title: Gonadotrophin-independent puberty in a boy with a beta-HCG-secreting brain tumour.
abstract: We report on a short-statured boy in whom therapy with recombinant human growth hormone was initiated at the age of 9.7 years for assumed idiopathic growth hormone deficiency. On recombinant human growth hormone, height improved from -1.9 (standard deviation score) to -0.9 within 1 year, and the patient entered puberty spontaneously at 10.7 years. At 11.6 years he showed low morning cortisol and thyroxine levels, but was otherwise well. He showed an inconspicuous growth, and puberty progressed adequately until the age of 13.4 years, when he developed signs of an increased intracranial pressure, and a suprasellar choriocarcinoma was diagnosed. This case confirms the fact that beta chorionic gonadotrophin secreting tumours will not be diagnosed by the characteristic clinical manifestation of gonadotrophin-independent puberty if they occur at a time when normal puberty is expected. Particularly, it raises the question of how often the CNS should be re-evaluated by magnetic resonance imaging in children with growth hormone deficiency and normal initial neuroradiological imaging, when they develop additional hormonal deficiencies but no other clinical symptoms of an intracranial process.
authors.affiliation: Division of Paediatric Endocrinology, University Hospital for Children and Adolescents, Loschgestrasse 25, D-91054 Erlangen, Germany.
authors.forename: M
authors.initials: M
authors.lastname: Marx
authors.forename: J D
authors.initials: JD
authors.lastname: Beck
authors.forename: G G
authors.initials: GG
authors.lastname: Grabenbauer
authors.forename: R
authors.initials: R
authors.lastname: Fahlbusch
authors.forename: H G
authors.initials: HG
authors.lastname: Dörr
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001932:Brain Neoplasms / Q000097:blood / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy; D002822:Choriocarcinoma / Q000097:blood / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy; D018997:Chorionic Gonadotropin, beta Subunit, Human / Q000378:metabolism*; D003131:Combined Modality Therapy; D005500:Follow-Up Studies; D006128:Growth; D019382:Human Growth Hormone / Q000627:therapeutic use*; D006801:Humans; D006854:Hydrocortisone / Q000097:blood; D019586:Intracranial Hypertension / Q000209:etiology; D008279:Magnetic Resonance Imaging; D008297:Male; D011627:Puberty / Q000502:physiology*; D011879:Radiotherapy Dosage; D011994:Recombinant Proteins / Q000627:therapeutic use; D013974:Thyroxine / Q000097:blood; D013997:Time Factors
chemical_list: D018997:Chorionic Gonadotropin, beta Subunit, Human; D011994:Recombinant Proteins; D019382:Human Growth Hormone; D013974:Thyroxine; D006854:Hydrocortisone