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- NET, neuroendocrine tumour
- MEN, multiple endocrine neoplasia
- NF1, neurofibromatosis type 1
- CgA, chromogranin A
- PTH, parathyroid hormone
- CEA, carcinoembryonic antigen
- β-HCG, β-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
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 C). Others that may be appropriate include thyroid function tests (TFTs), parathyroid hormone (PTH), calcium, calcitonin, prolactin, α-fetoprotein, carcinoembryonic antigen (CEA), and β-human chorionic gonadotrophin (β-HCG) (grade D);
specific biochemical tests should be requested depending on which syndrome is suspected (see table 4).
For detecting the primary tumour, a multimodality approach is best and may include computed tomography (CT), magnetic resonance imaging (MRI), somatostatin receptor scintigraphy (SSRS), endoscopic ultrasound (EUS), endoscopy, digital subtraction angiography (DSA), and venous sampling (grade B/C).
For assessing secondaries, SSRS is the most sensitive modality (grade B).
When a primary has been resected, SSRS may be indicated for follow up1 (grade D).
The extent of the tumour, its metastases, and secretory profile should be determined as far as possible before planning treatment (grade C).
Surgery should be offered to patients who are fit and have limited disease—that is, primary±regional lymph nodes (grade C).
Surgery should …
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