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10 slides generated from the cancer page, with a quiz from the open benchmark and speaker notes that cite the sources. Arrow keys move between slides; Print gives one slide per page.
A family of usually slow-growing tumours that start in hormone-producing cells of the gut, pancreas and lungs. They pioneered the idea of using the same molecule to see a tumour on a scan and then to treat it with radiation.
Neuroendocrine neoplasms range from indolent grade 1 tumours that patients live with for decades to poorly differentiated neuroendocrine carcinomas that behave like small-cell lung cancer. Most arise in the small bowel, pancreas, rectum or lung; many secrete hormones (serotonin, insulin, gastrin) that cause syndromes, and most well-differentiated tumours express somatostatin receptor 2 (SSTR2), which is the hinge of both diagnosis and therapy. Incidence has risen six-fold over 40 years, largely from incidental detection on imaging and endoscopy.
Therapy is sequenced by grade, receptor status and tempo. Somatostatin analogues (octreotide, lanreotide) control symptoms and slow growth (PROMID, CLARINET). For progression, peptide receptor radionuclide therapy with 177Lu-DOTATATE (NETTER-1; NETTER-2 first line for grade 2-3) is standard, and 177Lu-edotreotide beat everolimus head-to-head in COMPETE (PFS 23.9 vs 14.1 months) with an FDA decision due August 2026. Targeted pills (everolimus, sunitinib, and since March 2025 cabozantinib after CABINET) and chemotherapy (CAPTEM for pancreatic NETs; platinum-etoposide for neuroendocrine carcinoma) fill in. Surgery and liver-directed therapy (resection, embolisation, ablation, transplant in rare cases) remain central because disease is often liver-dominant.
| Setting | Approach | Guideline |
|---|---|---|
| Localised | Resection. | not mapped |
| Advanced | SSA → 177Lu-DOTATATE → everolimus/cabozantinib/chemotherapy; alpha therapy in trials. | not mapped |
| Diagnosis and staging | Histology with Ki-67 grading; 68Ga/64Cu-DOTATATE PET/CT ± FDG PET; triple-phase CT or MRI of the liver; chromogranin A and syndrome-specific hormones; germline testing for pancreatic NETs and paragangliomas. | NCCN Neuroendocrine and Adrenal Tumors |
| Localised disease | Surgical resection (including primary tumour resection with liver metastases where feasible); endoscopic resection for small rectal/gastric NETs; surveillance for small incidental lesions. | NCCN Category 2A |
| Advanced, grade 1-2, SSTR-positive, first line | Somatostatin analogue (octreotide LAR or lanreotide); 177Lu-DOTATATE first line for grade 2-3 (NETTER-2) with high burden. | NCCN Category 1 (SSA); category 1 PRRT for grade 2-3 first line |
| Advanced, progression on SSA | PRRT with 177Lu-DOTATATE (or 177Lu-edotreotide if approved); everolimus; sunitinib (pancreatic); cabozantinib (CABINET, all sites). | NCCN Category 1 for PRRT and cabozantinib; 2A sequencing |
| Advanced pancreatic NET needing tumour shrinkage | CAPTEM (E2211); PRRT; liver-directed therapy for hepatic-dominant disease. | NCCN Category 2A |
| Carcinoid syndrome | SSA dose escalation; telotristat ethyl for refractory diarrhoea; octreotide infusion peri-procedurally; echocardiographic screening for carcinoid heart disease. | NCCN Category 2A |