Gigantism Due to Pituitary Mammosomatotroph Hyperplasia

Moran, Antoinette; Sylvia, L.; Kovács, Kálmán; Horváth, Éva; Singer, William; Sagman, Uri; Reubi, Jean–Claude; Wilson, Charles B.; Larson, Robert A.; Pescovitz, Ora Hirsch

doi:10.1056/nejm199008023230507

Cited by 74 publications

(32 citation statements)

References 27 publications

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“…Hyperprolactinemia was noted in 12 of 15 paediatric patients, whereas in adults with a GH-secreting adenoma, its prevalence is 20 -40%. Even a congenital onset of GH excess has been suggested by linear growth acceleration occurring within the first months of life in children with documented gigantism (12,18,19). To our knowledge, the presence of extensive mixed Band T-cell inflammatory response in the tissue of a pituitary adenoma is unusual and it may suggest a component of autoimmune hypophysitis.…”

Section: Discussionmentioning

confidence: 90%

Pegvisomant therapy in pituitary gigantism: successful treatment in a 12-year-old girl

Rix¹,

Laurberg²,

Hoejberg³

et al. 2005

eur j endocrinol

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Objective: The use of a growth hormone (GH) receptor antagonist, pegvisomant has shown great promise in adults with acromegaly, but experience in paediatric patients is lacking. We aimed to describe the results of pegvisomant therapy in a 12-year-old girl with an aggressive GH-secreting pituitary tumour. Design: To evaluate the ability of pegvisomant therapy to control the effects of peripheral GH excess in a case of pituitary gigantism. Methods: Pegvisomant was introduced at 10 mg/day, given subcutaneously, and gradually increased to 20 mg/day until serum IGF-I was normal for age. Results: A large pituitary adenoma with suprasellar extension was diagnosed in a 12-year-old girl with progressive tall stature (178 cm), GH hypersecretion without suppression during oral glucose loading (nadir serum GH, 90 mU/l), high serum IGF-I and serum prolactin levels. Surgical extirpation was not possible because tumour tissue was fibrous and adherent to the optical nerves. Histological examination showed a mixed GH-and prolactin-secreting adenoma with lymphocytic infiltration of B and T cells. Treatment with a dopamine agonist, cabergoline, normalized serum prolactin, but GH secretion was resistant to both somatostatin analogue, octreotide and cabergoline. Radiation followed by pegvisomant therapy titrated up in dose to 20 mg/day led to a marked reduction in GH secretion and normalization of IGF-I, and to growth arrest and improvement of well-being. Conclusions: We suggest that treatment in pituitary gigantism with pegvisomant is safe and may normalize IGF-I levels and effectively stop growing.

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Section: Discussionmentioning

confidence: 90%

Pegvisomant therapy in pituitary gigantism: successful treatment in a 12-year-old girl

Rix¹,

Laurberg²,

Hoejberg³

et al. 2005

eur j endocrinol

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“…Although not definitively proven, clinical cases that support this hypothesis include congenital gigantism with massive diffuse pituitary hyperplasia, in which biochemical studies suggested central GHRH hypersecretion (5), as well as a case of mammosomatotroph hyperplasia in which systemic GHRH concentrations were found to be normal (4). The involvement of mammosomatotrophs, frequently a feature of GH excess originating in childhood (22), is further suggestive of early onset increased GHRH exposure because this cell type predominates in fetal life but is rare in the adult.…”

Section: Ghrh Excessmentioning

confidence: 99%

Gigantism

Eugster

Pescovitz

1999

The Journal of Clinical Endocrinology &Amp; Metabolism

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“…Usually, GH hypersecretion is caused by pituitary adenomas; however, other causes such as somatotroph hyperplasia of the pituitary [1, 2, 3], an optic glioma, or a hypothalamic mass causing inhibition of the somatostatin tone [4, 5, 6]have been described.…”

Section: Introductionmentioning

confidence: 99%

Five-Year Follow-Up of a 13-Year-Old Boy with a Pituitary Adenoma Causing Gigantism – Effect of Octreotide Therapy

Schoof

Dörr²,

Kieß³

et al. 2004

Horm Res Paediatr

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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.

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Gigantism Due to Pituitary Mammosomatotroph Hyperplasia

Cited by 74 publications

References 27 publications

Pegvisomant therapy in pituitary gigantism: successful treatment in a 12-year-old girl

Pegvisomant therapy in pituitary gigantism: successful treatment in a 12-year-old girl

Gigantism

Five-Year Follow-Up of a 13-Year-Old Boy with a Pituitary Adenoma Causing Gigantism – Effect of Octreotide Therapy

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