| Title | Five-year follow-up of a 13-year-old boy with a pituitary adenoma causing gigantism--effect of octreotide therapy. | | Author(s) | Schoof E, Dörr HG, Kiess W, Lüdecke DK, Freitag E, Zindel V, Rascher W, Dötsch J | | Institution | Department of Pediatrics, University of Erlangen-Nürnberg, Loschgestrasse 15, D-91054 Erlangen, Germany. | | Source | Horm Res 2004; 61(4):184-9. | | MeSH | Adenoma
Adolescent
Antineoplastic Agents, Hormonal
GTP-Binding Protein alpha Subunits, Gs
Gigantism
Human Growth Hormone
Humans
Magnetic Resonance Imaging
Male
Neoplasm, Residual
Octreotide
Pituitary Neoplasms
| | Abstract | BACKGROUND/
AIM: 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. | | Language | eng | | Pub Type(s) | Case Reports
Journal Article
| | PubMed ID | 14739526 |
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