Background: Childhood brain tumour survivors are at risk of developing endocrine secondary effects throughout lifetime. Transition between childhood and adult care is a critical moment during which patients may stop medical visits and treatments.
Objective: To describe endocrine care transition in our cohort of patients with primary brain tumors followed from 2010-2015, who are ≥=18 years old by December 31st 2017.
Methods: Retrospective and prospective observational study, data collection from medical records of patients seen at least once between 2010-2015. Patients with pituitary adenomas, untreated fortuitously diagnosed gliomas (NF1 context), who died before transition or with insufficient data were excluded.
Results: 74 patients were included (34 females), with a mean age of 21.7 years (17.1-29.9). Tumour types: medulloblastomas (37.8%), craniopharyngiomas (36.5%), gliomas (13.5%), dysgerminomas (5.4%), others (6.8%). Fifty-nine out of 74 (79.7%) have started transition, 9 (12.2%) are still in regular pediatric care; 2 have been considered as not needing adult endocrine follow-up, and 4 are lost to follow-up before starting transition. Forty-four out of 59 who started transition (74.5%) have been seen at La Pitié-Salpêtrière hospital or other adult centres in the last year.
Endocrine deficiencies: growth hormone deficiency (GHD): 89.2%, 57/66 diagnosed during childhood (43/57 within the first year of endocrine follow-up) and 9/66 after attaining adult height. At transition reevaluation, 60/66 have persistent GHD and 4/66 have a normal GH response (≥=15 mUI/l). TSHD or peripheral hypothyroidism concerns 71.6% of patients. Routine thyroid ultrasound was performed in 28 patients; 15 of them have thyroid nodules, including two cancers (5.5 and 11 years post radiotherapy). ACTHD affects 51.3% patients. Hypogonadotropic hypogonadism affects 43.2% patients, and gonadal insufficiency, 24.3%. Diabetes insipidus affects only suprasellar tumors (28/39). Mean final height is lower in patients with non suprasellar tumors: -0.8±1.3 SD compared to suprasellar: 0.1±1.4 (P=0.01), attributable to spinal radiotherapy. BMI is higher in patients with suprasellar tumors: 29.2±9.2 Kg/m2 (21% overweight, 39% obesity) than in non suprasellar: 22.6±3.9 Kg/m2 (14% overweight, 3% obesity), P<0.001. 27/57 reports a handicap (visual or neurological) that impairs their studies or work.
Conclusions: Transition is a critical time for patients with chronic diseases, including childhood brain tumor survivors. It is important to ensure an effective passage towards adult endocrine care, in order to maintain care continuity and prevent complications linked to non compliance to treatment. Our transition program between a pediatric and an adult hospital seems to contribute to this care continuum.
19 - 21 Sep 2019
European Society for Paediatric Endocrinology