GUIDELINES

Guidelines for the management of gastroenteropancreatic neuroendocrine (including carcinoid) tumours

J K Ramage^*, A H G Davies^*, J Ardill, N Bax, M Caplin, A Grossman, R Hawkins, A M McNicol, N Reed, R Sutton, R Thakker, S Aylwin, D Breen, K Britton, K Buchanan, P Corrie, A Gillams, V Lewington, D McCance, K Meeran, A Watkinson on behalf of UKNETwork for neuroendocrine tumours

Correspondence to:
Correspondence to:
Dr J Ramage
North Hampshire Hospital, Aldermaston Road, Basingstoke, Hants, UK; johnramage1@compuserve.com

Abbreviations: NET, neuroendocrine tumour; MEN, multiple endocrine neoplasia; NF1, neurofibromatosis type 1; CgA, chromogranin A; PTH, parathyroid hormone; CEA, carcinoembryonic antigen; ß-HCG, ß-human chorionic gonadotrophin; 5-HIAA, 5-hydroxy indole acetic acid; ACTH, adrenocorticotrophic hormone; CT, computed tomography; MRI, magnetic resonance imaging; SSRS, somatostatin receptor scintigraphy; SSTR, somatostatin receptors; EUS, endoscopic ultrasound; TFTs, thyroid function tests; DSA, digital subtraction angiography; SMS, somatostatin

Keywords: guidelines; gastroenteropancreatic neuroendocrine tumours; carcinoid tumours

The first 150 words of the full text of this article appear below.

1.0 SUMMARY OF RECOMMENDATIONS

• Clinical examination to exclude complex cancer syndromes (for example, multiple endocrine neoplasia 1 (MEN1)) should be performed in all cases of neuroendocrine tumours (NETs), and a family history taken (grade C).
  
• In all cases where there is a family history of carcinoids or NET, or a second endocrine tumour, a familial syndrome should be suspected (grade C).
  
• Individuals with sporadic or familial bronchial or gastric carcinoid should have a family history evaluation and consideration of testing for germline MEN1 mutations. Management of MEN1 families includes screening for endocrine parathyroid and enteropancreatic tumours from late childhood, with predictive testing for first degree relatives of known mutation carriers (grade C).
  
• All patients should be evaluated for second endocrine tumours and possibly for other gut cancers (grade C)
  

If a patient presents with symptoms suspicious of a gastroenteropancreatic NET:

• baseline tests should include chromogranin A (CgA) and 5-hydroxy indole acetic acid (5-HIAA) (grade . . . [Full text of this article]

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The site distribution of gastrointestinal carcinoids differs between races

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