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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.
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.
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.
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.
No conventional lymphatics: gliomas spread along white matter tracts and, rarely, through cerebrospinal fluid; medulloblastoma can seed the spine.
Same organ: Lactotroph pituitary neuroendocrine tumour (prolactinoma), Somatotroph pituitary neuroendocrine tumour (acromegaly), Corticotroph pituitary neuroendocrine tumour (Cushing disease and silent corticotroph tumour), Gonadotroph pituitary neuroendocrine tumour (non-functioning adenoma), Thyrotroph pituitary neuroendocrine tumour (TSH-secreting), Pineocytoma and pineal parenchymal tumour of intermediate differentiation, Pineoblastoma, Papillary tumour of the pineal region, Choroid plexus carcinoma, 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, Brain and spinal cord tumours (all types), Astrocytoma, IDH-mutant (grades 2 to 4), Oligodendroglioma, IDH-mutant and 1p/19q-codeleted, Paediatric high-grade glioma (excluding diffuse midline glioma), Meningioma, Brain metastases (secondary brain tumours), Vestibular schwannoma (acoustic neuroma), Central nervous system germ cell tumours (germinoma and non-germinomatous), Spinal cord tumours (intramedullary and intradural), WNT-activated medulloblastoma, SHH-activated medulloblastoma, Group 3 and group 4 medulloblastoma (non-WNT/non-SHH)
Nothing recorded yet.
Nothing recorded yet.
Also on OnCo: Symptoms and red flags · Early detection roadmap.
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.
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One cancer page on OnCo cites this paper by its DOI; this record gives the citation a page of its own so a reader can follow it without leaving OnCo. Read the abstract above alongside the citing page listed under Related; the record was created automatically from the Europe PMC entry and its figures have not been checked by hand.
Query 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.
Temozolomide as first-line chemotherapy.
Lineage-based classification.
The targets of this cancer's medicines and the ones linked to it directly.
Cases by country, the UK and NHS pathway and other country lenses, the expert centres with trials on record, and the centres named on this cancer's subtypes.
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Bleeding that does not stop by itself, bleeding from more than one site, or new bruising in several places or one large area.
Sudden severe bone pain, or back pain with weakness or numbness in the legs (possible spinal cord compression).
Sudden severe abdominal pain, a hard or very tender abdomen, or abdominal pain with vomiting and fever. Boxed warning for gastrointestinal perforation on bevacizumab.
Take on an empty stomach or at bedtime to reduce nausea; PJP prophylaxis during concurrent chemoradiation.
The shared side effects of drugs that block blood vessel growth (bevacizumab, ramucirumab and VEGFR kinase inhibitors): high blood pressure, protein leaking into the urine, nosebleeds and more serious bleeding, slow wound healing, and rarely holes in the bowel.
Death of brain tissue months to years after radiosurgery or high-dose brain radiotherapy, which can look exactly like tumour growing back on a scan.
See all on the product pages:BevacizumabLutetium-177 dotatateTemozolomide·Printable cards in the navigator
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