5488; 1474-5488
• DOI: 10.1016/S1470-2045(07)70410-2

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.