Five-year follow-up of a 13-year-old boy with a pituitary adenoma causing gigantism--effect of octreotide therapy.
OBJECTIVE
In children, there is little experience with octreotide therapy for pituitary tumors, especially growth hormone (GH) producing adenomas. We report on a 13-year-old boy with gigantism due to a GH-producing pituitary adenoma caused by a Gsalpha mutation on the basis of McCune-Albright syndrome.


METHODS
At the age of 6.5 years a GH- and prolactin-producing pituitary adenoma was diagnosed. The adenoma was surgically removed. Immediately thereafter, the small adenoma residuum was treated with octreotide (2 x 100 microg/day s.c.).


RESULTS
During therapy with octreotide, the growth rate dropped to normal values; however, rose again after 2 years of treatment. The insulin-like growth factor I (IGF-I) levels remained above the 95th percentile, the GH level mostly >2 microg/l. After 5 years of octreotide therapy, GH (6.9 microg/l), IGF-I (620 microg/l), IGF-binding protein 3 (5.4 mg/l), and prolactin (17.0 ng/ml) levels were still elevated. The growth velocity was +2.4 SDS (standard deviation score), the pubertal status was mature, and the bone age was 14.3 years (prospective final height 208 cm). A magnetic resonance imaging scan showed an unchanged residual 4-mm rim of adenoma at the pituitary site. Side effects from octreotide therapy were not reported by the patient or his family. The therapy was changed to the long-acting release octreotide analog octreotide-LAR. After 1 year of treatment with octreotide-LAR, the GH level was 1.0 microg/l, and the prospective final height dropped by 10 cm.


CONCLUSIONS
This case demonstrates that combined surgical and medical treatment can influence the prognosis of childhood gigantism; however, the prognosis of this rare condition remains uncertain.
Schoof
Ellen
E
Department of Pediatrics, University of Erlangen-Nürnberg, Loschgestrasse 15, D-91054 Erlangen, Germany.
Dörr
Helmuth G
HG
Kiess
Wieland
W
Lüdecke
Dieter K
DK
Freitag
Eduard
E
Zindel
Volker
V
Rascher
Wolfgang
W
Dötsch
Jörg
J
D000236:Adenoma / Q000150:complications* / Q000188:drug therapy* / Q000235:genetics / Q000601:surgery; D000293:Adolescent; D018931:Antineoplastic Agents, Hormonal / Q000627:therapeutic use*; D019205:GTP-Binding Protein alpha Subunits, Gs / Q000235:genetics; D005877:Gigantism / Q000175:diagnosis / Q000209:etiology*; D019382:Human Growth Hormone / Q000378:metabolism; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D018365:Neoplasm, Residual / Q000188:drug therapy; D015282:Octreotide / Q000627:therapeutic use*; D010911:Pituitary Neoplasms / Q000150:complications* / Q000188:drug therapy* / Q000235:genetics / Q000601:surgery
D018931:Antineoplastic Agents, Hormonal; D019382:Human Growth Hormone; D019205:GTP-Binding Protein alpha Subunits, Gs; D015282:Octreotide