Background: The Craniopharyngioma service in our centre has changed since 2005 with a standardised preoperative assessment and a new approach to surgery and use of radiotherapy. We review the subsequent outcomes.
Treatment strategy: Preoperative endocrinology: prior to dexamethasone therapy: standard dose short synacthen test (SST), thyroid function test (TFT), IGF1, LH, FSH, testosterone/oestradiol±GH stimulation test. Staging: MRI and tumour staging (Paris classification). Cystic lesions: endoscopic decompression (ED)+repeat MRI+reclassification. Surgery: total (TR), near-total (NTR) or subtotal tumour resection (STR) determined by Paris stage. Radiotherapy: elective for residual disease in older children. Postoperative endocrinology: 57 days following dexamethasone: 0900 h cortisol+ACTH, low dose SST, TFT. 36 months: anterior pituitary function tests.
Results: Data from 23 subjects (10 M), age (median and range) 10.9 years (1.515.5) are reported. Treatment: ED: (n=13) seven tumours downgraded following ED. Surgery: TR 5/23, NTR 5/23, STR 11/23, awaiting surgery 2/23. Radiotherapy: 9/20 (four following tumour progression/recurrence). Auxology: baseline (n=22): height SDS, 0.89±1.55; BMI SDS, 1.46±2.53. Greater than equal to 6 months (n=18): height SDS, 0.71±1.51; BMI SDS, 1.61±1.91. Endocrinology (patients tested/patients abnormal results): i) baseline: SST 19/8, TFT 23/3, IGF1 19/10, GH 2/2, LH, FSH 15/13, diabetes insipidus (DI) 24/0. ii) Follow-up: SST 17/12, TFT 17/17, IGF1 15/10, GH 16/15, LH, FSH 17/12, DI 21/17, panhypopituitarism 17/10.
Conclusions: Pituitary hormone deficiencies are common at presentation and progress following treatment. ED led to downgrading of some tumours. This enabled more extensive resection in 50% of patients, and reduced use of radiotherapy, but short-term outcome data are comparable with previous studies.
09 - 11 Nov 2011
British Society for Paediatric Endocrinology and Diabetes