Synopsis
Classify neuroendocrine tumours by site, differentiation, grade, stage and hormone function, control dangerous secretory syndromes and select surgery, somatostatin analogues, radionuclide, targeted or cytotoxic therapy coherently.
- Non-functioning mass is a pivotal clue in Neuroendocrine tumours: Incidental imaging, pain, jaundice, obstruction or metastasis is more common than a classic hormone syndrome across many NET sites.
- Immediate priority in unstable Neuroendocrine tumours: Stabilise the physiological syndrome, obtain specialist endocrine-oncology input, use intravenous octreotide promptly for suspected carcinoid crisis and treat airway, shock, glucose, bleeding or obstruction through the relevant emergency pathway.
- Core biopsy with expert pathology is used early to establish differentiation, primary-site markers, mitotic count and Ki-67 proliferation index. Report well versus poorly differentiated morphology and grade on adequate tissue; small crushed samples can underestimate heterogeneity.
Key red flags
Episodic flushing, diarrhoea, wheeze and right-sided valvular symptoms suggest carcinoid syndrome.
Profound flushing, bronchospasm, labile blood pressure and shock during tumour stress is an endocrine-oncology emergency.
Investigation priorities
Establish differentiation, primary-site markers, mitotic count and Ki-67 proliferation index.
Management branches
A biopsy, imaging finding or hormone syndrome suggests a neuroendocrine neoplasm.
- Obtain expert pathology with differentiation, mitoses, Ki-67 and site markers, repeating biopsy if small tissue conflicts with clinical behaviour.
- Stage with multiphase anatomical imaging and SSTR PET-CT for well-differentiated disease; add FDG PET when grade or tempo suggests aggressive biology.
Key medicines
Octreotide long-acting releaseA common licensed starting regimen for functioning gastroenteropancreatic NET is 20 mg by deep intramuscular injection every 4 weeks; many specialist pathways use 30 mg every 4 weeks and titrate to symptoms and tumour control.
Lanreotide AutogelUse 120 mg by deep subcutaneous injection every 28 days for antiproliferative treatment of eligible grade 1 or selected grade 2 gastroenteropancreatic NET, with interval adjustment only through the specialist protocol.