Neuroendocrine and hormone-secreting tumours
Grading by Ki-67 and differentiation, carcinoid syndrome and carcinoid heart disease, the functioning pancreatic tumours, the right tests (urine 5-HIAA, Ga-68 DOTATATE PET, chromogranin A and its false positives) and the treatment ladder from somatostatin analogues to Lu-177 DOTATATE.
Tier 2Condition
Written with AI assistance and fact-checked against the sources below. For exam study, not clinical decisions. How notes are made
At a glance
- Neuroendocrine neoplasms arise from neuroendocrine cells, mostly in the gut, pancreas and lung. Incidence is rising, largely from incidental detection.
- Well-differentiated NETs (graded by Ki-67) behave very differently from poorly differentiated neuroendocrine carcinomas, which are treated like small cell cancer.
- Most NETs are non-functioning. A minority cause a hormone syndrome: carcinoid syndrome, insulinoma, gastrinoma, VIPoma, glucagonoma, ectopic ACTH.
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Sources
- European Neuroendocrine Tumour Society (ENETS) 2024 guidance paper for the management of well-differentiated small intestine neuroendocrine tumoursJournal of Neuroendocrinology (ENETS), 2024
- ENETS 2023 guidance paper for nonfunctioning pancreatic neuroendocrine tumoursJournal of Neuroendocrinology (ENETS), 2023
- ENETS 2023 guidance paper for functioning pancreatic neuroendocrine tumour syndromesJournal of Neuroendocrinology (ENETS), 2023
- ENETS 2022 guidance paper for carcinoid syndrome and carcinoid heart diseaseJournal of Neuroendocrinology (ENETS), 2022
- [177Lu]Lu-DOTA-TATE plus long-acting octreotide versus high-dose long-acting octreotide for first-line treatment of advanced grade 2 or 3 gastroenteropancreatic neuroendocrine tumours (NETTER-2)Lancet (Singh S, et al.; 403:2807-17), 2024
- Phase 3 trial of cabozantinib to treat advanced neuroendocrine tumors (CABINET)New England Journal of Medicine (Chan JA, et al.; 392:653-65), 2025
- Lanreotide (Mytolac): PBAC outcome for acromegaly, functional carcinoid tumour and non-functional GEP-NETAU/NZPharmaceutical Benefits Scheme, 2022
- PBS Schedule: octreotide 30 mg modified release injection, non-functional GEP-NET restriction (checked November 2026)AU/NZAustralian Government Department of Health, Disability and Ageing, 2026
- Last updated
- 14 November 2026
What changed
- 14 November 2026 Personal emails, About story and Australian-style copy