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 Gsα 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 × 100 µg/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 µg/l. After 5 years of octreotide therapy, GH (6.9 µg/l), IGF-I (620 µg/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 µg/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.

Eight-Year Follow-Up of a Child with a GH/Prolactin-Secreting Adenoma: Efficacy of Pegvisomant Therapy

“…Medical therapy in acromegalic patients has highly progressed with the availability of dopamine agonists and somatostatin analogues (such as octreotide and lanreotide). In gigantism, treatment with somatostatin analogues has been found to be effective in some cases [8,10,11,12], but not in others, even in combination with cabergoline [13,14,15,16]. Since 2004, pegvisomant, a pegylated GH receptor antagonist, represents a new therapeutic option for the management of patients with acromegaly.…”

Case Study of a 15-Year-old Boy with McCune-Albright Syndrome Combined with Pituitary Gigantism: Effect of Octreotide-long Acting Release (LAR) and Cabergoline Therapy

“…Studies of octreotide-LAR to acromegaly in adulthood demonstrated that several patients developed gallstones and impairment of glucose tolerance [16][17][18]. In two previous reports of treatment of GH excess in MAS, no adverse event due to octreotide-LAR was reported [5,12]. Three-year duration of octreotide-LAR is likely to cause side effects in our patient.…”

“…Two patients who treated with the combination of cabergoline and octreotide-LAR were children (12 and 14 years-old). Schoof et al [12] reported a 13 year-old boy of MAS and gigantism. They also used octreotide-LAR for one year and it was partially effective for the patient.…”

“…Furthermore, GH excess in MAS is a risk factor for loss of vision and hearing due to expansion of fibrous dysplasia of craniofacial bone [5,7,8]. Medical therapy has been done to treat GH excess in MAS; however, the effects are limited [9][10][11][12].…”

Genetic Analysis of Tall Stature

“…In young patients with excessive growth hormone secretion their height usually exceeds 3 or 4 SDs. In most of these children the excessive growth hormone secretion results from a pituitary adenoma or hyperplasia, which is genetically determined in a minority of cases [27, 28]. One of these genetic conditions is the McCune-Albright syndrome.…”