OnCo
cancersCancer

Neuroendocrine tumours

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.

The frontier is alpha-emitting PRRT: 212Pb-DOTAMTATE (AlphaMedix) met all primary endpoints in phase 2 with a 54% response rate in PRRT-naive patients and Breakthrough designation, and 225Ac-DOTATATE (RYZ101) is in the phase 3 ACTION-1 trial after lutetium failure. SSTR antagonist ligands, dosimetry-personalised dosing, and combinations with CAPTEM or immunotherapy are being tested. Open problems include the lack of randomised evidence for sequencing, the absence of effective therapy for SSTR-negative and high-grade disease, a 2-3% risk of therapy-related leukaemia after PRRT, and isotope supply.

State of the art today

  • Theranostic paradigm; first-line PRRT in higher-grade disease.
  • Theranostic paradigm is routine: SSTR PET selects, 177Lu-DOTATATE treats, including first line for grade 2-3 disease.
  • First head-to-head radioligand-versus-drug trial (COMPETE) won on PFS; FDA decision on 177Lu-edotreotide due 28 August 2026.
  • Cabozantinib approved (2025) across pancreatic and extra-pancreatic NETs after prior therapy, with an 81% reduction in progression risk in lung/thymic NETs.
  • Alpha PRRT (212Pb-DOTAMTATE) met all phase 2 endpoints with Breakthrough designation; 225Ac-DOTATATE in phase 3.
  • Germline testing and syndrome-directed care (belzutifan for VHL) are standard for pancreatic NETs.
Who it affects

About 7 per 100,000 people per year in the US, rising six-fold since the 1970s; prevalence is high because many patients live for years (>170,000 living with NETs in the US).

Where the cases are

Cases by country · GLOBOCAN 2022
All countries →

No country-level case numbers. Neuroendocrine tumours are reported under their organ of origin (pancreas, lung, small intestine) and cannot be separated.

Standard of care

11top
Localised

Resection.

NCCN Guidelines: Neuroendocrine and Adrenal Tumors
Advanced

SSA → 177Lu-DOTATATE → everolimus/cabozantinib/chemotherapy; alpha therapy in trials.

NCCN Guidelines: Neuroendocrine and Adrenal Tumors
Lutetium-177 dotatateActinium-225 DOTATATE
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
Somatostatin receptor PET (68Ga/64Cu-DOTATATE)Neuroendocrine tumour grade (Ki-67) and WHO classificationChromogranin AGermline (hereditary) testingMEN1 and hereditary neuroendocrine syndromes
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
Robotic & minimally invasive surgery
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…
Somatostatin analogues (octreotide, lanreotide)PROMIDCLARINETLutetium-177 dotatatePeptide receptor radionuclide therapy (PRRT)
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 se…
Lutetium-177 dotatate177Lu-edotreotideCOMPETEEverolimusRADIANT-3 and RADIANT-4SunitinibCabozantinibCABINET (Alliance A021602)
Advanced pancreatic NET needing tumour shrinkage

CAPTEM (E2211); PRRT; liver-directed therapy for hepatic-dominant disease.

NCCN · Category 2A
Capecitabine + temozolomide (CAPTEM)Transarterial chemoembolisation (TACE)Radioembolisation (TARE / SIRT, yttrium-90)Thermal ablation (RFA, microwave, cryo)
Carcinoid syndrome

SSA dose escalation; telotristat ethyl for refractory diarrhoea; octreotide infusion peri-procedurally; echocardiographic screening for carcinoid heart disease.

NCCN · Category 2A
Somatostatin analogues (octreotide, lanreotide)Carcinoid syndrome and carcinoid heart disease
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.

NCCN · Category 2A
CarboplatinTarlatamabAtezolizumab
After PRRT failure

Everolimus or cabozantinib; alpha PRRT in trials (ACTION-1, AlphaMedix); PRRT retreatment in selected patients.

NCCN · Trials preferred
Actinium-225 DOTATATEACTION-1212Pb-DOTAMTATEBeta PRRT → alpha PRRT
VHL-associated pancreatic NET

Belzutifan (approved 2021) for non-metastatic tumours not requiring immediate surgery.

NCCN · Category 2A
BelzutifanMEN1 and hereditary neuroendocrine syndromes

Subtypes & biomarkers

top
Subtypes
  • Small-bowel (midgut) NET, often with carcinoid syndrome
  • Pancreatic NET (functioning: insulinoma, gastrinoma, glucagonoma; non-functioning)
  • Lung NET (typical and atypical carcinoid)
  • Rectal and appendiceal NET (often incidental, excellent prognosis)
  • Grade 3 well-differentiated NET
  • Neuroendocrine carcinoma (small- and large-cell), treated like SCLC
  • Hereditary : MEN1, VHL, NF1, TSC; paraganglioma/phaeochromocytoma (SDHx)
Biomarkers clinicians test

Target prevalence in this cancer

Target / alterationPrevalenceSource
Somatostatin receptor 2
Lower in grade 3
80-90%
Wikipedia

How common each drug target or alteration is in this cancer. Population-level and approximate; see the target page for detail. Full matrix.

History

17top
  1. 1907Oberndorfer coins 'Karzinoid' for small-bowel tumours
  2. 1954Carcinoid syndrome described (Thorson)
    Carcinoid syndrome and carcinoid heart disease
  3. 1987Octreotide approved
  4. 1988Octreotide approved for carcinoid syndrome
    Somatostatin analogues (octreotide, lanreotide)
  5. 1994111In-octreotide scintigraphy (OctreoScan) approved

    First SSTR imaging; later replaced by PET.

    Somatostatin receptor PET (68Ga/64Cu-DOTATATE)
  6. 2000First 90Y- and 177Lu-DOTATOC/DOTATATE PRRT series (Rotterdam, Basel)
    Peptide receptor radionuclide therapy (PRRT)
  7. 2009PROMID: octreotide slows tumour growth
    PROMID
  8. 2011Everolimus (RADIANT-3) and sunitinib approved for pancreatic NETs
    RADIANT-3 and RADIANT-4EverolimusSunitinib
  9. 2014CLARINET: lanreotide antiproliferative approval
    CLARINET
  10. 201668Ga-DOTATATE PET (Netspot) approved; RADIANT-4 extends everolimus to lung/GI NETs
    Somatostatin receptor PET (68Ga/64Cu-DOTATATE)RADIANT-3 and RADIANT-4
  11. 2018Lutathera approved
    Lutetium-177 dotatate
  12. 2018Lutathera approved (NETTER-1): PRRT enters standard care
    Lutetium-177 dotatatePeptide receptor radionuclide therapy (PRRT)
  13. 2020SANET trials positive in China (surufatinib)
    SANET-ep and SANET-p
  14. 2024NETTER-2: first-line PRRT
  15. 2024NETTER-2: PRRT first line in grade 2-3; CABINET published; AlphaMedix Breakthrough designation
    Lutetium-177 dotatateCABINET (Alliance A021602)212Pb-DOTAMTATE
  16. 2025Cabozantinib approved (March); COMPETE positive (ENETS, Lancet); AlphaMedix phase 2 meets all endpoints (October)
    CabozantinibCOMPETEALPHAMEDIX-02
  17. 2026FDA accepts 177Lu-edotreotide NDA (PDUFA 28 August); ACTION-1 dosimetry published; pancreatic subgroup of COMPETE at ENETS
    177Lu-edotreotideACTION-1

Pipeline

14top
Phase 3Targeted alpha therapy
Actinium-225 DOTATATE
Phase 3Radioligand therapy (beta)
177Lu-edotreotide
Phase 2Targeted alpha therapy (lead-212)
212Pb-DOTAMTATE · AlphaMedix
ApprovedSmall-molecule multi-kinase inhibitor (MET, VEGFR2, AXL, RET)
Cabozantinib · Cabometyx
ApprovedBispecific T-cell engager (DLL3×CD3)
Tarlatamab · Imdelltra
COMPETEALPHAMEDIX-02ACTION-1Peptide receptor radionuclide therapy (PRRT)Somatostatin receptor PET (68Ga/64Cu-DOTATATE)Dosimetry-personalised PRRT instead of four fixed cyclesSSTR antagonist radioligands to increase tumour doseBeta PRRT → alpha PRRTDLL3

Open problems

  • Neuroendocrine carcinoma (high grade) behaves like SCLC.
  • Sequencing of PRRT vs targeted therapy.
  • Sequencing is unproven: no randomised trial orders SSA, PRRT, everolimus, cabozantinib and chemotherapy.
  • SSTR-negative, FDG-avid and high-grade disease has few options; neuroendocrine carcinoma outcomes remain poor.
  • Therapy-related MDS/AML (~2-3%) and renal toxicity after PRRT; long-term data on retreatment are thin.
  • Overall survival benefits are hard to demonstrate because patients live for years and cross over.
  • Isotope supply (177Lu, 212Pb, 225Ac) and nuclear-medicine capacity limit access outside major centres.
  • Chromogranin A is an unreliable marker; better blood tests (NETest, ctDNA) are not validated for decisions.
  • Rare syndromic and paediatric NETs lack trials; hereditary carriers need lifelong surveillance protocols.

Trials

top

Recruiting now (live from ClinicalTrials.gov)

Recruiting trials near you · live from ClinicalTrials.gov
Neuroendocrine tumours
condition: neuroendocrine tumor
Open on ClinicalTrials.gov →

Country and place are remembered in this browser only. A postcode is sent to OpenStreetMap's Nominatim service to find coordinates when you press the button; nothing else leaves your device.

Landmark trials in OnCo

ACTION-1ALPHAMEDIX-02CABINET (Alliance A021602)CLARINETCOMPETEPROMIDRADIANT-3 and RADIANT-4SANET-ep and SANET-p

Expert centres

top
Where the expertise is

Centres linked to this cancer in OnCo

Seeking a second opinion: ask your oncologist for a referral to a high-volume centre; most accept records and pathology by mail or telehealth. In the US, use the NCI's Find a Cancer Center tool or the nonprofit Cancer Commons, which navigates options for advanced cancers at no cost.

Questions to ask

top
Bring to your appointment

Questions to ask your oncologist about Neuroendocrine tumours

Generated from this cancer's standard of care, biomarkers, and pipeline · 31 questions

Newly diagnosed

  1. What is my exact diagnosis, stage, and grade, and which tests established them?
    Why: Everything else follows from an accurate stage and subtype.
  2. Which biomarkers have been tested on my tumour (for example Ki-67 grade, SSTR PET uptake, Chromogranin A, MEN1, DAXX/ATRX, Ki-67 index and mitotic count), and what were the results?
    Why: These results decide eligibility for targeted therapy, immunotherapy, and trials.
  3. Which subtype is my cancer, and does that change the recommended treatment?
    Why: Recognised subtypes for this cancer include Small-bowelNET, often with carcinoid syndrome, Pancreatic NET, Lung NET.
  4. Is germline (inherited) genetic testing recommended for me or my family?
    Why: Inherited variants can change treatment and matter for relatives.

Localised

  1. For my situation (localised), which of the standard options do you recommend and why?
    Why: Guideline options include: Resection.

Advanced

  1. For my situation (advanced), which of the standard options do you recommend and why?
    Why: Guideline options include: SSA → 177Lu-DOTATATE → everolimus/cabozantinib/chemotherapy; alpha therapy in trials.
  2. Am I a candidate for Lutetium-177 dotatate, Actinium-225 DOTATATE, and what side effects should I expect?
    Why: Knowing the expected toxicities helps you plan work, family, and supportive care.

Diagnosis and staging

  1. For my situation (diagnosis and staging), which of the standard options do you recommend and why?
    Why: Guideline options include: 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.

Localised disease

  1. For my situation (localised disease), which of the standard options do you recommend and why?
    Why: Guideline options include: Surgical resection (including primary tumour resection with liver metastases where feasible); endoscopic resection for small rectal/gastric NETs; surveillance for small incidental lesions.

Advanced, grade 1-2, SSTR-positive, first line

  1. For my situation (advanced, grade 1-2, sstr-positive, first line), which of the standard options do you recommend and why?
    Why: Guideline options include: Somatostatin analogue (octreotide LAR or lanreotide); 177Lu-DOTATATE first line for grade 2-3 (NETTER-2) with high burden.
  2. Am I a candidate for Somatostatin analogues (octreotide, lanreotide), Lutetium-177 dotatate, and what side effects should I expect?
    Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
  3. How do the results of PROMID and CLARINET apply to someone like me?
    Why: Trial populations differ from individual patients; ask how closely you match.

Advanced, progression on SSA

  1. For my situation (advanced, progression on ssa), which of the standard options do you recommend and why?
    Why: Guideline options include: PRRT with 177Lu-DOTATATE (or 177Lu-edotreotide if approved); everolimus; sunitinib (pancreatic); cabozantinib (CABINET, all sites).
  2. Am I a candidate for Lutetium-177 dotatate, 177Lu-edotreotide, Everolimus or related drugs, and what side effects should I expect?
    Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
  3. How do the results of COMPETE and RADIANT-3 and RADIANT-4 apply to someone like me?
    Why: Trial populations differ from individual patients; ask how closely you match.

Advanced pancreatic NET needing tumour shrinkage

  1. For my situation (advanced pancreatic net needing tumour shrinkage), which of the standard options do you recommend and why?
    Why: Guideline options include: CAPTEM (E2211); PRRT; liver-directed therapy for hepatic-dominant disease.
  2. Am I a candidate for Capecitabine + temozolomide (CAPTEM), and what side effects should I expect?
    Why: Knowing the expected toxicities helps you plan work, family, and supportive care.

Carcinoid syndrome

  1. For my situation (carcinoid syndrome), which of the standard options do you recommend and why?
    Why: Guideline options include: SSA dose escalation; telotristat ethyl for refractory diarrhoea; octreotide infusion peri-procedurally; echocardiographic screening for carcinoid heart disease.
  2. Am I a candidate for Somatostatin analogues (octreotide, lanreotide), and what side effects should I expect?
    Why: Knowing the expected toxicities helps you plan work, family, and supportive care.

Neuroendocrine carcinoma (poorly differentiated)

  1. For my situation (neuroendocrine carcinoma (poorly differentiated)), which of the standard options do you recommend and why?
    Why: Guideline options include: Platinum-etoposide (as in SCLC) ± PD-L1 inhibitor by extrapolation; FOLFIRINOX or CAPTEM in later lines; DLL3-directed agents in trials.
  2. Am I a candidate for Carboplatin, Tarlatamab, Atezolizumab, and what side effects should I expect?
    Why: Knowing the expected toxicities helps you plan work, family, and supportive care.

After PRRT failure

  1. For my situation (after prrt failure), which of the standard options do you recommend and why?
    Why: Guideline options include: Everolimus or cabozantinib; alpha PRRT in trials (ACTION-1, AlphaMedix); PRRT retreatment in selected patients.
  2. Am I a candidate for Actinium-225 DOTATATE, 212Pb-DOTAMTATE, and what side effects should I expect?
    Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
  3. How do the results of ACTION-1 apply to someone like me?
    Why: Trial populations differ from individual patients; ask how closely you match.

VHL-associated pancreatic NET

  1. For my situation (vhl-associated pancreatic net), which of the standard options do you recommend and why?
    Why: Guideline options include: Belzutifan (approved 2021) for non-metastatic tumours not requiring immediate surgery.
  2. Am I a candidate for Belzutifan, and what side effects should I expect?
    Why: Knowing the expected toxicities helps you plan work, family, and supportive care.

Any stage

  1. Are there clinical trials I could join, for example of Actinium-225 DOTATATE, 177Lu-edotreotide, COMPETE, 212Pb-DOTAMTATE?
    Why: Trials are how the next standard of care is set; asking early keeps options open.
  2. Would a second opinion at a high-volume centre change anything, and can you help arrange it?
    Why: Rare or high-stakes decisions benefit from a centre that treats many similar patients.
  3. What supportive care (symptom control, nutrition, exercise, mental health, financial help) is available from the start?
    Why: Supportive care improves quality of life and helps patients complete treatment.
  4. I read that “Neuroendocrine carcinoma (high grade) behaves like SCLC”. How does that affect my plan?
    Why: Open problems are where trials and second opinions matter most.
  5. I read that “Sequencing of PRRT vs targeted therapy”. How does that affect my plan?
    Why: Open problems are where trials and second opinions matter most.

Print this page for your appointment (your browser's print command). These prompts are for discussion; your clinical team knows your case.

Everything relevant

131top

Direct links plus the targets, companies, and technologies of this cancer's products.

technologies

24
Anti-angiogenic therapyAutologous stem cell transplant (high-dose therapy)Cytotoxic chemotherapyGermline (hereditary) testingHistopathology & immunohistochemistryImmune checkpoint inhibitorsMIBG imaging and 131I-MIBG therapyMRD / molecular residual disease testingPeptide receptor radionuclide therapy (PRRT)Percutaneous hepatic perfusion (chemosaturation)PET (positron emission tomography)PET/CTPlatinum agentsRadioembolisation (TARE / SIRT, yttrium-90)Radioligand plus DNA-repair inhibitor combinationsRadioligand therapy (beta emitters)Robotic & minimally invasive surgerySmall-molecule kinase inhibitorsSomatostatin receptor PET (68Ga/64Cu-DOTATATE)T-cell engagers (bispecific)Targeted alpha therapyThermal ablation (RFA, microwave, cryo)Topoisomerase-I inhibitors (and ADC payloads)Transarterial chemoembolisation (TACE)

targets

13
AKTCD3DLL3HIF-2αKITKMT2A (MLL) rearrangementMeninMETPD-L1PIK3CA / PI3K-alphaRETSomatostatin receptor 2VEGF / VEGFR

drugs

15
Phase 3Radioligand therapy (beta)
177Lu-edotreotide
Phase 2Targeted alpha therapy (lead-212)
212Pb-DOTAMTATE · AlphaMedix
Phase 3Targeted alpha therapy
Actinium-225 DOTATATE
ApprovedMonoclonal antibody (anti-PD-L1)
Atezolizumab · Tecentriq / Tecentriq Hybreza (SC)
ApprovedSmall-molecule HIF-2α inhibitor
Belzutifan · Welireg
ApprovedSmall-molecule multi-kinase inhibitor (MET, VEGFR2, AXL, RET)
Cabozantinib · Cabometyx
EstablishedOral cytotoxic regimen
Capecitabine + temozolomide (CAPTEM)
ApprovedCytotoxic chemotherapy (platinum)
Carboplatin
Not mapped hereTopoisomerase II inhibitor (podophyllotoxin derivative)
Etoposide · VePesid / Etopophos / Toposar
ApprovedSmall-molecule mTOR inhibitor
Everolimus · Afinitor
Not mapped herePET radiotracer (somatostatin receptor ligand)
Gallium-68 DOTATATE (and Cu-64 DOTATATE) · Netspot / Detectnet
ApprovedRadioligand therapy (beta)
Lutetium-177 dotatate · Lutathera
ApprovedPeptide hormone analogue (SSTR2 agonist)
Somatostatin analogues (octreotide, lanreotide) · Sandostatin LAR, Somatuline Depot
ApprovedSmall-molecule multi-kinase inhibitor (VEGFR, PDGFR, KIT)
Sunitinib · Sutent
ApprovedBispecific T-cell engager (DLL3×CD3)
Tarlatamab · Imdelltra

companies

15
AmgenBristol Myers SquibbCuriumExelixisIpsenITM Isotope Technologies MunichMerck & Co. (MSD)NovartisOrano MedPerspective TherapeuticsPfizer (incl. Seagen)RadioMedixRayzeBio (BMS)Roche / GenentechSanofi

institutions

21
Alliance for Clinical Trials in OncologyAustralasian Gastro-Intestinal Trials GroupChulabhorn Hospital / Chulabhorn Royal AcademyECOG-ACRIN Cancer Research GroupErasmus MC Cancer InstituteHeidelberg University Hospital / NCT / DKFZHolden Comprehensive Cancer Center, University of IowaInselspital, Bern University Hospital / University Cancer Center InselspitalIRCCS Istituto Romagnolo per lo Studio dei Tumori 'Dino Amadori' (IRST)IRCCS Ospedale San RaffaeleMemorial Sloan Kettering Cancer CenterPeking Union Medical College HospitalPostgraduate Institute of Medical Education and Research, ChandigarhRutgers Cancer Institute of New JerseyThe Royal MarsdenTUM Klinikum rechts der Isar / CCC MünchenUniversitätsklinikum Hamburg-EppendorfUniversity Hospital Basel / Tumour CentreUniversity Hospital Düsseldorf / CIO DüsseldorfUppsala University Hospital / Uppsala UniversityWest German Cancer Center (WTZ), University Hospital Essen

terms

14
Alpha vs beta emittersCarcinoid syndrome and carcinoid heart diseaseChromogranin ADosimetryLiver-directed therapy (TACE, TARE, HAI, ablation)MEN1 and hereditary neuroendocrine syndromesNeuroendocrine tumour grade (Ki-67) and WHO classificationPRRT (peptide receptor radionuclide therapy)TACE (transarterial chemoembolisation)TARE / SIRT (radioembolisation with yttrium-90)TheranosticsTumour differentiation (well / moderately / poorly differentiated)Tumour markers (CEA, LDH, chromogranin, thyroglobulin)Von Hippel-Lindau disease

trials

8
ACTION-1ALPHAMEDIX-02CABINET (Alliance A021602)CLARINETCOMPETEPROMIDRADIANT-3 and RADIANT-4SANET-ep and SANET-p

pairings

3
Beta PRRT → alpha PRRTBeta radioligand → alpha radioligandSSTR PET → PRRT

roadmaps

1
Radiopharmaceutical roadmap: iodine → lutetium → actinium

ideas

11
A funded expert second opinion for every new high-stakes or rare cancer diagnosisA same-week expert second opinion for every rare cancer diagnosisAn open model bank for the rare tumours nobody has models forDosimetry-personalised PRRT instead of four fixed cyclesMandatory national virtual tumour boards for rare and complex cancersOne global rare cancer network with n-of-1 and Bayesian trial frameworksOne standing umbrella trial for all rare cancers in a countryRandomise inside the registry that already follows every patientSSTR antagonist radioligands to increase tumour doseThe pathology lab triggers a trial referral the day a rare cancer is diagnosedTrial-in-a-box: a preconfigured protocol kit any hospital can open for a rare cancer

people

2
Emily K. BergslandSteve Jobs

bottlenecks

3
Manufacturing cost and time for living and radioactive medicinesRare and paediatric cancers without marketsWrong doses

key papers

1
NETTER-2: lutetium-177 dotatate as first treatment for higher-grade gastroenteropancreatic neuroendocrine tumours

Key papers

1top
rctThe Lancet 2024changed practice
NETTER-2: lutetium-177 dotatate as first treatment for higher-grade gastroenteropancreatic neuroendocrine tumours

Patients newly diagnosed with an advanced grade 2 or 3 neuroendocrine tumour of the gut or pancreas that shows somatostatin receptors on imaging can now receive lutetium dotatate as their first treatment, gaining more than a year of additional disease control and a much higher chance of tumour shrinkage. It does not settle whether radioligand therapy is better than other first-line options such as capecitabine-temozolomide or everolimus, and long-term marrow safety with earlier use needs surveillance.

Latest papers

top
Literature trend169 papers in the last 12 months+19% vs prior 12How this is computed
Latest papers · live from Europe PMC
Open in Europe PMC

Query for this cancer: (TITLE:"Neuroendocrine tumours" OR ABSTRACT:"Neuroendocrine tumours") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Neuroendocrine tumours, not a curated reading list.

Connected

113top

Pages like this

not linked directly; found by shared links

technologies

16
Germline (hereditary) testingHistopathology & immunohistochemistryMIBG imaging and 131I-MIBG therapyPeptide receptor radionuclide therapy (PRRT)Percutaneous hepatic perfusion (chemosaturation)PET (positron emission tomography)PET/CTRadioembolisation (TARE / SIRT, yttrium-90)Radioligand plus DNA-repair inhibitor combinationsRadioligand therapy (beta emitters)Robotic & minimally invasive surgerySmall-molecule kinase inhibitorsSomatostatin receptor PET (68Ga/64Cu-DOTATATE)Targeted alpha therapyThermal ablation (RFA, microwave, cryo)Transarterial chemoembolisation (TACE)

targets

7
AKTDLL3HIF-2αMeninMETSomatostatin receptor 2VEGF / VEGFR

drugs

15
Phase 3Radioligand therapy (beta)
177Lu-edotreotide
Phase 2Targeted alpha therapy (lead-212)
212Pb-DOTAMTATE · AlphaMedix
Phase 3Targeted alpha therapy
Actinium-225 DOTATATE
ApprovedMonoclonal antibody (anti-PD-L1)
Atezolizumab · Tecentriq / Tecentriq Hybreza (SC)
ApprovedSmall-molecule HIF-2α inhibitor
Belzutifan · Welireg
ApprovedSmall-molecule multi-kinase inhibitor (MET, VEGFR2, AXL, RET)
Cabozantinib · Cabometyx
EstablishedOral cytotoxic regimen
Capecitabine + temozolomide (CAPTEM)
ApprovedCytotoxic chemotherapy (platinum)
Carboplatin
Not mapped hereTopoisomerase II inhibitor (podophyllotoxin derivative)
Etoposide · VePesid / Etopophos / Toposar
ApprovedSmall-molecule mTOR inhibitor
Everolimus · Afinitor
Not mapped herePET radiotracer (somatostatin receptor ligand)
Gallium-68 DOTATATE (and Cu-64 DOTATATE) · Netspot / Detectnet
ApprovedRadioligand therapy (beta)
Lutetium-177 dotatate · Lutathera
ApprovedPeptide hormone analogue (SSTR2 agonist)
Somatostatin analogues (octreotide, lanreotide) · Sandostatin LAR, Somatuline Depot
ApprovedSmall-molecule multi-kinase inhibitor (VEGFR, PDGFR, KIT)
Sunitinib · Sutent
ApprovedBispecific T-cell engager (DLL3×CD3)
Tarlatamab · Imdelltra

companies

11
Bristol Myers SquibbExelixisIpsenITM Isotope Technologies MunichNovartisOrano MedPerspective TherapeuticsPfizer (incl. Seagen)RadioMedixRayzeBio (BMS)Sanofi

institutions

21
Alliance for Clinical Trials in OncologyAustralasian Gastro-Intestinal Trials GroupChulabhorn Hospital / Chulabhorn Royal AcademyECOG-ACRIN Cancer Research GroupErasmus MC Cancer InstituteHeidelberg University Hospital / NCT / DKFZHolden Comprehensive Cancer Center, University of IowaInselspital, Bern University Hospital / University Cancer Center InselspitalIRCCS Istituto Romagnolo per lo Studio dei Tumori 'Dino Amadori' (IRST)IRCCS Ospedale San RaffaeleMemorial Sloan Kettering Cancer CenterPeking Union Medical College HospitalPostgraduate Institute of Medical Education and Research, ChandigarhRutgers Cancer Institute of New JerseyThe Royal MarsdenTUM Klinikum rechts der Isar / CCC MünchenUniversitätsklinikum Hamburg-EppendorfUniversity Hospital Basel / Tumour CentreUniversity Hospital Düsseldorf / CIO DüsseldorfUppsala University Hospital / Uppsala UniversityWest German Cancer Center (WTZ), University Hospital Essen

terms

14
Alpha vs beta emittersCarcinoid syndrome and carcinoid heart diseaseChromogranin ADosimetryLiver-directed therapy (TACE, TARE, HAI, ablation)MEN1 and hereditary neuroendocrine syndromesNeuroendocrine tumour grade (Ki-67) and WHO classificationPRRT (peptide receptor radionuclide therapy)TACE (transarterial chemoembolisation)TARE / SIRT (radioembolisation with yttrium-90)TheranosticsTumour differentiation (well / moderately / poorly differentiated)Tumour markers (CEA, LDH, chromogranin, thyroglobulin)Von Hippel-Lindau disease

trials

8
ACTION-1ALPHAMEDIX-02CABINET (Alliance A021602)CLARINETCOMPETEPROMIDRADIANT-3 and RADIANT-4SANET-ep and SANET-p

pairings

3
Beta PRRT → alpha PRRTBeta radioligand → alpha radioligandSSTR PET → PRRT

roadmaps

1
Radiopharmaceutical roadmap: iodine → lutetium → actinium

ideas

11
A funded expert second opinion for every new high-stakes or rare cancer diagnosisA same-week expert second opinion for every rare cancer diagnosisAn open model bank for the rare tumours nobody has models forDosimetry-personalised PRRT instead of four fixed cyclesMandatory national virtual tumour boards for rare and complex cancersOne global rare cancer network with n-of-1 and Bayesian trial frameworksOne standing umbrella trial for all rare cancers in a countryRandomise inside the registry that already follows every patientSSTR antagonist radioligands to increase tumour doseThe pathology lab triggers a trial referral the day a rare cancer is diagnosedTrial-in-a-box: a preconfigured protocol kit any hospital can open for a rare cancer

people

2
Emily K. BergslandSteve Jobs

bottlenecks

3
Manufacturing cost and time for living and radioactive medicinesRare and paediatric cancers without marketsWrong doses

key papers

1
NETTER-2: lutetium-177 dotatate as first treatment for higher-grade gastroenteropancreatic neuroendocrine tumours