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Gigantism due to growth hormone excess in a boy with optic glioma
Author(s) -
Drimmie F. M.,
Maclennan A. C.,
Nicoll J. A. R.,
Simpson E.,
McNeill E.,
Donaldson M. D. C.
Publication year - 2000
Publication title -
clinical endocrinology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.055
H-Index - 147
eISSN - 1365-2265
pISSN - 0300-0664
DOI - 10.1046/j.1365-2265.2000.01041.x
Subject(s) - gigantism , endocrinology , medicine , acromegaly , octreotide , somatostatin , neurofibromatosis , tall stature , short stature , endocrine system , precocious puberty , adenoma , abnormality , hormone , growth hormone , pathology , psychiatry
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.