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Dr Karel Pacak, USA 18 November 2018

Classification of NETs – Carcinoids and gastroenteropancreatic tumors (GEPs); Chromaffin cell tumors; Multiple endocrine neoplasia (MEN)1-, MEN2-, neurofibromatosis (NF)1-related NETs; Medullary thyroid carcinoma (MTC); Poorly differentiated small cell carcinoma; NETs of unknown origin.
Early accurate diagnosis leads to better prognosis.
NETS – Specific characteristics: Take up hormone precursors; Synthesize, store and release hormones; Express specific transporters and receptors; Have specific metabolomic profiles; They are immunogenically cold.
Endoscopic methods in NETs negative on imaging – Capsule endoscopy; Double balloon enteroscopy.
The North American Neuroendocrine Society (NANETS) recommendation or NETs -
1. Surgery: 50% recurrence rate; surveillance should continue beyond 5 years, not clear if 10 years but recommended, especially for young patients and those with positive LNs;
2. Surveillance: every 6-12 months, CT/MRI abdomen, initially Octreoscan or ⁶⁸Ga-DOTATAE recommended.
A study evaluated the efficacy and safety of lutetium-177 (¹⁷⁷Lu)-DOTATAE in patients with advanced, progressive, somatostatin-receptor-positive midgut NETs. The treatment led to markedly longer progression-free survival and a significantly higher response rate than high-dose octreotide LAR. (Strosberg J, et al. N Engl J Med. 2017 Jan 12;376(2):125-135).

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