ACROMEGALY

Acromegaly is caused by growth hormone (GH) secre9on from a pituitary tumour, usually a macroadenoma, and carries an ~twofold
excess mortality when untreated.

Clinical features Management

• If GH hypersecre9on occurs before puberty, then the The main aims are to improve symptoms and to normalise serum
presenta9on is with gigan9sm. GH and IGF-1 to reduce morbidity and mortality:
• More commonly, GH excess occurs in adult life and presents with
acromegaly. Surgical
• If hypersecre9on starts in adolescence and persists into adult • Trans-sphenoidal surgery is usually the first line of treatment and
life, then the two condi9ons may be combined. may result in cure of GH excess, especially in pa9ents with
• The most common complaints are headache and swea9ng. microadenomas.
• Addi9onal features include those of any pituitary tumour. • More oVen, surgery serves to debulk the tumour and further
second-line therapy is required, according to post-opera9ve
Inves8ga8ons imaging and glucose tolerance test results.
• The clinical diagnosis must be confirmed by measuring GH levels
during an oral glucose tolerance test and measuring serum Radiotherapy
IGF-1. In normal subjects, plasma GH suppresses to below 0.5 • External radiotherapy is usually employed as second-line
μg/L (~2 mIU/L). In acromegaly, GH does not suppress and in treatment if acromegaly persists aVer surgery, to stop tumour
about 30% of pa9ents there is a paradoxical rise; IGF-1 is also growth and lower GH levels.
elevated. • However, GH levels fall slowly (over many years) and there is a
• The rest of pituitary func9on should be inves9gated. risk of hypopituitarism.
• Prolac9n concentra9ons are elevated in about 30% of pa9ents
due to co-secre9on of prolac9n from the tumour. Medical
• Addi9onal tests in acromegaly may include screening for colonic • If acromegaly persists aVer surgery, medical therapy is usually
neoplasms with colonoscopy. employed to lower GH levels to below 1.0 μg/L (~3 mIU/L) and
to normalise IGF-1 concentra9ons.
• Medical therapy may be discon9nued aVer several years in
pa9ents who have received radiotherapy.
• Somatosta9n analogues (such as octreo9de, lanreo9de or
pasireo9de) can be administered as slow-release injec9ons
every few weeks. Somatosta9n analogues can also be used as
primary therapy for acromegaly either as an alterna9ve or in
advance of surgery, given evidence that they can induce modest
tumour shrinkage in some pa9ents.
• Dopamine agonists are less effec9ve at lowering GH but may
some9mes be helpful, especially with associated prolac9n
excess.
• Pegvisomant is a pep9de GH receptor antagonist administered
by daily self-injec9on and may be indicated in some pa9ents
whose GH and IGF-1 concentra9ons fail to suppress sufficiently
following somatosta9n analogue therapy.