With the ENETS guidelines update, there are a number of key issues tackled with this common subtype of NET. Terminology is clarified. Whether one uses neuroendocrine tumour, or neoplasm, as mentioned in the guidelines, the terms midgut or hindgut are thought not to be as helpful as indicating the primary anatomical site e.g. jejuno-ileal, rectal, colonic, caecal, appendiceal, etc.
Grading with Ki-67 proliferation index, assessed from histology, is considered to be mandatory for prognostication. Ideally a 68Gallium-labelled octreotide PET-CT scan should be obtained after diagnosis with CT or MR imaging and if not available, somatostatin receptor scintigraphy SPECT/CT. CgA and urinary 5-HIAA should be performed on diagnosis and on follow up.
Palliative resection in the presence of liver metastases is discussed in the updated guidelines. Resection should be considered in symptomatic patients with pending obstruction on imaging and overall outcome is better in asymptomatic patients although the direct causal relationship has not been proven.
Annual transthoracic echocardiography should be performed annually for patients with carcinoid syndrome and carcinoid heart disease. Nt-proBNP and cardiac MRI are considered to be useful for assessing these patients. Closure of any PFO is debated but it is suggested in these guidelines acknowledging there is limited data for this approach.
Management of distant disease (liver metastases) is discussed in a separate guideline but includes somatostatin analogues, interferon, surgical resection (including de-bulking >90% or resection of <90% burden for symptoms), PRRT, with Everolimus now also an alternative option.
05 Dec 2016
UK and Ireland Neuroendocrine Tumour Society