Lung neuroendocrine tumours (typical and atypical carcinoid)
Lung neuroendocrine tumours, called typical and atypical carcinoids, are slow-growing tumours of the airways that are usually cured by surgery. When they spread, everolimus is the one drug tested in a randomised trial for this site, cabozantinib was approved in 2025, and somatostatin analogues and lutetium radioligand therapy are borrowed from gut tumours.
Overview
Lung neuroendocrine tumours are graded differently from their gut counterparts: the WHO lung classification separates typical carcinoid (fewer than two mitoses per two square millimetres and no necrosis) from atypical carcinoid (two to ten mitoses or foci of necrosis), with Ki-67 used to support the count rather than define it, and places both alongside small-cell and large-cell neuroendocrine carcinoma in a single neuroendocrine group. Most typical carcinoids sit centrally in a main or lobar bronchus and present with cough, wheeze, haemoptysis or recurrent pneumonia behind an obstructed airway; peripheral tumours are found incidentally. A few produce ectopic ACTH and Cushing's syndrome, carcinoid syndrome is uncommon without liver metastases, and diffuse idiopathic pulmonary neuroendocrine cell hyperplasia is a rare precursor that seeds multiple tumourlets. About a twentieth arise in patients with MEN1.
Surgery is the treatment for localised disease and usually the cure: lobectomy or a parenchyma-sparing sleeve resection with systematic nodal dissection, with endobronchial resection reserved for patients who cannot tolerate an operation. Adjuvant therapy has no proven benefit and follow-up is prolonged because atypical carcinoids can recur years later. For advanced disease the evidence is thin. RADIANT-4 (Lancet 2016) is the only randomised trial to include lung tumours in numbers: 302 patients with non-functional lung or gastrointestinal neuroendocrine tumours were randomised to everolimus or placebo and progression-free survival lengthened from 3.9 to 11.0 months, and the FDA approved everolimus for lung neuroendocrine tumours in 2016. The phase 2 LUNA trial (2017) tested pasireotide, everolimus and the combination in lung and thymic tumours and found each active, without a randomised comparison against placebo.
The rest of the sequence is borrowed. Somatostatin analogues are used for somatostatin receptor-positive tumours on the strength of gut trials and the small SPINET study of lanreotide, and lutetium-177 dotatate is given off-label to receptor-positive lung tumours on series data, since NETTER-1 and NETTER-2 enrolled only gastroenteropancreatic disease. CABINET (New England Journal of Medicine 2024) included lung and thymic tumours in its extra-pancreatic cohort, where cabozantinib lengthened progression-free survival from 3.9 to 8.4 months, and a subgroup analysis presented in 2025 showed a large reduction in progression risk in the lung and thymic tumours; cabozantinib's 2025 approval covers them. Temozolomide-based chemotherapy is used for atypical carcinoids that need shrinkage, and platinum-etoposide is reserved for tumours behaving like carcinoma.
State of the art
- Surgery cures most typical carcinoids, and parenchyma-sparing sleeve resection preserves lung function.
- Everolimus is the only drug with randomised evidence specific to lung neuroendocrine tumours, and cabozantinib joined it in 2025 through the CABINET extra-pancreatic cohort.
- The lung classification by mitotic count and necrosis, rather than Ki-67, still governs treatment, and the two systems are being reconciled.
Red cards
From the labels and guidelines behind the standard of care. Your team's thresholds win.- Emergency services nowBleeding or bruising
Bleeding that will not stop, black or bloody stools, or unexplained bruising when platelets are expected to be low.
- Emergency services nowBone pain flare or fracture (radium-223)
Sudden severe bone pain, or back pain with weakness or numbness in the legs (possible spinal cord compression).
- Emergency services nowBowel perforation
Sudden severe abdominal pain, a hard or very tender abdomen, or abdominal pain with vomiting and fever. Boxed warning for gastrointestinal perforation on bevacizumab.
- Emergency services nowFainting or palpitations
Fainting, near-fainting, or an irregular or racing heartbeat; several kinase inhibitors prolong the QT interval and the labels require ECG and electrolyte monitoring.
- Check before combiningFood and drink: Cabozantinib
Tablets: take on an empty stomach (no food 2 hours before or 1 hour after). Avoid grapefruit.
- Check before combiningFood and drink: Everolimus
Avoid grapefruit. Live vaccines are contraindicated.
See all on the product pages:CabozantinibCapecitabine + temozolomide (CAPTEM)EverolimusLutetium-177 dotatate·Printable cards in the navigator
Anatomy and lymph node drainage
- Central airways (squamous, small-cell)
- Periphery (adenocarcinoma)
- Apex (Pancoast)
- Pleura (mesothelioma)
- Thymus (anterior mediastinum)
- Nodes: hilar (N1)
- Nodes: mediastinal (N2)
- Nodes: supraclavicular (N3)
Central tumours arise in the large airways, peripheral ones in the alveoli; both drain to hilar then mediastinal nodes, and the pleural lining is a separate cancer site.
- Central airways (squamous, small-cell)Typical carcinoid of the central bronchus (low mitotic count, no necrosis) · Peripheral lung neuroendocrine tumour (incidental, sometimes multiple) · Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) with tumourlets · MEN1-associated lung neuroendocrine tumour · Thymic neuroendocrine tumour (grouped with lung in trials)
- Periphery (adenocarcinoma)Peripheral lung neuroendocrine tumour (incidental, sometimes multiple)
- Apex (Pancoast)
- Pleura (mesothelioma)
- Thymus (anterior mediastinum)Thymic neuroendocrine tumour (grouped with lung in trials)
- hilar (N1)
- mediastinal (N2)
- supraclavicular (N3)
Same organ: Non-small-cell lung cancer, Lung cancer (all types), Small-cell lung cancer, Mesothelioma, Pleural mesothelioma, Thymoma and thymic carcinoma, Childhood lung and airway tumours (pleuropulmonary blastoma, tracheobronchial tumours), Inflammatory myofibroblastic tumour (IMT), EGFR-mutated non-small-cell lung cancer, ALK-positive non-small-cell lung cancer, KRAS G12C-mutant non-small-cell lung cancer, ROS1-positive non-small-cell lung cancer, MET exon 14 and MET-amplified non-small-cell lung cancer, RET fusion-positive non-small-cell lung cancer, BRAF V600E-mutant non-small-cell lung cancer, HER2-mutant non-small-cell lung cancer, NTRK fusion-positive non-small-cell lung cancer, PD-L1-high non-small-cell lung cancer without a driver mutation, Resectable stage I to III non-small-cell lung cancer, Unresectable stage III non-small-cell lung cancer, Limited-stage small-cell lung cancer, Extensive-stage small-cell lung cancer
A small minority of lung cancers, occurring in younger patients and non-smokers more often than other lung cancers; typical carcinoids are usually cured by surgery, atypical carcinoids recur more often.
Cases by country
No country-level case numbers. This cancer is not mapped to a GLOBOCAN site.
One section per setting: the options named, what each is for, the trials behind them, the recorded trade-offs and the questions to ask.
Bronchoscopy with biopsy for central tumours, CT of the chest and abdomen, somatostatin receptor PET, and pathology graded by mitotic count and necrosis.
Lobectomy or sleeve resection with systematic nodal dissection; endobronchial resection for patients unfit for surgery; no adjuvant therapy.
Octreotide or lanreotide, by extrapolation from gut trials and the SPINET study.
Everolimus (RADIANT-4); cabozantinib (CABINET); lutetium-177 dotatate off-label for receptor-positive tumours; temozolomide-based chemotherapy for atypical carcinoids needing shrinkage.
Somatostatin analogues for carcinoid syndrome; steroidogenesis inhibitors or resection for ectopic ACTH.
Subtypes & biomarkers
top- Typical carcinoid of the central bronchus (low mitotic count, no necrosis)
- Atypical carcinoid (two to ten mitoses or necrosis, higher recurrence)
- Peripheral lung neuroendocrine tumour (incidental, sometimes multiple)
- Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia (DIPNECH) with tumourlets
- Lung carcinoid with ectopic ACTH and Cushing's syndrome
- MEN1-associated lung neuroendocrine tumour
- Thymic neuroendocrine tumour (grouped with lung in trials)
- Mitotic count and necrosis (WHO typical versus atypical)
- Ki-67 index (supportive, not definitional in the lung)
- Somatostatin receptor PET (staging and somatostatin analogue or radioligand eligibility)
- Chromogranin A (monitoring)
- ACTH and cortisol where Cushing's syndrome is suspected
- Germline MEN1 in young or multiple tumours
How often this target appears
- 1972Arrigoni defines atypical carcinoid as a separate entity
- 2015WHO lung classification groups carcinoids with small-cell and large-cell neuroendocrine carcinoma as neuroendocrine tumours
- 2016RADIANT-4: everolimus approved for lung neuroendocrine tumours
- 2017LUNA: pasireotide and everolimus active in lung and thymic tumours
- 2024CABINET published with lung and thymic tumours in the extra-pancreatic cohort
- 2025Cabozantinib approved for previously treated neuroendocrine tumours including lung; ESMO subgroup analysis
Dated changes read from the records linked to this cancer: approvals, regulatory steps, reported trials, guideline versions and milestones. Newest first; no date is inferred.
All 10 changes by month →- 2026-09-17This recordLung neuroendocrine tumours (typical and atypical carcinoid)Facts on this page last checked
When this page itself was last checked or edited.
- 2025MilestoneCabozantinibCabozantinib approved for previously treated neuroendocrine tumours including lung; ESMO subgroup analysis
A milestone in how this cancer is treated.
- 2024Trial resultCABINET (Alliance A021602)CABINET (Alliance A021602) reported
PFS HR 0.
- 2024MilestoneCABINET (Alliance A021602)CABINET published with lung and thymic tumours in the extra-pancreatic cohort
A milestone in how this cancer is treated.
- 2017MilestoneLung neuroendocrine tumours (typical and atypical carcinoid)LUNA: pasireotide and everolimus active in lung and thymic tumours
A milestone in how this cancer is treated.
- 2016MilestoneRADIANT-3 and RADIANT-4RADIANT-4: everolimus approved for lung neuroendocrine tumours
A milestone in how this cancer is treated.
What is in development for Lung neuroendocrine tumours (typical and atypical carcinoid), drawn from the whole corpus: 3 items. Drugs are grouped by the most advanced trial phase they have reached anywhere; approved treatments sit under standard of care. Technologies are the methods being tested for this cancer, trials are the studies recorded here, and ideas are proposals not yet in a trial.
Trials under way · 1
Trials reported · 2
- CABINET (Alliance A021602) · phase 3 · 2024 · positive
- RADIANT-3 and RADIANT-4 · phase 3 · 2011 · positive
Open problems and what is being done
No randomised trial has tested somatostatin analogues or radioligand therapy specifically in lung neuroendocrine tumours.
Lung and gastroenteropancreatic grading systems disagree, so trial eligibility and guideline advice do not map cleanly.
Atypical carcinoids relapse late and there is no proven adjuvant therapy.
Trials
topTrials recruiting now
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
Expert centres
topExpert centres
Seoul · hospital | South Korea | none recorded | 0 | 464 | 3,248 | #22 | |
Chicago, IL · consortium | United States | none recorded | 1 | 42 | 482 | - | |
Shanghai · hospital | China | none recorded | 0 | 2,872 | 31,534 | - | |
Sydney · cancer center | Australia | none recorded | 0 | 2,222 | 33,278 | - | |
Beijing · hospital | China | none recorded | 0 | 1,784 | 18,230 | - | |
Guangzhou · hospital | China | none recorded | 0 | 1,245 | 12,931 | - | |
Tianjin · cancer center | China | none recorded | 0 | 1,219 | 11,455 | - | |
Beijing · hospital | China | none recorded | 0 | 1,154 | 11,808 | - | |
Rotterdam · cancer center | Netherlands | none recorded | 0 | 852 | 12,180 | - | |
| United Kingdom | none recorded | 0 | 693 | 7,168 | - | ||
Goyang · cancer center | South Korea | none recorded | 0 | 573 | 9,401 | - | |
Gothenburg · hospital | Sweden | none recorded | 0 | 553 | 5,211 | - | |
Shanghai · hospital | China | none recorded | 0 | 539 | 6,491 | - | |
Taoyuan · hospital | Taiwan | none recorded | 0 | 528 | 5,174 | - | |
Osaka · cancer center | Japan | none recorded | 0 | 505 | 4,266 | - |
These are the things we can measure; they are not a ranking of quality. Each column is a field on the institution record or a count over what OnCo has linked; a centre that treats many patients with Lung neuroendocrine tumours but is thinly recorded here will look small.
Not known: OnCo holds no case-volume or outcome figures for centres, so none are shown. Where a national audit or registry publishes them, the centre's page links to it. Default order: Newsweek rank, then trials for this cancer, then research output.
Questions to ask
topQuestions to ask your oncologist about Lung neuroendocrine tumours
Newly diagnosed
- What is my exact diagnosis, stage, and grade, and which tests established them?Why: Everything else follows from an accurate stage and subtype.
- Which biomarkers have been tested on my tumour (for example Mitotic count and necrosis, Ki-67 index, Somatostatin receptor PET, Chromogranin A, ACTH and cortisol where Cushing's syndrome is suspected), and what were the results?Why: These results decide eligibility for targeted therapy, immunotherapy, and trials.
- Which subtype is my cancer, and does that change the recommended treatment?Why: Recognised subtypes for this cancer include Typical carcinoid of the central bronchus, Atypical carcinoid, Peripheral lung neuroendocrine tumour.
- Is germline (inherited) genetic testing recommended for me or my family?Why: Inherited variants can change treatment and matter for relatives.
Diagnosis and staging
- For my situation (diagnosis and staging), which of the standard options do you recommend and why?Why: Guideline options include: Bronchoscopy with biopsy for central tumours, CT of the chest and abdomen, somatostatin receptor PET, and pathology graded by mitotic count and necrosis.
- Am I a candidate for Gallium-68 DOTATATE (and Cu-64 DOTATATE), and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
Localised disease
- For my situation (localised disease), which of the standard options do you recommend and why?Why: Guideline options include: Lobectomy or sleeve resection with systematic nodal dissection; endobronchial resection for patients unfit for surgery; no adjuvant therapy.
Advanced, somatostatin receptor-positive, slow tempo
- For my situation (advanced, somatostatin receptor-positive, slow tempo), which of the standard options do you recommend and why?Why: Guideline options include: Octreotide or lanreotide, by extrapolation from gut trials and the SPINET study.
- 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.
- How do the results of CLARINET apply to someone like me?Why: Trial populations differ from individual patients; ask how closely you match.
Advanced, progressive
- For my situation (advanced, progressive), which of the standard options do you recommend and why?Why: Guideline options include: Everolimus (RADIANT-4); cabozantinib (CABINET); lutetium-177 dotatate off-label for receptor-positive tumours; temozolomide-based chemotherapy for atypical carcinoids needing shrinkage.
- Am I a candidate for Everolimus, Cabozantinib, Lutetium-177 dotatate or related drugs, and what side effects should I expect?Why: Knowing the expected toxicities helps you plan work, family, and supportive care.
- How do the results of RADIANT-3 and RADIANT-4 and CABINET (Alliance A021602) apply to someone like me?Why: Trial populations differ from individual patients; ask how closely you match.
Hormone syndromes
- For my situation (hormone syndromes), which of the standard options do you recommend and why?Why: Guideline options include: Somatostatin analogues for carcinoid syndrome; steroidogenesis inhibitors or resection for ectopic ACTH.
- 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.
Any stage
- Are there clinical trials I could join, for example of CABINET (Alliance A021602), Zanzalintinib Versus Everolimus in Participants With Locally Advanced or Metastatic Neuroendocrine Tumors, Peptide receptor radionuclide therapy (PRRT), Somatostatin receptor PET (68Ga/64Cu-DOTATATE)?Why: Trials are how the next standard of care is set; asking early keeps options open.
- 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.
- 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.
- I read that “No randomised trial has tested somatostatin analogues or radioligand therapy specifically in lung neuroendocrine tumours”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
- I read that “Lung and gastroenteropancreatic grading systems disagree, so trial eligibility and guideline advice do not map cleanly”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
Newly diagnosed? Read the first 60 days with Lung neuroendocrine tumours, then print the one-page appointment sheet with room for the answers.
Print this page for your appointment (your browser's print command). These prompts are for discussion; your clinical team knows your case.
Direct links plus the targets, companies, and technologies of this cancer's products.
technologies
10targets
7drugs
6companies
4terms
5trials
4Latest papers
topQuery for this cancer: (TITLE:"Lung neuroendocrine tumours" OR ABSTRACT:"Lung neuroendocrine tumours" OR TITLE:"typical and atypical carcinoid" OR ABSTRACT:"typical and atypical carcinoid" OR TITLE:"Bronchial carcinoid" OR ABSTRACT:"Bronchial carcinoid" OR TITLE:"Pulmonary carcinoid" OR ABSTRACT:"Pulmonary carcinoid" OR TITLE:"Typical carcinoid" OR ABSTRACT:"Typical carcinoid" OR TITLE:"Atypical carcinoid" OR ABSTRACT:"Atypical carcinoid") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Lung neuroendocrine tumours (typical and atypical carcinoid), not a curated reading list.
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