Gigantism due to growth hormone excess in a boy with optic glioma

True gigantism is rare in early childhood and is usually due to excess GH secretion from a pituitary adenoma. We report a case in which the endocrine abnormality is secondary to an optic glioma. Careful endocrine evaluation has shown that GH peak amplitude was not increased but rather there was fail...

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Bibliographic Details
Published in:Clinical endocrinology (Oxford) 2000-10, Vol.53 (4), p.535-538
Main Authors: Drimmie, F. M., Maclennan, A. C., Nicoll, J. A. R., Simpson, E., McNeill, E., Donaldson, M. D. C.
Format: Article
Language:English
Subjects:
Adult
Biological and medical sciences
Child, Preschool
Diseases of visual field, optic nerve, optic chiasma and optic tracts
Gigantism - diagnosis
Gigantism - drug therapy
Gigantism - etiology
Gonadotropin-Releasing Hormone - analogs & derivatives
Goserelin - therapeutic use
Growth Hormone - blood
Humans
Magnetic Resonance Imaging
Male
Medical sciences
Neurofibromatosis 1 - complications
Neurofibromatosis 1 - diagnosis
Neurofibromatosis 1 - drug therapy
Octreotide - therapeutic use
Ophthalmology
Optic Nerve Glioma - complications
Optic Nerve Glioma - diagnosis
Optic Nerve Glioma - drug therapy
Puberty, Precocious - complications
Puberty, Precocious - diagnosis
Puberty, Precocious - drug therapy
Somatostatin - analogs & derivatives
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Summary:True gigantism is rare in early childhood and is usually due to excess GH secretion from a pituitary adenoma. We report a case in which the endocrine abnormality is secondary to an optic glioma. Careful endocrine evaluation has shown that GH peak amplitude was not increased but rather there was failure of GH levels to suppress to baseline and a lack of pulsatility. There is no evidence of a direct secretory role for the tumour and we postulate that the tumour is affecting GH secretion through an effect on somatostatin tone. Specific tumour therapy is not indicated for this patient in the absence of mass effect or visual disturbance. The GH excess is being treated with somatostatin analogue (Octreotide) and as he has developed precocious puberty he is also receiving long acting GnRH analogue (Zoladex). This boy appears likely to have neurofibromatosis type 1 (NF1) which raises the question of subtle GH excess in NF1 patients with tall stature.
ISSN:0300-0664
1365-2265
DOI:10.1046/j.1365-2265.2000.01041.x