{"entity":{"id":"pituitary-tumours","kind":"cancer","name":"Pituitary tumours (pituitary neuroendocrine tumours) and pituitary carcinoma","aka":["Pituitary adenoma","PitNET","Prolactinoma","Acromegaly","Cushing disease","Pituitary carcinoma"],"tldr":"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.","summary":"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.\n\nTreatment 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.\n\nOpen 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.","asOf":"2026-09-10","wikipedia":"https://en.wikipedia.org/wiki/Pituitary_adenoma","links":[{"label":"NCI PDQ: pituitary tumours","url":"https://www.cancer.gov/types/pituitary"},{"label":"ESE guideline: aggressive pituitary tumours and carcinomas (2018)","url":"https://doi.org/10.1530/EJE-17-0796"},{"label":"Pituitary Society prolactinoma consensus (2023)","url":"https://doi.org/10.1038/s41574-023-00886-5"}],"tags":["nci-coverage","rare","endocrine","cns"],"related":["craniopharyngioma","meningioma","vestibular-schwannoma","cns-germ-cell-tumours"],"cancers":[],"sections":[],"technologies":["endocrine-therapy","sbrt","imrt-igrt","prrt","mri","cytotoxic-chemotherapy"],"targets":["sstr2"],"drugs":["octreotide-lanreotide","temozolomide","lutathera","bevacizumab","bromocriptine","cabergoline","pasireotide","pegvisomant","osilodrostat","metyrapone"],"companies":[],"institutions":[],"pathways":["pi3k-akt-mtor"],"terms":["mgmt","rare-cancers"],"trials":[],"people":[],"bottlenecks":["b-overdiagnosis","b-rare-cancers"],"keyPapers":["paper-petersenn-nat-rev-endocrinol","paper-raverot-eur-j-endocrinol"],"journals":[],"dependsOn":[],"notes":[],"group":"endocrine","burden":"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.","subtypes":["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)"],"biomarkers":["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"],"standardOfCare":[{"setting":"Prolactinoma","approach":"Cabergoline first line, titrated to normal prolactin and tumour shrinkage; surgery for resistance, intolerance or pituitary apoplexy.","refs":["endocrine-therapy"],"guideline":{"version":"Pituitary Society international consensus on prolactinoma 2023","url":"https://doi.org/10.1038/s41574-023-00886-5"}},{"setting":"Acromegaly, Cushing disease, symptomatic non-functioning tumours","approach":"Endoscopic transsphenoidal resection; medical therapy for persistent disease (somatostatin analogues, pasireotide, pegvisomant for acromegaly; osilodrostat, metyrapone for Cushing); radiotherapy or radiosurgery for residual tumour.","refs":["octreotide-lanreotide","sbrt","imrt-igrt"],"guideline":{"version":"Endocrine Society guidelines: acromegaly (2014), Cushing (2015)","url":"https://doi.org/10.1210/jc.2014-2700"}},{"setting":"Aggressive PitNET or pituitary carcinoma","approach":"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.","refs":["temozolomide","prrt","lutathera","bevacizumab"],"guideline":{"version":"European Society of Endocrinology guideline 2018","url":"https://doi.org/10.1530/EJE-17-0796"}}],"stateOfArt":["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."],"history":[{"year":1886,"title":"Pierre Marie describes acromegaly","refs":[]},{"year":1909,"title":"Cushing performs transsphenoidal pituitary surgery","refs":[]},{"year":1932,"title":"Cushing describes pituitary basophilism (Cushing disease)","refs":[]},{"year":1985,"title":"Bromocriptine and later cabergoline establish medical therapy of prolactinoma","refs":[]},{"year":2006,"title":"Temozolomide responses in aggressive pituitary tumours reported","refs":["temozolomide"]},{"year":2018,"title":"ESE guideline for aggressive pituitary tumours and carcinomas","note":"Temozolomide as first-line chemotherapy.","refs":["temozolomide"]},{"year":2022,"title":"WHO renames pituitary adenoma as PitNET","note":"Lineage-based classification.","refs":[]}],"pipeline":["temozolomide","prrt","sbrt"],"openProblems":["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."],"parent":"brain-tumours"},"route":"/cancers/pituitary-tumours/","neighbours":{"cancer":[{"id":"brain-tumours","kind":"cancer","name":"Brain and spinal cord tumours (all types)","route":"/cancers/brain-tumours/"},{"id":"cns-germ-cell-tumours","kind":"cancer","name":"Central nervous system germ cell tumours (germinoma and non-germinomatous)","route":"/cancers/cns-germ-cell-tumours/"},{"id":"corticotroph-pitnet","kind":"cancer","name":"Corticotroph pituitary neuroendocrine tumour (Cushing disease and silent corticotroph tumour)","route":"/cancers/corticotroph-pitnet/"},{"id":"craniopharyngioma","kind":"cancer","name":"Craniopharyngioma","route":"/cancers/craniopharyngioma/"},{"id":"gonadotroph-pitnet","kind":"cancer","name":"Gonadotroph pituitary neuroendocrine tumour (non-functioning adenoma)","route":"/cancers/gonadotroph-pitnet/"},{"id":"lactotroph-pitnet","kind":"cancer","name":"Lactotroph pituitary neuroendocrine tumour (prolactinoma)","route":"/cancers/lactotroph-pitnet/"},{"id":"meningioma","kind":"cancer","name":"Meningioma","route":"/cancers/meningioma/"},{"id":"multiple-endocrine-neoplasia","kind":"cancer","name":"Multiple endocrine neoplasia syndromes (MEN1, MEN2, MEN4)","route":"/cancers/multiple-endocrine-neoplasia/"},{"id":"men1-syndrome","kind":"cancer","name":"Multiple endocrine neoplasia type 1 (MEN1)","route":"/cancers/men1-syndrome/"},{"id":"somatotroph-pitnet","kind":"cancer","name":"Somatotroph pituitary neuroendocrine tumour (acromegaly)","route":"/cancers/somatotroph-pitnet/"},{"id":"thyrotroph-pitnet","kind":"cancer","name":"Thyrotroph pituitary neuroendocrine tumour (TSH-secreting)","route":"/cancers/thyrotroph-pitnet/"},{"id":"vestibular-schwannoma","kind":"cancer","name":"Vestibular schwannoma (acoustic neuroma)","route":"/cancers/vestibular-schwannoma/"}],"technology":[{"id":"cytotoxic-chemotherapy","kind":"technology","name":"Cytotoxic chemotherapy","route":"/technologies/cytotoxic-chemotherapy/"},{"id":"endocrine-therapy","kind":"technology","name":"Endocrine therapy (SERMs, AIs, SERDs)","route":"/technologies/endocrine-therapy/"},{"id":"gamma-knife","kind":"technology","name":"Gamma Knife","route":"/technologies/gamma-knife/"},{"id":"imrt-igrt","kind":"technology","name":"IMRT / IGRT (modern external beam)","route":"/technologies/imrt-igrt/"},{"id":"intraoperative-mri-ct","kind":"technology","name":"Intraoperative MRI and CT","route":"/technologies/intraoperative-mri-ct/"},{"id":"mri","kind":"technology","name":"MRI","route":"/technologies/mri/"},{"id":"prrt","kind":"technology","name":"Peptide receptor radionuclide therapy (PRRT)","route":"/technologies/prrt/"},{"id":"sbrt","kind":"technology","name":"SBRT / SABR (stereotactic radiotherapy)","route":"/technologies/sbrt/"},{"id":"zap-x","kind":"technology","name":"ZAP-X gyroscopic radiosurgery","route":"/technologies/zap-x/"}],"target":[{"id":"drd2","kind":"target","name":"Dopamine D2 receptor","route":"/targets/drd2/"},{"id":"sstr2","kind":"target","name":"Somatostatin receptor 2","route":"/targets/sstr2/"}],"drug":[{"id":"bevacizumab","kind":"drug","name":"Bevacizumab","route":"/drugs/bevacizumab/"},{"id":"bromocriptine","kind":"drug","name":"Bromocriptine","route":"/drugs/bromocriptine/"},{"id":"cabergoline","kind":"drug","name":"Cabergoline","route":"/drugs/cabergoline/"},{"id":"lutathera","kind":"drug","name":"Lutetium-177 dotatate","route":"/drugs/lutathera/"},{"id":"metyrapone","kind":"drug","name":"Metyrapone","route":"/drugs/metyrapone/"},{"id":"osilodrostat","kind":"drug","name":"Osilodrostat","route":"/drugs/osilodrostat/"},{"id":"pasireotide","kind":"drug","name":"Pasireotide","route":"/drugs/pasireotide/"},{"id":"pegvisomant","kind":"drug","name":"Pegvisomant","route":"/drugs/pegvisomant/"},{"id":"octreotide-lanreotide","kind":"drug","name":"Somatostatin analogues (octreotide, lanreotide)","route":"/drugs/octreotide-lanreotide/"},{"id":"temozolomide","kind":"drug","name":"Temozolomide","route":"/drugs/temozolomide/"}],"pathway":[{"id":"pi3k-akt-mtor","kind":"pathway","name":"PI3K / AKT / mTOR","route":"/pathways/pi3k-akt-mtor/"}],"term":[{"id":"mgmt","kind":"term","name":"MGMT promoter methylation","route":"/terms/mgmt/"},{"id":"rare-cancers","kind":"term","name":"Rare cancers","route":"/terms/rare-cancers/"}],"bottleneck":[{"id":"b-overdiagnosis","kind":"bottleneck","name":"Overdiagnosis and false alarms","route":"/bottlenecks/b-overdiagnosis/"},{"id":"b-rare-cancers","kind":"bottleneck","name":"Rare and paediatric cancers without markets","route":"/bottlenecks/b-rare-cancers/"}],"paper":[{"id":"paper-petersenn-nat-rev-endocrinol","kind":"paper","name":"Diagnosis and management of prolactin-secreting pituitary adenomas: a Pituitary Society international Consensus Statement","route":"/key-papers/paper-petersenn-nat-rev-endocrinol/"},{"id":"paper-raverot-eur-j-endocrinol","kind":"paper","name":"European Society of Endocrinology Clinical Practice Guidelines for the management of aggressive pituitary tumours and carcinomas","route":"/key-papers/paper-raverot-eur-j-endocrinol/"}],"trial":[{"id":"nct05804669","kind":"trial","name":"A Study to Evaluate the Safety and PK of CRN04894 for the Treatment of Cushing's Syndrome","route":"/trials/nct05804669/"},{"id":"nct03271918","kind":"trial","name":"Cabergoline in Nonfunctioning Pituitary Adenomas","route":"/trials/nct03271918/"},{"id":"nct02697734","kind":"trial","name":"Efficacy and Safety Evaluation of Osilodrostat in Cushing's Disease","route":"/trials/nct02697734/"},{"id":"nct01137682","kind":"trial","name":"Efficacy and Safety of Pasireotide Long Acting Release (LAR) Versus Octreotide LAR or Lanreotide Autogel (ATG) in Patients With Inadequately Controlled Acromegaly","route":"/trials/nct01137682/"},{"id":"nct00383708","kind":"trial","name":"Lanreotide Autogel and Pegvisomant Combination Therapy in Acromegalic Patients","route":"/trials/nct00383708/"},{"id":"nct00143416","kind":"trial","name":"Long Term Study With B2036-PEG","route":"/trials/nct00143416/"},{"id":"nct03439709","kind":"trial","name":"Multicenter Evaluation of the Effect of Upfront Radiosurgery on Residual Growth Hormone-secreting Pituitary Adenoma","route":"/trials/nct03439709/"},{"id":"nct00434148","kind":"trial","name":"Safety and Efficacy of Different Dose Levels of Pasireotide in Patients With de Novo, Persistent or Recurrent Cushing's Disease","route":"/trials/nct00434148/"},{"id":"nct01331239","kind":"trial","name":"Safety and Efficacy of LCI699 in Cushing's Disease Patients","route":"/trials/nct01331239/"},{"id":"nct00600886","kind":"trial","name":"Safety and Efficacy of Pasireotide Long Acting Release (LAR) vs. Octreotide LAR in Patients With Active Acromegaly","route":"/trials/nct00600886/"},{"id":"nct07463235","kind":"trial","name":"Safety and Potency of a High Cabergoline Dosage in Microprolactinomas","route":"/trials/nct07463235/"},{"id":"nct02952885","kind":"trial","name":"Strict IGF-1 Control in Acromegaly","route":"/trials/nct02952885/"},{"id":"nct00889525","kind":"trial","name":"Study of Cabergoline in Treatment of Corticotroph Pituitary Tumor","route":"/trials/nct00889525/"}],"journal":[{"id":"brain-tumor-pathology","kind":"journal","name":"Brain tumor pathology","route":"/journals/brain-tumor-pathology/"},{"id":"cns-oncology","kind":"journal","name":"CNS oncology","route":"/journals/cns-oncology/"},{"id":"endocrine-related-cancer","kind":"journal","name":"Endocrine-related cancer","route":"/journals/endocrine-related-cancer/"},{"id":"journal-of-neuro-oncology","kind":"journal","name":"Journal of neuro-oncology","route":"/journals/journal-of-neuro-oncology/"}]}}