Principles of diagnosis and management of neuroendocrine tumours

• Published in: Canadian Medical Association journal (CMAJ) 2017-03, Vol.189 (10), p.E398-E404
• Main Authors: Raphael, Michael J, Chan, David L, Law, Calvin, Singh, Simron
• Format: Article
• Language: English
• Subjects: Angiogenesis; Cancer therapies; Care and treatment; Cell growth; Cervical cancer; Chemotherapy; Chemotherapy, Adjuvant; Development and progression; Diarrhea; Endoscopy, Digestive System; Hormones; Humans; Intestinal Neoplasms - complications; Intestinal Neoplasms - diagnosis; Intestinal Neoplasms - pathology; Intestinal Neoplasms - therapy; Ischemia; Lung cancer; Lung Neoplasms - complications; Lung Neoplasms - diagnosis; Lung Neoplasms - pathology; Lung Neoplasms - therapy; Magnetic Resonance Imaging; Malignant Carcinoid Syndrome - etiology; Medical prognosis; Metabolism; Metastasis; Neoplasm Grading; Neoplasm Staging; Neuroendocrine Tumors - complications; Neuroendocrine Tumors - diagnosis; Neuroendocrine Tumors - pathology; Neuroendocrine Tumors - therapy; Pancreatic Neoplasms - complications; Pancreatic Neoplasms - diagnosis; Pancreatic Neoplasms - pathology; Pancreatic Neoplasms - therapy; Peptides; Pharmaceutical industry; Positron Emission Tomography Computed Tomography; Principles; Quality of life; Review; Surgical Procedures, Operative; Systematic review; Tomography, X-Ray Computed; Tumors
• ISSN: 0820-3946; 1488-2329
• DOI: 10.1503/cmaj.160771

The second modality is functional imaging, which takes advantage of the overexpression of somatostatin receptors commonly seen in NETs. Radiolabelled somatostatin analogues are administered intravenously, concentrate in NETs, and the emitted radiation is detected to localize tumours. 111Indium (In)labelled pentetreotide is the most commonly used radiotracer in Canada. A recent meta-analysis of molecular imaging methods for NETs reported that 111In-labelled pentetreotide somatostatin receptor scintigraphic imaging had a sensitivity of 46%-100% for abdominal NETs, 46%-83% for pancreatic NETs and 71% for bronchial NETs.45 Everolimus is an oral mammalian target of rapamycin (mTOR) inhibitor that is thought to act by inhibiting cell proliferation, angiogenesis and cell metabolism. Everolimus has proven progression-free survival benefit in well-conducted phase 3 RCTs in gastrointestinal and lung NETs,62-64 and pancreatic NETs.65,66 In the RADIANT-4 phase III RCT involving 302 patients with advanced progressive nonfunctional lung or gastrointestinal NETs, everolimus was associated with a 52% reduction in the risk of progression or death (HR 0.48, 95% CI 0.35-0.67) compared with placebo. Similarly, in the RADIANT-3 phase 3 RCT involving 410 patients with advanced low- or intermediate-grade pancreatic NETs, everolimus was associated with a 65% reduction in the risk of progression or death (HR 0.35, 95% CI 0.27-0.45) compared with placebo. We searched MEDLINE, Embase, Google Scholar and the Cochrane Database of Systemic Reviews for articles relating to the diagnosis, classification and management of neuroendocrine tumours. Search terms included "neuroendocrine tumors," "neuroendocrine neoplasms," "carcinoid tumors," "islet cell tumors" and "bronchopulmonary neuroendocrine tumors." We then searched for abstracts from major international oncology conferences to identify any recently completed studies. The reference lists of all relevant articles were reviewed for studies not captured in the original search.