{"entity":{"id":"corticotroph-pitnet","kind":"cancer","name":"Corticotroph pituitary neuroendocrine tumour (Cushing disease and silent corticotroph tumour)","aka":["Corticotroph tumour","Corticotroph tumour (Cushing disease, silent corticotroph)","ACTH-secreting pituitary adenoma","Cushing disease","Silent corticotroph adenoma","Crooke cell tumour"],"tldr":"A corticotroph tumour is a pituitary tumour of the cells that make ACTH. When it secretes it floods the body with cortisol, causing Cushing disease (weight gain, diabetes, high blood pressure, thin skin); the silent form is found as a large non-functioning mass. Surgery through the nose is the main treatment; cortisol-lowering drugs, repeat surgery or radiotherapy follow for those not cured.","summary":"The 2022 WHO classification places the corticotroph tumour in the TPIT lineage with densely granulated, sparsely granulated and Crooke cell subtypes, functioning (Cushing disease) or silent (Asa 2022). Silent corticotroph tumours were graded as high-risk adenomas in the 2017 classification; a meta-analysis of 14 studies with 297 patients found a recurrence rate of 5.96 per 100 person-years and recurrence in 31 percent at five or more years, without proof of a higher risk than other non-functioning adenomas (JCEM 2018). In 100 silent corticotroph against 841 other non-functioning adenomas at one institution, the silent corticotroph group presented more often with cranial neuropathy (13 against 5.7 percent) and the series defined recurrence patterns and treatment strategies (J Neurosurg 2021).\n\nHow it differs from its parent: the functioning tumour is usually a microadenoma diagnosed by the endocrine syndrome and cured by selective surgery in most, whereas the silent tumour is a macroadenoma diagnosed by mass effects; both recur more than other pituitary tumours and the Crooke cell subtype is aggressive.\n\nHow common: no separate incidence figure in the sources read.\n\nTreatment: transsphenoidal selective adenomectomy first; for persistent or recurrent Cushing disease, repeat surgery, radiotherapy (including radiosurgery), and cortisol-directed drugs (osilodrostat, ketoconazole, metyrapone), pasireotide or cabergoline directed at the tumour, or bilateral adrenalectomy; silent tumours by surgery and radiotherapy for regrowth, with temozolomide for aggressive tumours, as on the parent page.","asOf":"2026-09-24","wikipedia":"https://en.wikipedia.org/wiki/Cushing%27s_disease","links":[{"label":"NCI PDQ: pituitary tumours treatment","url":"https://www.cancer.gov/types/pituitary/treatment"},{"label":"Asa 2022, Endocrine Pathology: overview of the 2022 WHO classification of pituitary tumours","url":"https://doi.org/10.1007/s12022-022-09703-7"},{"label":"JCEM 2018: recurrence in silent corticotroph adenomas after primary treatment, meta-analysis of 297 patients","url":"https://doi.org/10.1210/jc.2018-01956"},{"label":"J Neurosurg 2021: silent corticotroph adenomas, 100 patients against 841 other non-functioning adenomas","url":"https://doi.org/10.3171/2020.10.jns203236"}],"tags":["subtype-page","wave4","rare"],"related":["pituitary-tumours","lactotroph-pitnet","somatotroph-pitnet","gonadotroph-pitnet","adrenocortical"],"cancers":[],"sections":[],"technologies":["radiosurgery-srs"],"targets":[],"drugs":["osilodrostat","pasireotide","cabergoline","mifepristone","temozolomide"],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"endocrine","burden":"The tumour behind Cushing disease and about a tenth of non-functioning pituitary tumours in its silent form; one centre operated on 100 silent corticotroph and 841 other non-functioning adenomas over 2000 to 2019 (J Neurosurg 2021). GLOBOCAN does not count pituitary tumours.","subtypes":["Densely granulated corticotroph tumour (typical Cushing disease microadenoma)","Sparsely granulated corticotroph tumour","Crooke cell tumour (aggressive)","Silent corticotroph tumour (ACTH-immunopositive, non-functioning macroadenoma; 31 percent recur)"],"biomarkers":["Late-night salivary cortisol, urinary free cortisol, dexamethasone suppression, plasma ACTH","TPIT lineage and ACTH immunostaining; Crooke hyaline change","Inferior petrosal sinus sampling when MRI is negative","Ki-67 and USP8 mutation (functioning microadenomas)"],"standardOfCare":[{"setting":"Cushing disease, first line","approach":"Transsphenoidal selective adenomectomy.","refs":["pituitary-tumours"]},{"setting":"Persistent or recurrent","approach":"Repeat surgery, radiotherapy, osilodrostat or other cortisol-directed drugs, pasireotide or cabergoline, or bilateral adrenalectomy; temozolomide for aggressive tumours.","refs":["osilodrostat","pasireotide","cabergoline","radiosurgery-srs","temozolomide","mifepristone"]}],"stateOfArt":[],"history":[],"pipeline":[],"openProblems":[],"parent":"pituitary-tumours"},"route":"/cancers/corticotroph-pitnet/","neighbours":{"cancer":[{"id":"adrenocortical","kind":"cancer","name":"Adrenocortical carcinoma","route":"/cancers/adrenocortical/"},{"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":"pituitary-tumours","kind":"cancer","name":"Pituitary tumours (pituitary neuroendocrine tumours) and pituitary carcinoma","route":"/cancers/pituitary-tumours/"},{"id":"somatotroph-pitnet","kind":"cancer","name":"Somatotroph pituitary neuroendocrine tumour (acromegaly)","route":"/cancers/somatotroph-pitnet/"}],"technology":[{"id":"radiosurgery-srs","kind":"technology","name":"Stereotactic radiosurgery (Gamma Knife, CyberKnife, linac SRS)","route":"/technologies/radiosurgery-srs/"}],"drug":[{"id":"cabergoline","kind":"drug","name":"Cabergoline","route":"/drugs/cabergoline/"},{"id":"mifepristone","kind":"drug","name":"Mifepristone","route":"/drugs/mifepristone/"},{"id":"osilodrostat","kind":"drug","name":"Osilodrostat","route":"/drugs/osilodrostat/"},{"id":"pasireotide","kind":"drug","name":"Pasireotide","route":"/drugs/pasireotide/"},{"id":"temozolomide","kind":"drug","name":"Temozolomide","route":"/drugs/temozolomide/"}]}}