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Pituitary tumors are growths in the pituitary gland that can affect hormone production. Learn about their types, causes, symptoms, treatment, and prognosis at Guangzhou Fosun Chancheng Hospital.
Pituitary adenomas (now termed pituitary neuroendocrine tumors, PitNETs) are benign monoclonal neoplasms of the anterior pituitary gland. They represent 10-15% of intracranial neoplasms:
Genetic Mutations: Sporadic: GNAS (gsp oncogene) mutations in 30-40% of somatotroph adenomas. USP8 mutations in 35-60% of corticotroph adenomas. PTTG overexpression. MEN1 (menin), CDKN1B (p27), AIP (aryl hydrocarbon receptor-interacting protein), GPR101 (X-LAG syndrome) in familial syndromes.
Familial Syndromes: MEN1 (pituitary + hyperparathyroidism + pancreatic NET), MEN4 (CDKN1B), Carney complex (PRKAR1A): GH-secreting ± PPNAD, spotty pigmentation, myxomas. Familial isolated pituitary adenoma (FIPA): AIP mutations in 15-20%, younger onset, mostly GH/PRL.
Classification: Functional: prolactinoma (40-50%), non-functioning (30-35%), GH-secreting/acromegaly (10-15%), ACTH-secreting/Cushing disease (5-10%), TSH-secreting (<1>4cm).
Demographics: Peak incidence 30-60 years. Female predominance for prolactinomas (micro) and Cushing disease. Equal distribution for acromegaly and NFPA. Autopsy prevalence 10-15% (most are incidental microadenomas).
Hormonal Hypersecretion: Prolactinoma: galactorrhea, amenorrhea/oligomenorrhea, infertility, decreased libido, gynecomastia (men). Acromegaly: acral enlargement, coarse facial features, prognathism, macroglossia, carpal tunnel, diabetes, hypertension, cardiomyopathy, sleep apnea. Cushing disease: central obesity, moon face, buffalo hump, striae, diabetes, hypertension, osteoporosis, mood changes, infections.
Mass Effect: Visual: bitemporal hemianopsia (chiasmal compression most classic), decreased acuity, optic atrophy (chronic). Headache (stretch of diaphragma sellae). Hypopituitarism: fatigue, weight loss/gain, decreased libido, cold intolerance, hypotension (ACTH deficiency - adrenal crisis risk), polyuria/polydipsia (DI).
Pituitary Apoplexy: Sudden hemorrhage/infarction of pituitary adenoma. Severe sudden headache (thunderclap), visual loss, ophthalmoplegia, altered consciousness. Adrenal crisis (ACTH deficiency). Neurosurgical emergency.
Incidental: Asymptomatic pituitary lesion found on imaging for other reasons. Most are microadenomas. Requires hormonal workup to determine functionality.
Prolactinoma: Medical first-line: dopamine agonists (cabergoline preferred, bromocriptine alternative). Normalizes prolactin in 80-90%, tumor shrinkage in 80%. Surgery reserved for DA resistance/intolerance, apoplexy, or CSF leak during DA treatment.
Acromegaly (GH-secreting): Surgery first-line (transsphenoidal). Remission: 80-90% micro, 40-60% macro. Medical: somatostatin analogues (octreotide LAR, lanreotide, pasireotide), GH receptor antagonist (pegvisomant), dopamine agonists (cabergoline for mild disease). RT for residual/recurrent.
Cushing Disease (ACTH-secreting): Surgery first-line. Remission 80-90% micro, 50-70% macro. Medical: steroidogenesis inhibitors (ketoconazole, metyrapone, osilodrostat), pasireotide, cabergoline, mifepristone (glucocorticoid receptor antagonist). Bilateral adrenalectomy for refractory cases (Nelson syndrome risk).
Non-functioning: Surgery for symptomatic (visual deficit, hypopituitarism, apoplexy, growth). Observation for small asymptomatic NFPA (serial MRI). Adjuvant RT for residual/recurrent.
Radiation: SRS for well-defined residual (<3cm>3-5mm from chiasm). Fractionated RT for larger residuals or chiasm proximity. Hormonal normalization takes years. Hypopituitarism risk 30-50% at 5-10 years.
Prolactinoma: DA normalizes prolactin in 80-90%. Tumor shrinkage >50% in 80%. Normalization of gonadal function. Long-term DA needed; withdrawal possible after 2+ years if strict criteria met (normal prolactin, no visible tumor).
Acromegaly: Surgical remission 80-90% (micro), 40-60% (macro). Medical control in >90% with combination therapy. Mortality 2-4x general population if uncontrolled (cardiovascular, respiratory, malignancy); normalized to general population with biochemical control (GH <1>
Cushing Disease: Surgical remission 80-90% (micro). Recurrence 15-25% at 10 years. Medical control possible in most. Mortality 4-5x general population if uncontrolled (cardiovascular, infections, VTE).
Non-functioning: 5-year progression-free survival 80-90% after surgery ± RT. Recurrence 10-20% at 10 years. Hypopituitarism common after treatment.
Prognostic Factors: Size (micro vs. macro), cavernous sinus invasion, extrasellar extension, Ki-67 index (>3% associated with aggressive behavior), p53 expression, mitotic count. Aggressive adenomas and pituitary carcinomas (rare) have poor prognosis.
Hormonal Workup: All pituitary lesions need endocrine evaluation. Prolactin, IGF-1, 24h UFC/dexamethasone testing, TSH/FT4, LH/FSH/testosterone or estradiol. ACTH/cortisol for adrenal axis. Stimulation testing if deficiencies suspected.
Visual Assessment: Formal visual field testing (Humphrey/Goldmann perimetry) at diagnosis and follow-up. Optical coherence tomography for optic nerve/chiasm evaluation. Urgent if chiasmal compression.
Perioperative Care: Stress-dose steroids if ACTH deficiency or Cushing disease (postoperative adrenal insufficiency). Diabetes insipidus management (triphasic response common post-surgery). CSF leak monitoring, meningitis prevention. Sodium monitoring (SIADH or cerebral salt wasting).
Hormone Replacement: Cortisol: hydrocortisone 15-25 mg/day divided doses, sick day rules, emergency injection kit. Thyroxine: check cortisol first to avoid adrenal crisis. Sex hormones: testosterone/estrogen when appropriate. GH replacement: after cure or deficiency confirmed.
Long-term Follow-up: Serial MRI (3 months post-surgery, then annually). Hormonal monitoring (control of hypersecretion + detection of new hypopituitarism). Visual fields. Cardiovascular risk management (Cushing, acromegaly). Bone density (hypogonadism, Cushing). Colonoscopy for acromegaly.
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