title: Endocrine outcome in long-term survivors of low-grade hypothalamic/chiasmatic glioma.
abstract: OBJECTIVE
  We have evaluated the frequency of endocrine abnormalities in a large group of patients with hypothalamic/chiasmatic glioma (H/CG) and its correlation with the different forms of therapy.
  
  
  METHODS
  Descriptive retrospective study using case note review analysis.
  
  
  METHODS
  The records of 68 children who survived H/CG were analysed. One third had neurofibromatosis. The mean age at tumour presentation was 5 years. The median time of follow-up was 3.6 years. Thirty-eight children received cranial radiation, of whom 17 also had surgery. Surgery was performed in a total of 24 patients. Fifteen patients received only chemotherapy. Eight children, all with neurofibromatosis, received no specific tumour treatment.
  
  
  METHODS
  Endocrine dysfunction was determined by clinical manifestations and biochemical evaluation of hypothalamic-pituitary function.
  
  
  RESULTS
  Endocrine dysfunction occurred in 42% of the children. The most common disorder was GH deficiency (GHD). Of 50 children evaluated, 15 of the 19 with GHD received cranial irradiation (P < 0.05). HOwever, 15 children treated with more than 15 Gy grew normally. Precocious puberty was diagnosed in 11 patients. Nine patients, all treated with cranial irradiation, developed hypogonadotrophic hypogonadism. Of the 14 patients with hypothyroidism, 10 had surgery (P < 0.005). Hypoadrenalism and diabetes insipidus each occurred in eight patients, and were associated with multiple endocrine deficiencies and surgery. Endocrine deficiencies occurred in children with neurofibromatosis as frequently as children without neurofibromatosis but only when comparing those treated with cranial irradiation or surgery.
  
  
  CONCLUSIONS
  Nearly all studies assessing the patients with different tumour therapy evaluate patients wit different tumour types. This study investigates a specific and large population of patients with H/CG and correlates the different form of treatment with the endocrine outcome. Precocious puberty, in children with this tumour, is probably due to tumour location rather than oncological therapy. Conversely, although endocrine deficiencies can be a result of tumour location, the major causes of endocrine abnormalities were field irradiation and tumour surgery. A notable finding not previously reported is that endocrine dysfunction occurs less often in neurofibromatosis patients treated conservatively. Furthermore, this study documents that a significant number of young children grew normally despite receiving brain irradiation of greater than 45 Gy.
authors.affiliation: Department of Pediatrics, The Children's Hospital of Philadelphia, University of Pennsylvania, USA.
authors.forename: P F
authors.initials: PF
authors.lastname: Collet-Solberg
authors.forename: H
authors.initials: H
authors.lastname: Sernyak
authors.forename: M
authors.initials: M
authors.lastname: Satin-Smith
authors.forename: L L
authors.initials: LL
authors.lastname: Katz
authors.forename: L
authors.initials: L
authors.lastname: Sutton
authors.forename: P
authors.initials: P
authors.lastname: Molloy
authors.forename: T
authors.initials: T
authors.lastname: Moshang
mesh_terms: D000293:Adolescent; D000305:Adrenal Cortex Hormones / Q000172:deficiency; D000328:Adult; D002648:Child; D016371:Cranial Irradiation / Q000009:adverse effects; D003390:Cranial Nerve Neoplasms / Q000378:metabolism* / Q000532:radiotherapy / Q000601:surgery; D003919:Diabetes Insipidus / Q000209:etiology; D005260:Female; D005500:Follow-Up Studies; D005910:Glioma / Q000378:metabolism* / Q000532:radiotherapy / Q000601:surgery; D013006:Growth Hormone / Q000172:deficiency*; D006801:Humans; D007006:Hypogonadism / Q000209:etiology; D007029:Hypothalamic Neoplasms / Q000378:metabolism* / Q000532:radiotherapy / Q000601:surgery; D007037:Hypothyroidism / Q000209:etiology; D008297:Male; D017253:Neurofibromatoses / Q000378:metabolism; D009897:Optic Chiasm; D011629:Puberty, Precocious / Q000209:etiology; D012189:Retrospective Studies; D017741:Survivors
chemical_list: D000305:Adrenal Cortex Hormones; D013006:Growth Hormone