Five-year follow-up of a 13-year-old boy with a pituitary adenoma causing gigantism--effect of octreotide therapy.

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.

• Authors: Ellen Schoof, Helmuth G Dörr, Wieland Kiess, Dieter K Lüdecke, Eduard Freitag, Volker Zindel, Wolfgang Rascher, Jörg Dötsch
• Journal: Hormone research (Horm Res) Vol. 61 Issue 4 Pg. 184-9 ( 2004) ISSN: 0301-0163 [Print] Switzerland
• PMID: 14739526 (Publication Type: Case Reports, Journal Article)
• Copyright: Copyright 2004 S. Karger AG, Basel

Chemical References

• Antineoplastic Agents, Hormonal
• Human Growth Hormone
• GTP-Binding Protein alpha Subunits, Gs
• Octreotide

Topics

• Adenoma (complications, drug therapy, genetics, surgery)
• Adolescent
• Antineoplastic Agents, Hormonal (therapeutic use)
• GTP-Binding Protein alpha Subunits, Gs (genetics)
• Gigantism (diagnosis, etiology)
• Human Growth Hormone (metabolism)
• Humans
• Magnetic Resonance Imaging
• Male
• Neoplasm, Residual (drug therapy)
• Octreotide (therapeutic use)
• Pituitary Neoplasms (complications, drug therapy, genetics, surgery)