Pituitary tumours (pituitary neuroendocrine tumours) and pituitary carcinoma
Pituitary tumours are usually benign growths of the hormone gland at the base of the brain that cause trouble by overproducing hormones or pressing on the optic nerves. Prolactin-producing tumours melt away with a tablet, most others are cured by surgery through the nose, and the rare aggressive ones respond to the chemotherapy drug temozolomide.
Overview
Pituitary neuroendocrine tumours (PitNETs, the WHO 2022 term for pituitary adenomas) are classified by cell lineage using transcription factors (PIT1, TPIT, SF1) and hormone expression: lactotroph (prolactinoma), somatotroph (acromegaly), corticotroph (Cushing disease), gonadotroph (usually non-functioning) and rarer types. Most are sporadic; germline AIP, MEN1, CDKN1B and other mutations account for young-onset and familial cases. Aggressive PitNETs invade the cavernous sinus and recur despite surgery and radiotherapy; pituitary carcinoma is defined by cerebrospinal or systemic metastasis and is very rare. Because the NCI lists pituitary tumours among cancer types and their management sits between endocrinology, neurosurgery and oncology, they belong in a complete corpus.
Treatment is lineage-specific. Prolactinomas respond to dopamine agonists (cabergoline) in most cases, with surgery reserved for resistance or intolerance. Other functioning and symptomatic non-functioning tumours are treated by transsphenoidal endoscopic surgery; acromegaly not cured by surgery is controlled with first-generation somatostatin analogues (octreotide, lanreotide), pasireotide or pegvisomant; Cushing disease with surgery, then steroidogenesis inhibitors (osilodrostat, metyrapone) or pasireotide. Radiotherapy, increasingly stereotactic radiosurgery, controls residual or recurrent tumour. For aggressive tumours and carcinomas the European Society of Endocrinology guideline (2018) recommends temozolomide as first-line chemotherapy, with response predicted in part by low MGMT expression; checkpoint inhibitors have produced responses in corticotroph carcinomas in case series.
Open problems are defining aggressiveness before it declares itself, treatment after temozolomide failure (PRRT for SSTR-positive tumours, immunotherapy, bevacizumab), and the long-term endocrine and visual sequelae of treatment.
State of the art today
- Transcription-factor-based classification (WHO 2022) has replaced hormone staining alone and identifies high-risk lineages such as silent corticotroph and plurihormonal PIT1 tumours before they behave aggressively.
- Endoscopic transsphenoidal surgery with intraoperative MRI or navigation has improved remission and reduced complications.
- Temozolomide, borrowed from glioma, became the first effective chemotherapy for aggressive pituitary tumours and is guideline first line.
- Radiosurgery offers durable control of residual tumour with less hypopituitarism than conventional fractionated radiotherapy in small targets.
Where it starts and where it drains
Gliomas infiltrate along white matter and can cross the corpus callosum, medulloblastoma sits in the cerebellum, and CNS lymphoma favours deep periventricular tissue; none spread through lymph nodes.
- Frontal lobe (glioblastoma commonest)
- Temporal lobe
- Corpus callosum (butterfly glioma)
- Lower-grade IDH-mutant glioma
- Cerebellum (medulloblastoma)
- Brainstem (diffuse midline glioma)
- Ventricles and ependymal lining (ependymoma)
- Sella and pituitary (pituitary tumours, craniopharyngioma)Lactotroph tumour (prolactinoma) · Somatotroph tumour (acromegaly) · Corticotroph tumour (Cushing disease, silent corticotroph) · Gonadotroph tumour (most non-functioning adenomas) · Thyrotroph tumour · Plurihormonal PIT1-lineage tumour (aggressive) · Pituitary carcinoma (metastatic)
- Deep periventricular tissue (CNS lymphoma)
No conventional lymphatics: gliomas spread along white matter tracts and, rarely, through cerebrospinal fluid; medulloblastoma can seed the spine.
Same organ: Glioma & glioblastoma, Primary CNS lymphoma, Medulloblastoma, Paediatric low-grade glioma, Diffuse midline glioma, H3 K27-altered (including DIPG), Atypical teratoid/rhabdoid tumour (ATRT), Ependymoma, Craniopharyngioma
Pituitary tumours are among the most common intracranial tumours, found in about one in a thousand people clinically and far more often incidentally; true pituitary carcinoma with metastasis is extremely rare.
- AI in radiologyEstablished
- cfDNA fragmentomicsEstablished
- Colorectal cancer screening (colonoscopy, FIT, stool DNA, blood)Standard of care
- DNA methylation profilingEstablished
- HCC surveillance in cirrhosis (ultrasound + AFP)Standard of care
- High-risk pancreatic surveillance (CAPS / PRECEDE)Established
- A 28-day national pathway for people with a positive multi-cancer blood test
- A breath test to rule out cancer in people with vague symptoms
- A cancer blood test for older people arriving at A&E with unexplained symptoms
- A legislated, publicly reported 28-day standard from urgent referral to diagnosis
- A live national dashboard of stage at diagnosis as the scorecard for early detection
- A single 'cancer check at 60' appointment bundling all screening tests
Background: Alpha-fetoprotein (AFP), Barrett's oesophagus, CA 19-9, Early detection, Faecal immunochemical test (FIT). Also on OnCo: Symptoms and red flags · Early detection roadmap.
Where the cases are
No country-level case numbers. This cancer is not mapped to a GLOBOCAN site.
Cabergoline first line, titrated to normal prolactin and tumour shrinkage; surgery for resistance, intolerance or pituitary apoplexy.
Endoscopic transsphenoidal resection; medical therapy for persistent disease (somatostatin analogues, pasireotide, pegvisomant for acromegaly; osilodrostat, metyrapone for Cushing); radiotherapy or radiosurgery for residual tumour.
Temozolomide (standard schedule, at least 3 cycles before assessing response), with radiotherapy where not previously given; PRRT for SSTR-positive tumours, bevacizumab or checkpoint inhibitors in trials or case series after temozolomide failure.
Subtypes & biomarkers
top- Lactotroph tumour (prolactinoma)
- Somatotroph tumour (acromegaly)
- Corticotroph tumour (Cushing disease, silent corticotroph)
- Gonadotroph tumour (most non-functioning adenomas)
- Thyrotroph tumour
- Plurihormonal PIT1-lineage tumour (aggressive)
- Pituitary carcinoma (metastatic)
- Serum prolactin, IGF-1, cortisol and ACTH, TSH
- Transcription factor lineage (PIT1, TPIT, SF1) and hormone immunohistochemistry (WHO 2022)
- Ki-67 and mitotic count
- MGMT expression (temozolomide response)
- Cavernous sinus invasion (Knosp grade) on MRI
- Germline AIP and MEN1 in young or familial cases
Target prevalence in this cancer
- 1886Pierre Marie describes acromegaly
- 1909Cushing performs transsphenoidal pituitary surgery
- 1932Cushing describes pituitary basophilism (Cushing disease)
- 1985Bromocriptine and later cabergoline establish medical therapy of prolactinoma
- 2006Temozolomide responses in aggressive pituitary tumours reported
- 2018ESE guideline for aggressive pituitary tumours and carcinomas
Temozolomide as first-line chemotherapy.
- 2022WHO renames pituitary adenoma as PitNET
Lineage-based classification.
Open problems, and what is being done about each
Predicting aggressive behaviour at diagnosis: lineage, Ki-67 and molecular markers are being combined into risk scores.
Salvage after temozolomide: PRRT, immunotherapy and bevacizumab have only case-series evidence.
Hypopituitarism and visual loss from tumour and treatment: radiosurgery and surgical technique aim to reduce them.
Incidentalomas: how to avoid overtreatment of the many small tumours found on imaging.
Trials
topRecruiting now (live from ClinicalTrials.gov)
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Expert centres
topCentres linked to this cancer in OnCo
- via SBRT / SABR (stereotactic radiotherapy)
- Aarhus University HospitalAarhus, DKvia SBRT / SABR (stereotactic radiotherapy), IMRT / IGRT (modern external beam)
- American Society for Radiation OncologyArlington, VA, USvia SBRT / SABR (stereotactic radiotherapy), IMRT / IGRT (modern external beam)
- Centre Oscar LambretLille, FRvia SBRT / SABR (stereotactic radiotherapy), IMRT / IGRT (modern external beam)
- Comprehensive Cancer Center Freiburg (CCCF)Freiburg im Breisgau, DEvia SBRT / SABR (stereotactic radiotherapy), IMRT / IGRT (modern external beam)
- European Society for Radiotherapy and OncologyBrussels, BEvia SBRT / SABR (stereotactic radiotherapy), IMRT / IGRT (modern external beam)
- Hacettepe University Cancer InstituteAnkara, TRvia SBRT / SABR (stereotactic radiotherapy), IMRT / IGRT (modern external beam)
- Hokkaido University HospitalSapporo, JPvia SBRT / SABR (stereotactic radiotherapy), IMRT / IGRT (modern external beam)
- IRCCS Humanitas Research HospitalRozzano (Milan), ITvia SBRT / SABR (stereotactic radiotherapy), IMRT / IGRT (modern external beam)
- Rajiv Gandhi Cancer Institute and Research CentreNew Delhi, INvia SBRT / SABR (stereotactic radiotherapy), IMRT / IGRT (modern external beam)
- via SBRT / SABR (stereotactic radiotherapy), IMRT / IGRT (modern external beam)
- TROG Cancer ResearchNewcastle, NSW, AUvia SBRT / SABR (stereotactic radiotherapy), IMRT / IGRT (modern external beam)
- UMC Utrecht Cancer CenterUtrecht, NLvia SBRT / SABR (stereotactic radiotherapy), IMRT / IGRT (modern external beam)
- Velindre Cancer CentreCardiff, GBvia SBRT / SABR (stereotactic radiotherapy), IMRT / IGRT (modern external beam)
- Apollo Hospitals (Apollo Cancer Centres)Chennai, INvia SBRT / SABR (stereotactic radiotherapy)
- via SBRT / SABR (stereotactic radiotherapy)
- via Endocrine therapy (SERMs, AIs, SERDs)
- via Endocrine therapy (SERMs, AIs, SERDs)
- via Endocrine therapy (SERMs, AIs, SERDs)
- Breast Cancer TrialsNewcastle, NSW, AUvia Endocrine therapy (SERMs, AIs, SERDs)
- Canadian Cancer Trials Group (CCTG)Kingston, ON, CAvia SBRT / SABR (stereotactic radiotherapy)
- Centre Antoine LacassagneNice, FRvia IMRT / IGRT (modern external beam)
- via SBRT / SABR (stereotactic radiotherapy)
- via IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- via Lutetium-177 dotatate
- via Endocrine therapy (SERMs, AIs, SERDs)
- Dan L Duncan Comprehensive Cancer Center, Baylor College of MedicineHouston, TX, USNCI comprehensivevia Endocrine therapy (SERMs, AIs, SERDs)
- Edinburgh Cancer Centre / CRUK Scotland CentreEdinburgh, GBvia Endocrine therapy (SERMs, AIs, SERDs)
- Erasmus MC Cancer InstituteRotterdam, NLvia Lutetium-177 dotatate
- ETOP IBCSG Partners FoundationBern, CHvia Endocrine therapy (SERMs, AIs, SERDs)
- European Association of Nuclear MedicineVienna, ATvia Lutetium-177 dotatate
- Fundación Arturo López PérezSantiago, CLvia SBRT / SABR (stereotactic radiotherapy)
- GEICAM Spanish Breast Cancer GroupMadrid, ESvia Endocrine therapy (SERMs, AIs, SERDs)
- Geneva University Hospitals (HUG)Geneva, CHvia IMRT / IGRT (modern external beam)
- via Endocrine therapy (SERMs, AIs, SERDs)
- Groote Schuur Hospital / University of Cape TownCape Town, ZAvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Hadassah Medical CenterJerusalem, ILvia SBRT / SABR (stereotactic radiotherapy)
- via IMRT / IGRT (modern external beam)
- via SBRT / SABR (stereotactic radiotherapy)
- HealthCare Global EnterprisesBengaluru, INvia SBRT / SABR (stereotactic radiotherapy)
- Ho Chi Minh City Oncology HospitalHo Chi Minh City, VNvia IMRT / IGRT (modern external beam)
- via Lutetium-177 dotatate
- Hospital Universitari i Politècnic La FeValencia, ESvia SBRT / SABR (stereotactic radiotherapy)
- Hunan Cancer HospitalChangsha, CNvia IMRT / IGRT (modern external beam)
- via Lutetium-177 dotatate
- Institut BergoniéBordeaux, FRvia IMRT / IGRT (modern external beam)
- Institut Jules BordetBrussels, BEvia Endocrine therapy (SERMs, AIs, SERDs)
- Institut National d'Oncologie, RabatRabat, MAvia IMRT / IGRT (modern external beam)
- Institut Salah AzaïezTunis, TNvia IMRT / IGRT (modern external beam)
- Institute of Oncology LjubljanaLjubljana, SIvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- International Extranodal Lymphoma Study GroupBellinzona, CHvia IMRT / IGRT (modern external beam)
- via Lutetium-177 dotatate
- Istanbul University Institute of OncologyIstanbul, TRvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- Juravinski Cancer Centre / Escarpment Cancer Research InstituteHamilton, ON, CAvia IMRT / IGRT (modern external beam)
- Kenyatta National HospitalNairobi, KEvia IMRT / IGRT (modern external beam)
- Koo Foundation Sun Yat-Sen Cancer CenterTaipei, TWvia SBRT / SABR (stereotactic radiotherapy)
- Korle Bu Teaching HospitalAccra, GHvia IMRT / IGRT (modern external beam)
- Lagos University Teaching HospitalLagos, NGvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- National Institute of Oncology, HungaryBudapest, HUvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- NSABP FoundationPittsburgh, PA, USvia Endocrine therapy (SERMs, AIs, SERDs)
- Ocean Road Cancer InstituteDar es Salaam, TZvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- via Lutetium-177 dotatate
- Rambam Health Care CampusHaifa, ILvia IMRT / IGRT (modern external beam)
- Rigshospitalet – Copenhagen University HospitalCopenhagen, DKvia IMRT / IGRT (modern external beam)
- Robert H. Lurie Comprehensive Cancer Center of Northwestern UniversityChicago, IL, USNCI comprehensivevia Temozolomide
- Royal Adelaide HospitalAdelaide, AUvia IMRT / IGRT (modern external beam)
- Siriraj Hospital, Mahidol UniversityBangkok, THvia IMRT / IGRT (modern external beam)
- Society of Nuclear Medicine and Molecular ImagingReston, VA, USvia Lutetium-177 dotatate
- Sunnybrook Odette Cancer CentreToronto, ON, CAvia SBRT / SABR (stereotactic radiotherapy)
- Tata Medical Center, KolkataKolkata, INvia IMRT / IGRT (modern external beam)
- Tawam HospitalAl Ain, AEvia IMRT / IGRT (modern external beam)
- Tel Aviv Sourasky Medical CenterTel Aviv, ILvia IMRT / IGRT (modern external beam)
- via IMRT / IGRT (modern external beam)
- via Lutetium-177 dotatate
- University of Malaya Medical CentreKuala Lumpur, MYvia IMRT / IGRT (modern external beam)
- University of Maryland Marlene and Stewart Greenebaum Comprehensive Cancer CenterBaltimore, MD, USNCI comprehensivevia SBRT / SABR (stereotactic radiotherapy)
- via IMRT / IGRT (modern external beam)
- via Lutetium-177 dotatate
- via SBRT / SABR (stereotactic radiotherapy)
- via IMRT / IGRT (modern external beam)
- via Lutetium-177 dotatate
- Zhejiang Cancer HospitalHangzhou, CNvia IMRT / IGRT (modern external beam)
Questions to ask
topQuestions to ask your oncologist about Pituitary tumours (pituitary neuroendocrine tumours) and pituitary carcinoma
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 Serum prolactin, IGF-1, cortisol and ACTH, TSH, Transcription factor lineageand hormone immunohistochemistry, Ki-67 and mitotic count, MGMT expression, Cavernous sinus invasionon MRI), 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 Lactotroph tumour, Somatotroph tumour, Corticotroph tumour.
- Is germline (inherited) genetic testing recommended for me or my family?Why: Inherited variants can change treatment and matter for relatives.
Prolactinoma
- For my situation (prolactinoma), which of the standard options do you recommend and why?Why: Guideline options include: Cabergoline first line, titrated to normal prolactin and tumour shrinkage; surgery for resistance, intolerance or pituitary apoplexy.
Acromegaly, Cushing disease, symptomatic non-functioning tumours
- For my situation (acromegaly, cushing disease, symptomatic non-functioning tumours), which of the standard options do you recommend and why?Why: Guideline options include: Endoscopic transsphenoidal resection; medical therapy for persistent disease (somatostatin analogues, pasireotide, pegvisomant for acromegaly; osilodrostat, metyrapone for Cushing); radiotherapy or radiosurgery for residual tumour.
- 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.
Aggressive PitNET or pituitary carcinoma
- For my situation (aggressive pitnet or pituitary carcinoma), which of the standard options do you recommend and why?Why: Guideline options include: Temozolomide (standard schedule, at least 3 cycles before assessing response), with radiotherapy where not previously given; PRRT for SSTR-positive tumours, bevacizumab or checkpoint inhibitors in trials or case series after temozolomide failure.
- Am I a candidate for Temozolomide, Lutetium-177 dotatate, Bevacizumab, 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 Temozolomide, Peptide receptor radionuclide therapy (PRRT), SBRT / SABR (stereotactic radiotherapy)?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 “Predicting aggressive behaviour at diagnosis: lineage, Ki-67 and molecular markers are being combined into risk scores”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
- I read that “Salvage after temozolomide: PRRT, immunotherapy and bevacizumab have only case-series evidence”. How does that affect my plan?Why: Open problems are where trials and second opinions matter most.
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Direct links plus the targets, companies, and technologies of this cancer's products.
technologies
9targets
2drugs
4companies
4pathways
1terms
2bottlenecks
2Latest papers
topQuery for this cancer: (TITLE:"Pituitary tumours pituitary neuroendocrine tumours and pituitary carcinoma" OR ABSTRACT:"Pituitary tumours pituitary neuroendocrine tumours and pituitary carcinoma" OR TITLE:"Pituitary adenoma" OR ABSTRACT:"Pituitary adenoma" OR TITLE:"PitNET" OR ABSTRACT:"PitNET" OR TITLE:"Prolactinoma" OR ABSTRACT:"Prolactinoma" OR TITLE:"Acromegaly" OR ABSTRACT:"Acromegaly" OR TITLE:"Cushing disease" OR ABSTRACT:"Cushing disease") AND (treatment OR therapy OR trial OR survival OR diagnosis). Results are unfiltered search hits about Pituitary tumours (pituitary neuroendocrine tumours) and pituitary carcinoma, not a curated reading list.
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