ENDOCRINE OUTCOME IN LONG-TERM SURVIVORS OF LOW-GRADE HYPOTHALAMIC CHIASMATIC GLIOMA/

OBJECTIVE We have evaluated the frequency of endocrine abnormalities i n a large group of patients with hypothalamic/chlasmatic glioma (H/CG) and its correlation with the different forms of therapy. DESIGN Descr iptive retrospective study using case note review analysis. PATIENTS T he records of 68 children who survived H/CG were analysed, One third h ad neurofibromatosis. The mean age at tumour presentation was 5 years. The median time of follow-up was 3.6 years. Thirty-eight children rec eived cranial radiation, of whom 17 also had surgery. Surgery was perf ormed in a total of 24 patients, Fifteen patients received only chemot herapy. Eight children, all with neurofibromatosis, received no specif ic tumour treatment. MEASUREMENTS 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 45 Gy grew norm ally. Precocious puberty was diagnosed in 11 patients. Nine patients, all treated with cranial irradiation, developed hypogonadotrophic hypo gonadism, Of the 14 patients with hypothyroidism, 10 had surgery (P<0. 005). Hypoadrenalism and diabetes insipidus each occurred in eight pat ients, and were associated with multiple endocrine deficiencies and su rgery. Endocrine deficiencies occurred in children with neurofibromato sis as frequently as children without neurofibromatosis but only when comparing those treated with cranial irradiation or surgery. CONCLUSIO NS Nearly all studies assessing the endocrine outcome after tumour the rapy evaluate patients with different tumour types. This study investi gates a specific and large population of patients with H/CG and correl ates the different forms of treatment with the endocrine outcome. Prec ocious puberty, in children with this tumour, is probably due to tumou r location rather than oncological therapy. Conversely, although endoc rine 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 dysfuncti on occurs less often in neurofibromatosis patients treated conservativ ely. Furthermore, this study documents that a significant number of yo ung children grew normally despite receiving brain irradiation of grea ter than 45 Gy.