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Pituitary Center (H.G.M., T.R.P., V.H.-B., H.S., S.M.), Cedars-Sinai Research Institute, UCLA School of Medicine, Los Angeles, California 90048; and Department of Pathology (K.K.), St. Michaels Hospital, University of Toronto, Ontario, M5B 1W8 Canada
Address correspondence and requests for reprints to: Shlomo Melmed, M.D., Academic Affairs, 2015, Cedars-Sinai Medical Center, 8700 Beverly Boulevard, Los Angeles, California 90048. E-mail: Melmed{at}csmc.edu
Gigantism is caused by GH hypersecretion occurring before
epiphyseal long bone closure and usually is associated with pituitary
adenoma. A 15-yr-old female patient presented with accelerated growth
due to a large pituitary tumor that was surgically resected to relieve
pressure effects. Second surgery to remove residual tumor tissue was
followed by administration of octreotide LAR, a long-acting depot
somatostatin analog, together with long-acting cabergoline. Height was
over the 95th percentile, with evidence of a recent growth spurt. Serum
GH levels were more than 60 ng/mL (normal, <10 ng/mL) with no
suppression to 75 g oral glucose, and serum PRL (>8000 ng/mL;
normal, <23 ng/mL) and insulin-like growth factor I levels (845 ng/mL;
age-matched normal, 242660 ng/mL) were elevated. Histology,
immunostaining, and electron microscopy demonstrated a pituitary
acidophil stem cell adenoma. Tumor tissue expressed both somatostatin
receptor type 2 and dopamine receptor type 2. The Gs
subunit, GHRH
receptor, and MEN1 genes were intact, and tumor tissue abundantly
expressed pituitary tumor transforming gene (PTTG). Serum GH and PRL
levels were controlled after two surgeries, and with continued
cabergoline and octreotide LAR GH, PRL, and insulin-like growth factor
I levels were normalized. In conclusion, administration of long-acting
somatostatin analog every 4 weeks in combination with a long-acting
dopamine agonist biweekly controlled biochemical parameters and
accelerated growth in a patient with gigantism caused by a rare
pituitary acidophil stem cell adenoma.
This article has been cited by other articles:
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S. Melmed Acromegaly N. Engl. J. Med., December 14, 2006; 355(24): 2558 - 2573. [Full Text] [PDF] |
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S.-G. Ren, S. Kim, J. Taylor, J. Dong, J.-P. Moreau, M. D. Culler, and S. Melmed Suppression of Rat and Human Growth Hormone and Prolactin Secretion by a Novel Somatostatin/Dopaminergic Chimeric Ligand J. Clin. Endocrinol. Metab., November 1, 2003; 88(11): 5414 - 5421. [Abstract] [Full Text] [PDF] |
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