Pituitary tumours: acromegaly.
Excessive production of the growth hormone (GH) is responsible for acromegaly. It is related to a pituitary GH-secreting adenoma in most cases. Prevalence is estimated 40-130 per million inhabitants. It is characterised by slowly progressive acquired somatic disfigurement (mainly involving the face and extremities) and systemic manifestations. The rheumatologic, cardiovascular, respiratory and metabolic consequences determine its prognosis. The diagnosis is confirmed by an increased serum GH concentration, unsuppressible by an oral glucose load and by detection of increased levels of insulin-like growth factor-I (IGF-I). Treatment is aimed at correcting (or preventing) tumour compression by excising the disease-causing lesion, and at reducing GH and IGF-I levels to normal values. When surgery, the usual first-line treatment, fails to correct GH/IGF-I hypersecretion, medical treatment with somatostatin analogues and/or radiotherapy can be used. The GH-receptor antagonist (pegvisomant) is helpful in patients who are resistant to somatostatin analogues. Thanks to this multistep therapeutic strategy, adequate hormonal disease control is achieved in most cases, allowing a normal life expectancy.
Chanson
Philippe
P
Assistance Publique-Hôpitaux de Paris, Hôpital de Bicêtre, Université Paris-Sud 11, INSERM U693, Le Kremlin-Bicêtre, France. philippe.chanson@bct.aphp.fr
Salenave
Sylvie
S
Kamenicky
Peter
P
Cazabat
Laure
L
Young
Jacques
J
D000172:Acromegaly / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology* / Q000628:therapy; D000236:Adenoma / Q000150:complications* / Q000175:diagnosis / Q000378:metabolism / Q000628:therapy; D002277:Carcinoma / Q000175:diagnosis / Q000378:metabolism / Q000628:therapy; D049912:Growth Hormone-Secreting Pituitary Adenoma / Q000150:complications* / Q000175:diagnosis / Q000378:metabolism / Q000628:therapy; D006801:Humans; D008954:Models, Biological; D011364:Professional Practice