title: Long-term first line medical treatment in a 4-year-old girl with Xq26.3 microduplication-negative somatotropinoma. Case report and literature review.
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.
authors.forename: Chiara
authors.initials: C
authors.lastname: Maggioli
authors.forename: Paola
authors.initials: P
authors.lastname: Cambiaso
authors.forename: Sara
authors.initials: S
authors.lastname: Ciccone
authors.forename: Giovanna Stefania
authors.initials: GS
authors.lastname: Colafati
authors.forename: Marco
authors.initials: M
authors.lastname: Cappa
mesh_terms: D000236:Adenoma / Q000188:drug therapy* / Q000235:genetics / Q000473:pathology; D000328:Adult; D002648:Child; D002675:Child, Preschool; D058674:Chromosome Duplication; D041321:Chromosomes, Human, X / Q000235:genetics*; D018491:Dopamine Agonists / Q000627:therapeutic use*; D005260:Female; D049912:Growth Hormone-Secreting Pituitary Adenoma / Q000188:drug therapy* / Q000235:genetics / Q000473:pathology; D019382:Human Growth Hormone / Q000378:metabolism*; D006801:Humans; D011379:Prognosis
chemical_list: D018491:Dopamine Agonists; D019382:Human Growth Hormone