Neuroendocrine tumours: lines of therapy

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11 standard-of-care settings across 5 lines and 2 biomarker subgroups. Rows come from the cancer page's standard of care; the grid places each on its line and subgroup.

Line	All comers	HR-positive
Screening, prevention and diagnosis	1	·
Early / localised	2	·
Advanced, first line	3	·
Second line	1	·
Other settings	3	1

Screening, prevention and diagnosis

Subgroup	Setting	Approach	Products and trials	Guideline	Evidence
All comers	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.	Somatostatin receptor PET (68Ga/64Cu-DOTATATE)Neuroendocrine tumour grade (Ki-67) and WHO classificationChromogranin AGermline (hereditary) testingMEN1 and hereditary neuroendocrine syndromes	NCCN · Neuroendocrine and Adrenal Tumors	75

Early / localised

Subgroup	Setting	Approach	Products and trials	Guideline	Evidence
All comers	Localised	Resection.		NCCN Guidelines: Neuroendocrine and Adrenal Tumors	—
All comers	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.	Robotic & minimally invasive surgery	NCCN · Category 2A	40

Advanced, first line

Subgroup	Setting	Approach	Products and trials	Guideline	Evidence
All comers	Advanced	SSA → 177Lu-DOTATATE → everolimus/cabozantinib/chemotherapy; alpha therapy in trials.	Lutetium-177 dotatateActinium-225 DOTATATE	NCCN Guidelines: Neuroendocrine and Adrenal Tumors	59
All comers	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.	Somatostatin analogues (octreotide, lanreotide)PROMIDCLARINETLutetium-177 dotatatePeptide receptor radionuclide therapy (PRRT)	NCCN · Category 1 (SSA); category 1 PRRT for grade…	84
All comers	Advanced pancreatic NET needing tumour shrinkage	CAPTEM (E2211); PRRT; liver-directed therapy for hepatic-dominant disease.	Capecitabine + temozolomide (CAPTEM)Transarterial chemoembolisation (TACE)Radioembolisation (TARE / SIRT, yttrium-90)Thermal ablation (RFA, microwave, cryo)	NCCN · Category 2A	40

Second line

Subgroup	Setting	Approach	Products and trials	Guideline	Evidence
All comers	Advanced, progression on SSA	PRRT with 177Lu-DOTATATE (or 177Lu-edotreotide if approved); everolimus; sunitinib (pancreatic); cabozantinib (CABINET, all sites).	Lutetium-177 dotatate177Lu-edotreotideCOMPETEEverolimusRADIANT-3 and RADIANT-4SunitinibCabozantinibCABINET (Alliance A021602)	NCCN · Category 1 for PRRT and cabozantinib; 2A se…	95

Other settings

Subgroup	Setting	Approach	Products and trials	Guideline	Evidence
All comers	Carcinoid syndrome	SSA dose escalation; telotristat ethyl for refractory diarrhoea; octreotide infusion peri-procedurally; echocardiographic screening for carcinoid heart disease.	Somatostatin analogues (octreotide, lanreotide)Carcinoid syndrome and carcinoid heart disease	NCCN · Category 2A	81
All comers	After PRRT failure	Everolimus or cabozantinib; alpha PRRT in trials (ACTION-1, AlphaMedix); PRRT retreatment in selected patients.	Actinium-225 DOTATATEACTION-1212Pb-DOTAMTATEBeta PRRT → alpha PRRT	NCCN · Trials preferred	38
All comers	VHL-associated pancreatic NET	Belzutifan (approved 2021) for non-metastatic tumours not requiring immediate surgery.	BelzutifanMEN1 and hereditary neuroendocrine syndromes	NCCN · Category 2A	87
HR-positive	Neuroendocrine carcinoma (poorly differentiated)	Platinum-etoposide (as in SCLC) ± PD-L1 inhibitor by extrapolation; FOLFIRINOX or CAPTEM in later lines; DLL3-directed agents in trials.	CarboplatinTarlatamabAtezolizumab	NCCN · Category 2A	85

Sequence and caution pairings

SequenceApproved

Beta PRRT → alpha PRRT

When lutetium radioligand therapy stops working, alpha-emitting versions can still control the disease.

SequenceApproved

Beta radioligand → alpha radioligand

After lutetium therapy stops working, an actinium version of the same drug can still produce responses.

Regimens in the library

CAPTEM 177Lu-DOTATATE (PRRT)Everolimus Cisplatin + etoposide (EP)Sunitinib 4/2

Lines and subgroups are parsed from the setting text of each standard-of-care row and can misclassify an unusual phrasing; the row’s own setting is always shown. Guideline chips reflect the NCCN category and ESMO-MCBS grade recorded on the cancer page, checked on its stated date. Not medical advice.