Introduction: Medulloblastoma is the commonest paediatric brain tumour and accounts for 2030% of all brain tumours in the first decade of life. Improvements in treatment strategies have enhanced long-term survival resulting in an increased risk of late sequelae.
Aim: Review the prevalence of endocrine dysfunction in survivors of medulloblastoma at a single centre.
Methods: Case note review of patients treated for medulloblastoma between 1982 and 2002.
Results: Twenty-nine patients (22 males) were identified. Mean (±S.D.) age at diagnosis was 7.7 (±4.1) years. All patients received radiotherapy and 76% also received chemotherapy. All except one patient had surgery. Mean dose of craniospinal irradiation and posterior fossa boost were 33.5 (±3.6) and 24 (±10) gray respectively. All except one patient received posterior fossa boost. All patients developed pituitary hormone insufficiency. The median duration from end of treatment to loss of GH was 1.65 years, loss of ACTH was 3.0 years and thyroid hormone deficiency was 3.75 years. All except one patient developed GH deficiency (96.5%) both clinically (growth failure, fatigue) and biochemically. Primary hypothyroidism was detected in 44.8% (n=13). Similar to previous reports, secondary hypothyroidism was rare. ACTH deficiency was detected in 31% (n=9). All patients with ACTH deficiency also had GH and thyroid hormone deficiency except one patient, who had received the lowest posterior fossa boost of 18 gray and had no surgical intervention. Forty-one percent (67% male) received GnRH analogues to optimize growth, but no patients developed gonadotrophin deficiency.
Conclusion: There is a high prevalence of endocrine dysfunction in medulloblastoma survivors. Figures from our unit for GH and ACTH insufficiency are higher than those published in the literature despite comparable radiation doses. Effective multidisciplinary management in a joint clinic ensures that all patients are closely monitored and hormone deficiencies detected and treated early to maximize growth and wellbeing.
20 - 22 Sep 2014
European Society for Paediatric Endocrinology