Overview
Pituitary tumours are the third most common adult brain tumour and are almost always benign and curable. They cause problems through excessive hormone production, the mass effect of the tumour itself, or inadequate hormone production from the rest of the pituitary gland.
Classification
Non-functional vs. functional
Pituitary adenomas can be functional or non-functional depending on whether they secrete hormones in excess or not:
- Non-functional – do not secrete a hormone in excess and are most common
- Functional – secrete a hormone in excess – from most common to least common:
- Prolactinomas – secrete excess prolactin
- Somatotroph adenomas – secrete excess growth hormone (GH)
- Corticotroph adenomas – secrete excess adrenocorticotropic hormone (ACTH)
- Thyrotroph adenomas – secrete excess thyroid-stimulating hormone (TSH)
- Gonadotroph adenomas – secrete excess follicle-stimulating hormone (FSH) and luteinising hormone (LH)
According to size
Pituitary adenomas can also be classified according to size:
- Macroadenoma: tumour >1cm
- Microadenoma: tumour <1cm
Epidemiology
- Third most common brain tumour in adults
- Adenomas make up most pituitary tumours, a minority of which are symptomatic
- Prolactinomas are the most common functional pituitary adenoma
- Many pituitary tumours are diagnosed incidentally
Risk Factors
- Multiple endocrine neoplasia type 1 (MEN-1)
- Autosomal dominant and associated with pituitary adenoma
- Family history
Presentation
Pituitary adenomas cause symptoms by:
- Excess production of a hormone:
- Hyperprolactinaemia – due to excess prolactin secretion
- Acromegaly – due to excess growth hormone secretion
- Cushing’s disease – due to excess ACTH secretion
- Hyperthyroidism – due to excess TSH secretion
- Mass effect of the tumour itself:
- Headaches – usually progressive and worse on waking/change with posture
- Visual field defects:
- Usually bitemporal hemianopia due to the tumour pressing on the optic chiasm, the upper half is affected more than the lower
- Squint
- Other features of Elevated Intracranial Pressure
- Visual field defects:
- Headaches – usually progressive and worse on waking/change with posture
- Inadequate hormone production from the rest of the pituitary gland:
- Compression on the rest of the pituitary gland can cause hypopituitarism
- Non-functioning tumours may cause generalised hypopituitarism (panhypopituitarism) for this reason
- Compression on the rest of the pituitary gland can cause hypopituitarism
Differential Diagnoses
Prolactin-secreting adenoma (prolactinoma)
- Excess prolactin exerts negative feedback on gonadotropin-releasing hormone (GnRH):
- In men: galactorrhoea, impotence, loss of libido
- In women: galactorrhoea, amenorrhoea, infertility
- Prolactin levels raised
Growth-hormone secreting adenoma (acromegaly)
- Coarse facial features, increased shoe size, prognathism, excess sweating
- Glucose intolerance present
- Serum insulin-like growth factor 1 (IGF-1) raised
ACTH-secreting adenoma (Cushing’s disease)
- Features of Cushing’s syndrome: central obesity, muscle wasting, bruising etc.
- Impaired glucose tolerance and hypokalaemic metabolic alkalosis may be seen
- Cortisol release is not suppressed with low-dose dexamethasone but is with high-dose
Thyroid-stimulating hormone-secreting adenoma
- Features of hyperthyroidism: tremors, weight loss, sweating, heat intolerance, palpitations
- TSH, T4, and T3 elevated
Non-functioning pituitary adenoma
- Mass effect-related problems predominate: headaches, visual field defects
- Panhypopituitarism may be present
- Bitemporal hemianopia usually worse in upper half of visual field
Craniopharyngioma
- More common in children and adults >60 years
- Patients may have cranial diabetes insipidus
- Bitemporal hemianopia usually worse in lower half of visual field
Investigations
- Pituitary function tests:
- Prolactin
- IGF-1
- FSH and LH
- Oestrogen and testosterone
- TFTs
- ACTH
- Morning cortisol
- Urea and electrolytes (U&Es):
- May show electrolyte disturbances
- Pituitary MRI:
- Shows pituitary mass
- Visual field testing:
- If imaging shows pressure on the optic chiasm
Management
Prolactin-secreting adenoma (prolactinoma)
- 1st-line: dopamine agonists (cabergoline/bromocriptine)
- If medical therapy ineffective/not tolerated: transsphenoidal surgery
Growth-hormone secreting adenoma (acromegaly)
- 1st-line: transsphenoidal surgery
- If surgery not possible/unsuccessful:
- 1st-line: somatostatin analogue (octreotide)
- 2nd-line: growth-hormone receptor antagonist (pegvisomant)
- Consider dopamine agonist (cabergoline)
- Consider radiotherapy
ACTH-secreting adenoma (Cushing’s disease)
- 1st-line: transsphenoidal surgery
Thyroid-stimulating hormone-secreting adenoma
- 1st-line: transsphenoidal surgery
Non-functioning pituitary adenoma
- If microadenoma: observation
- If macroadenoma with no mass effect: observation
- If macroadenoma with mass effect: consider transsphenoidal surgery
Complications
- Pituitary apoplexy – see Pituitary Apoplexy
- Hypopituitarism
Prognosis
- Remission is often achieved in most patients with microadenomas and around half of those with macroadenomas
- Most pituitary adenomas are benign and curable