Abstract
Growth hormone (GH) secreting adenoma represents a therapeutic challenge in childhood. Because of its rarity no treatment guidelines are available and pediatric management often results from recommendations issued for adults. We report a case of a 4-year-old girl with somatotropinoma successfully treated with only medical treatment. She presented with tall stature and history of growth acceleration. Imaging and laboratory confirmed the diagnosis of GH secreting macroadenoma. She started medical treatment with a somatostatin analogue and a dopamine agonist. During an 8-year follow-up period, a good clinical control of the disease and a shrinkage of the adenoma have been demonstrated. At the last observation she achieved normal near-adult height and pubertal development. According to our experience and limited literature evidences, first line treatment with somatostatin analogues can be attempted in patients with somatotropinoma. This approach seems to be able to control the clinical course of the disease, allowing to postpone transphenoidal surgery to adult age or to avoid it.
References
1. Personnier C, Cazabat L, Bertherat J, Gaillard S, Souberbielle JC, et al. Clinical features and treatment of pediatric somatotropinoma: case study of an aggressive tumor due to a new AIP mutation and extensive literature review. Horm Res Paediatr 2011;75:392–402.10.1159/000327831Search in Google Scholar PubMed
2. Nozières C, Berlier P, Dupuis C, Raynaud-Ravni C, Morel Y, et al. Sporadic and genetic forms of paediatric somatotropinoma: a retrospective analysis of seven cases and a review of the literature. Orphanet J Rare Dis 2011;6:67.10.1186/1750-1172-6-67Search in Google Scholar PubMed PubMed Central
3. Trivellin G, Daly AF, Faucz FR, Yuan B, Rostomyan L, et al. Gigantism and acromegaly due to Xq26 microduplications and GPR101 mutation. N Engl J Med 2014;371:2363–74.10.1056/NEJMoa1408028Search in Google Scholar PubMed PubMed Central
4. Abe T, Tara LA, Lüdecke DK. Growth hormone-secreting pituitary adenomas in childhood and adolescence: features and results of transnasal surgery. Neurosurgery 1999;45:1–10.10.1227/00006123-199907000-00001Search in Google Scholar
5. Schoof E, Dörr HG, Kiess W, Lüdecke DK, Freitag E, et al. Five-year follow-up of a 13-year-old boy with a pituitary adenoma causing gigantism-effect of octreotide therapy. Horm Res 2004;61: 184–9.10.1159/000076386Search in Google Scholar PubMed
6. Salenave S, Boyce AM, Collins MT, Chanson P. Acromegaly and McCune-Albright syndrome. J Clin Endocrinol Metab 2014;99:1955–69.10.1210/jc.2013-3826Search in Google Scholar PubMed PubMed Central
7. Giustina A, Chanson P, Kleinberg D, Bronstein MD, Clemmons DR, et al. Expert consensus document: a consensus on the medical treatment of acromegaly. Nat Rev Endocrinol 2014;10: 243–8.10.1038/nrendo.2014.21Search in Google Scholar PubMed
8. Wang L, Wang Q, Li G, Liu W. Dynamic changes in the hypothalamic- pituitary-adrenal axis during growth hormone therapy in children with growth hormone deficiency: a multicenter retrospective study. J Pediatr Endocrinol Metab 2015;28: 975–9.10.1515/jpem-2014-0097Search in Google Scholar PubMed
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