Long-term anterior pituitary function in patients with paediatric Cushing's disease treated with pituitary radiotherapy.
OBJECTIVE
Pituitary radiotherapy (RT) is an effective second-line treatment for paediatric Cushing's disease (CD). Although the short-term effects of pituitary RT are well documented, there are less data on possible long-term sequelae. We report the long-term anterior pituitary function in a cohort of paediatric CD patients treated with pituitary RT.


METHODS
Between 1983 and 2006, 12 paediatric CD patients (10 males and 2 females) of mean age 11.4 years at diagnosis (range 6.4-17.4) underwent second-line pituitary RT (45 Gy in 25 fractions), following unsuccessful transsphenoidal surgery. Out of 12, 11 patients were cured by RT (cure interval 0.13-2.86 years) defined by mean serum cortisol of <150 nmol/l on 5-point day curve and midnight sleeping cortisol of <50 nmol/l. Long-term data are available for six male patients, who received RT at the age of 7.0-17.6 years. The mean follow-up from the completion of RT was 10.5 years (6.6-16.5).


RESULTS
At a mean of 1.0 year (0.11-2.54) following RT, GH deficiency (peak GH <1-17.9 mU/l) was present in five out of six patients. On retesting at a mean of 9.3 years (7.6-11.3) after RT, three out of four patients were GH sufficient (peak GH 19.2-50.4 mU/l). Other anterior pituitary functions including serum prolactin in five out of six patients were normal on follow-up. All the six patients had testicular volumes of 20-25 ml at the age of 14.5-28.5 years.


CONCLUSIONS
This series of patients illustrates the absence of serious long-term pituitary deficiency after RT and emphasises the importance of continued surveillance.
Chan
L F
LF
Departments of Endocrinology, St Bartholomew's and The Royal London School of Medicine and Dentistry, London, UK.
Storr
H L
HL
Plowman
P N
PN
Perry
L A
LA
Besser
G M
GM
Grossman
A B
AB
Savage
M O
MO
D000293:Adolescent; D000324:Adrenocorticotropic Hormone / Q000097:blood; D002648:Child; D005500:Follow-Up Studies; D006062:Gonadotropins / Q000097:blood; D019382:Human Growth Hormone / Q000172:deficiency / Q000378:metabolism; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D008297:Male; D047748:Pituitary ACTH Hypersecretion / Q000097:blood / Q000378:metabolism* / Q000503:physiopathology / Q000532:radiotherapy*; D010903:Pituitary Gland, Anterior / Q000378:metabolism*; D010904:Pituitary Gland, Posterior / Q000378:metabolism; D010910:Pituitary Irradiation; D011627:Puberty; D013737:Testis / Q000254:growth & development; D013972:Thyrotropin / Q000097:blood
D006062:Gonadotropins; D019382:Human Growth Hormone; D000324:Adrenocorticotropic Hormone; D013972:Thyrotropin; D006854:Hydrocortisone