Gastrointestinal neuroendocrine (carcinoid) tumours: current diagnosis and management.
Neuroendocrine tumours (NETs) are increasing in both incidence and prevalence and, as a group, are more prevalent than either gastric, pancreatic, oesophageal or hepatobiliary adenocarcinomas, or any two of these cancers combined. Clinical awareness of the protean and intermittent symptoms of NETs (eg, sweating, flushing, diarrhoea, and bronchospasm) is critical for timely diagnosis; however, the classical carcinoid syndrome is relatively uncommon. The most useful diagnostic test for gastrointestinal NETs is measurement of plasma chromogranin A (CgA) levels. Disease extent is assessed by both anatomical imaging, and nuclear imaging with radiolabelled somatostatin analogues. Pathological evaluation comprises tumour-node-metastasis classification, a minimum pathological dataset, CgA and synaptophysin immunostaining, as well as mitotic count or Ki-67 index (a marker of cell proliferation) to define grading. Resection of the primary lesion and as much metastatic disease as possible increases the efficacy of medical therapy. Other management strategies include hepatic embolisation and peptide receptor radionuclide therapy. Patients with tumours expressing somatostatin receptors should be treated with somatostatin analogues. Depending on the tumour grade, other effective agents include cytotoxics, tyrosine kinase inhibitors, and antiangiogenics. The overarching requirement for best management of patients with NETs is to ensure that they have ready access to experienced multidisciplinary clinician groups located within centres of appropriate subspecialty expertise.
Modlin
Irvin M
IM
School of Medicine, Yale University, New Haven, Conn, USA. irvin.modlin@yale.edu
Moss
Steven F
SF
Oberg
Kjell
K
Padbury
Robert
R
Hicks
Rodney J
RJ
Gustafsson
Bjorn I
BI
Wright
Nicholas A
NA
Kidd
Mark
M
D002276:Carcinoid Tumor / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology / Q000556:secondary / Q000628:therapy*; D053379:Chromogranin A / Q000097:blood; D016099:Endoscopy, Gastrointestinal; D005770:Gastrointestinal Neoplasms / Q000175:diagnosis* / Q000401:mortality / Q000473:pathology / Q000628:therapy*; D006801:Humans; D007414:Intestinal Neoplasms / Q000175:diagnosis / Q000628:therapy; D008113:Liver Neoplasms / Q000556:secondary; D010190:Pancreatic Neoplasms / Q000175:diagnosis / Q000628:therapy; D011379:Prognosis; D018000:Receptors, Peptide; D013004:Somatostatin / Q000031:analogs & derivatives; D013274:Stomach Neoplasms / Q000175:diagnosis / Q000628:therapy
D053379:Chromogranin A; D018000:Receptors, Peptide; D013004:Somatostatin