Rare lung and chest tumours are not one disease. The bronchopulmonary neuroendocrine spectrum — typical carcinoid, atypical carcinoid, large cell neuroendocrine carcinoma — sits alongside the salivary-type airway tumours adenoid cystic carcinoma and mucoepidermoid carcinoma, the pre-neoplastic DIPNECH, and the rare pleural mesenchymal tumour solitary fibrous tumour of the pleura. Each has its own behaviour, staging, and operation. Mr Lawrence Okiror evaluates and operates on these tumours at London Bridge Hospital and The Lister Chelsea, with NHS practice at Guy's and St Thomas' and integrated review through the King's College Hospital ENETS Neuroendocrine MDT where complex disease calls for it. Private appointments within 2–3 working days. Self-referrals welcome.
Last reviewed: May 2026 · Mr Lawrence Okiror FRCS(CTh) FRCSEd(CTh) · GMC 6150382
What these tumours share is rarity. What they require differs — airway resection for one, lung-sparing surgery for another, pleural-based resection for the third.
Locally advanced and recurrent neuroendocrine disease is discussed at the King's College Hospital ENETS NET MDT. That route is built in — not an afterthought.
For sleeve airway resections, for negative-margin pleural resection, and for parenchyma-sparing carcinoid surgery, the operation determines the long-term outcome.
The rare end of the thoracic tumour spectrum is heterogeneous. A typical carcinoid in the upper lobe of a 35-year-old, an adenoid cystic carcinoma filling the trachea of a 50-year-old, and a giant solitary fibrous tumour pressing on the heart of a 70-year-old have one feature in common: each is uncommon enough that the average thoracic surgical practice may see only a handful in a year. Beyond that, almost nothing about them is the same. Their cells of origin differ. Their imaging looks different. Their staging tests differ. The right operation differs. The follow-up differs.
This page is organised around that heterogeneity. The neuroendocrine spectrum — typical and atypical carcinoid, large cell neuroendocrine carcinoma, and at the far end small cell lung cancer — sits in one cluster because the tumours share a cell of origin and a graded biology. The salivary-type airway tumours — adenoid cystic and mucoepidermoid carcinoma — sit in a second cluster because they share an anatomical home in the central airway. Solitary fibrous tumour sits separately because it arises from the pleural mesothelium, not from the lung. DIPNECH sits alongside the carcinoid section because the two are biologically linked.
For each, the sections below describe what the tumour is, how it presents, what the staging looks like, and what operation Mr Okiror offers at London Bridge Hospital and The Lister Chelsea, with the King's NET MDT route built in where the tumour calls for it.
The bronchopulmonary neuroendocrine tumours arise from the neuroendocrine cells of the airway lining. The current IASLC and WHO classification (5th edition, 2021) groups them into four entities along a single biological gradient — from the most indolent to the most aggressive: typical carcinoid, atypical carcinoid, large cell neuroendocrine carcinoma (LCNEC), and small cell lung cancer (SCLC).
The first two are well-differentiated neuroendocrine tumours (NETs) and behave like indolent malignancies; the last two are poorly-differentiated neuroendocrine carcinomas (NECs) and behave like high-grade cancers. The line between the first three is surgically meaningful: Mr Okiror operates on typical carcinoid, atypical carcinoid, and LCNEC where the disease is resectable. Small cell lung cancer is almost always treated by chemoradiotherapy rather than surgery, with very rare exceptions for early T1N0 disease.
The distinction between typical and atypical carcinoid is made on pathology, not imaging. It rests on mitotic count and the presence or absence of necrosis: less than 2 mitoses per 2 mm² and no necrosis defines typical carcinoid; 2–10 mitoses per 2 mm² or focal necrosis defines atypical carcinoid. Beyond ten mitoses, the diagnosis moves into LCNEC or SCLC.
The pathology grade determines the operation, the adjuvant treatment, and the follow-up. A specialist pathology review at a high-volume centre matters — misclassification between AC and LCNEC, or between AC and SCLC, changes the entire treatment plan.
Lung carcinoids account for around 2% of all lung tumours. The annual incidence is approximately 0.7 per 100,000. Up to half of patients are diagnosed incidentally on chest CT or chest X-ray performed for an unrelated reason — a screening scan, a pre-operative work-up, or imaging for an unrelated cough. The remainder present with one of the bronchial-obstruction symptoms: a persistent cough, intermittent haemoptysis, wheeze, recurrent pneumonia distal to a partially blocked airway, or, in central tumours, breathlessness on exertion. Carcinoid syndrome — flushing, diarrhoea, and right-sided heart valve disease — is rare in lung carcinoid, occurring in 2–12% of cases and almost always in the presence of liver metastases.
Roughly 60–84% of lung carcinoids are centrally located in the main or lobar bronchi and can be seen at bronchoscopy as a vascular polypoid mass within the airway. Peripheral carcinoids are less common but more often atypical. Staging is built on three scans: a contrast CT of the chest, abdomen, and pelvis; a DOTATATE PET-CT (more sensitive than standard FDG PET for somatostatin-receptor-positive tumours); and bronchoscopy with biopsy where the tumour is accessible. DOTATATE PET-CT is available at both Guy's and St Thomas' and London Bridge Hospital — access on both NHS and private sides of the practice.
The aim is complete resection while preserving as much functional lung as possible. For a small peripheral tumour, robotic segmentectomy or wedge resection may suffice. For a central tumour, sleeve resection of the bronchus with end-to-end anastomosis preserves the distal lung and avoids pneumonectomy. Systematic lymph node sampling is performed in every case. Wide resection margins are not required for carcinoid in the way they are required for lung cancer — the operation is anatomical, not radical.
Atypical carcinoid behaves more aggressively. The standard operation is at least a lobectomy with systematic mediastinal lymph node dissection — sublobar resection is generally not adequate. Where the tumour is large or invasive, sleeve lobectomy, bilobectomy, or in rare cases pneumonectomy may be required. The pathology of every resected carcinoid is reviewed for mitotic count and necrosis; an unexpected upgrade from typical to atypical at final pathology may prompt MDT discussion about completion lymphadenectomy or surveillance strategy.
There is no evidence to support adjuvant chemotherapy after complete resection of either typical or atypical carcinoid. Adjuvant radiotherapy is reserved for positive or uncertain margins and is discussed at the multidisciplinary team. For patients with borderline lung function before the operation, regional functional mapping with VQ SPECT-CT is used to inform what can be safely removed — particularly relevant for carcinoid because the operation may be central and sleeve-based rather than a standard lobectomy.
Long-term follow-up is essential. Carcinoid can recur many years — sometimes more than a decade — after a seemingly complete operation. Surveillance is built on serial low-dose CT, with DOTATATE PET-CT reserved for equivocal radiology, and is more intensive for atypical carcinoid, for node-positive disease, and for patients with multifocal disease or background DIPNECH.
Complex neuroendocrine disease benefits from multidisciplinary expertise that no single specialty can deliver alone. King's College Hospital is a European Neuroendocrine Tumour Society (ENETS) Centre of Excellence — one of a small number in the UK — and serves as the south-east England specialist NET service. The unit has held Centre of Excellence status since 2012 and operates a weekly Thursday-afternoon multidisciplinary team meeting attended by endocrinologists, medical oncologists, nuclear medicine specialists, specialist NET surgeons, radiologists, pathologists, and specialist NET clinical nurses.
For straightforward resectable typical carcinoid, the operation itself remains at Guy's and St Thomas' under Mr Okiror's care. For the cases where additional expertise changes the answer, the King's NET MDT is the route.
When King's MDT is requested
What King's adds
Nuclear medicine expertise in DOTATATE-PET interpretation and in peptide receptor radionuclide therapy. Medical oncology expertise specific to neuroendocrine biology, including selection of patients for somatostatin analogue therapy, temozolomide-based chemotherapy, and PRRT. Specialist endocrinology for the rare functioning tumours. Specialist NET nursing for patient navigation through what can be a long treatment journey. A dedicated carcinoid heart disease service for the small number of patients in whom the syndrome has caused valve disease.
Large cell neuroendocrine carcinoma (LCNEC) is a high-grade neuroendocrine carcinoma that behaves clinically and biologically more like non-small-cell lung cancer than like the lower-grade carcinoids. For resectable disease, the operation follows the principles of NSCLC surgery: lobectomy or larger anatomical resection, with systematic mediastinal lymph node dissection. Adjuvant chemotherapy is usually considered post-operatively, often using platinum-based regimens, with decisions made at the lung cancer MDT. The five-year survival after complete resection of localised LCNEC is significantly lower than for carcinoid — the biology is high-grade.
Small cell lung cancer (SCLC) sits at the far end of the neuroendocrine spectrum. The standard of care for SCLC is chemoradiotherapy — surgery has a role only in the small minority of patients presenting with truly early-stage T1–T2 N0 disease, where a complete anatomical resection (typically lobectomy with mediastinal lymph node dissection) followed by adjuvant chemotherapy can be offered to selected fit patients. Most patients with SCLC, including most with apparent stage I disease on imaging, have occult nodal or distant spread on PET-CT, brain MRI, or invasive mediastinal staging — which is why thorough staging is essential before any surgical decision.
For both LCNEC and SCLC, integration with the lung cancer multidisciplinary team is the route. See Lung Cancer Surgery in 2026 for the broader staging-to-surgery framework.
DIPNECH — diffuse idiopathic pulmonary neuroendocrine cell hyperplasia — is a proliferation of the lung's neuroendocrine cells that remains confined to the airways. It is most commonly seen in middle-aged women, and is often mistaken for asthma for years before the correct diagnosis is made. Symptoms include a chronic cough, breathlessness on exertion, and wheeze.
The diagnosis is suspected on a high-resolution CT showing multiple small lung nodules and a mosaic pattern of air trapping. Confirmation requires histology, usually from a surgical biopsy of one of the larger lesions. The histological hallmarks are neuroendocrine cell hyperplasia confined to the bronchial mucosa (DIPNECH), tumourlets (lesions under 5 mm) where the cells have extended through the basement membrane, and full carcinoid tumours (lesions over 5 mm) where overt neoplastic change is present.
Approximately half of patients with DIPNECH remain clinically and radiologically stable for years. The other half progress slowly, with development of new tumourlets or carcinoids over time. Surgical resection is reserved for the largest lesions where they are felt to be at risk of becoming or already are carcinoid — not for the diffuse background. King's NET MDT discussion guides the surveillance strategy.
DIPNECH is not a diagnosis of cancer. It is a diagnosis of being at risk of developing carcinoid in some patients — an indication for organised surveillance, not for empirical surgery.
Adenoid cystic carcinoma (ACC) of the trachea and bronchi is a rare salivary-gland-type malignancy. It accounts for around 30–40% of primary tracheal cancers but for only 0.1–0.2% of all lung and bronchus cancers. The tumour arises from the submucosal bronchial glands and is biologically distinct from common lung cancers — smoking is not a risk factor. ACC is what surgeons describe as "slowly progressive": the tumour is rarely an emergency at presentation, but it requires a precisely planned operation because of how it spreads.
Two features define the surgical challenge. First, submucosal extension: ACC creeps along the inside surface of the airway, often well beyond what is visible at bronchoscopy. Second, perineural invasion: the tumour follows nerve sheaths and can extend along them invisibly. Together these mean that an apparently localised tumour may have wider involvement than the imaging shows. Adequate surgical margins are essential, and intra-operative frozen section assessment of the airway resection edges is part of the standard approach.
Symptoms relate to airway narrowing — persistent cough, wheeze, breathlessness, recurrent chest infections, and sometimes haemoptysis. Many patients are treated for asthma for months before the correct diagnosis is made. The diagnostic test is rigid or flexible bronchoscopy with biopsy. Staging requires a CT thorax with contrast and, increasingly, MRI for the assessment of mediastinal soft tissue involvement.
Reported five-year survival for surgically treated ACC is in the range of 71–79%, with ten-year survival around 51–57%. Tracheal ACC carries a slightly better prognosis than lung-based ACC. Adjuvant radiotherapy is often added where the resection margin is positive or uncertain.
The most important point about ACC is late recurrence. Recurrence can occur ten or more years after the original operation, and the pattern is often local rather than systemic. Long-term radiological and bronchoscopic surveillance is essential — many series report that the patients lost to recurrence are those who lapsed from follow-up at the five-year mark.
For more on airway-focused surgical and interventional procedures, see Central Airway Interventions.
Mucoepidermoid carcinoma (MEC) is a salivary-gland-type tumour that arises from the bronchial mucous glands. It accounts for less than 1% of all lung cancers. The WHO classification separates MEC into low-grade and high-grade forms, and the distinction matters enormously: low-grade MEC is one of the most curable malignancies in the chest, while high-grade MEC behaves much like non-small-cell lung cancer.
Like ACC, MEC most commonly arises in the central airways — the main, lobar, or segmental bronchi — rather than in peripheral lung tissue. The typical presentation is symptoms of airway obstruction: persistent cough, wheeze, post-obstructive infection, or breathlessness. At bronchoscopy, low-grade MEC often appears as a smooth polypoid intrabronchial mass. The pathology can sometimes be mistaken for adenosquamous carcinoma; specialist pathology review is part of the work-up.
Surgery is the mainstay of treatment. For low-grade MEC, sleeve lobectomy or other lung-sparing resection achieves complete removal in the great majority of cases, with excellent long-term outcomes. The aim is parenchyma preservation — a sleeve resection often allows what would otherwise have been a pneumonectomy to be avoided. For high-grade MEC, the operation follows lung cancer principles: anatomical resection with systematic lymph node dissection, with adjuvant therapy considered post-operatively at the lung cancer MDT.
The patient with a low-grade MEC sitting in a main bronchus is usually a candidate for parenchyma-sparing sleeve resection, not pneumonectomy. The conversation matters because the lifelong functional outcome depends on which operation is chosen.
Solitary fibrous tumour of the pleura (SFTP) is a rare mesenchymal tumour arising from the mesothelial layer of the pleura — the smooth lining around the lung. It is distinct from the neuroendocrine and salivary-type tumours discussed above and from lung cancer itself. The cell of origin is connective tissue, not lung tissue. SFT can also arise outside the chest, but the pleural form is the most common thoracic presentation.
A defining feature of SFTP is that it can grow remarkably large before being detected. Reported diameters in the literature range from 1 cm to over 40 cm. Most are pedunculated (attached by a stalk to the visceral pleura) and can be benign-behaving even when very large. Roughly 80% are benign on histology; 20% are malignant. Five-year survival for benign SFTP is approximately 89%; for malignant SFTP it is approximately 45%. Recurrence can occur years after the original operation — in some series 10% or more.
Many SFTPs are found incidentally on imaging for an unrelated reason. When they do cause symptoms, the picture is cough, breathlessness on exertion from displacement of the lung, and sometimes chest discomfort. Two paraneoplastic syndromes deserve mention because they are characteristic and reversible after resection: Doege-Potter syndrome (paraneoplastic hypoglycaemia from tumour secretion of insulin-like growth factor), and digital clubbing.
The diagnostic gold standard at the pathology level is the NAB2-STAT6 fusion gene, present in essentially all SFTs and now identifiable on routine immunohistochemistry by STAT6 nuclear staining. This marker has effectively closed the diagnostic uncertainty that surrounded SFT in earlier decades.
Surgery is the standard treatment. The aim is complete resection with negative margins. The operation chosen depends on size, attachment, and anatomy:
A small subset of resected SFTs are classified as malignant or high-risk on histology and risk-stratification scoring. For these, discussion at the sarcoma multidisciplinary team is appropriate. Adjuvant therapy is not standard but is considered in selected cases.
Long-term radiological surveillance with periodic CT is the rule for all resected SFT, even when the histology is benign — recurrence can occur years later, and the paraneoplastic syndromes (where present pre-operatively) typically resolve after surgery and reappear at recurrence.
For more on pleural disease and the broader range of pleural surgical conditions, see Pleural Disease.
Rare tumours suffer disproportionately from limited surgical experience. A unit that performs hundreds of lung cancer resections a year may see only a handful of carcinoids, one or two MECs, and a single SFT. The operation that is right for a common cancer may not be the right operation for a rare one — a pneumonectomy where a sleeve resection would have served, a wedge excision where formal lobectomy was indicated, an extended resection for a low-grade tumour that needed only complete removal.
A specialist second opinion is reasonable whenever the diagnosis is rare and the recommendation is for a major or extended operation. Mr Okiror reviews imaging personally at the first consultation, with the pathology report and, where helpful, the histology slides themselves. Where the question is one of multidisciplinary routing rather than surgery alone, the King's NET MDT is the route for neuroendocrine disease; sarcoma MDT for borderline or malignant SFT.
If you have a rare tumour diagnosis and want an independent surgeon's view of the imaging, the pathology, and the operation being recommended, most patients are seen within 2–3 working days.
Book a Consultation →Or call Jo Mitchelson:
020 7952 2882
Bringing your imaging and pathology to the consultation allows the case to be reviewed directly rather than summarised from the written report. Appointments at London Bridge Hospital and The Lister Chelsea within 2–3 working days. Self-referrals welcome.
Jo Mitchelson, PA · 020 7952 2882 · pa@lungsurgeon.co.uk
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DOTATATE PET, CT, MRI — the imaging that defines what these tumours are and where they sit.
Central Airway InterventionsBronchoscopy, airway stenting, sleeve resection — for ACC, MEC, and central carcinoid.
Lung Cancer Surgery in 2026The broader staging-to-surgery framework that LCNEC and high-grade rare tumours plug into.
Borderline Lung FunctionVQ SPECT-CT regional function mapping — relevant when carcinoid surgery is central and sleeve-based.
Pleural DiseaseThe broader pleural surgical context for solitary fibrous tumour and related pleural-based conditions.
Specialist Second OpinionIndependent review of imaging, pathology, and surgical recommendation within 2–3 working days.