ESPE Abstracts (2018) 89 P-P1-196

aService D’Endocrinologie, Diabétologie et Gynécologie Pédiatriques, Hôpital Universitaire Necker -- Enfants Malades, Paris, France; bESPE -- Clinical Fellowship, Paris, France; cCentre De référence des Maladies Endocriniennes Rares de la Croissance (CRMERC), Paris, France; dUniversité Paris Descartes, Paris, France; eInstitut Gustave Roussy, Villejuif, France; fService de Neurochirurgie, Hôpital Universitaire Necker-Enfants Malades, Paris, France; gService d’Endocrinologie et Médecine de la Reproduction, Hôpital Universitaie La Ptié-Salpêtrière, Paris, France; hInstitut Curie, Paris, France; iCHU Montpellier, Hôpital Gui de Chauliac, Unité de Neurochirurgie Pédiatrique, Montpellier, France; jHôpital Femme Mère Enfant - HCL, Lyon, France; kService d’Endocrinologie Pédiatrique, Hôpital Universitaire Trousseau, Paris, France. lService d’Endocrinologie et DIabétologie Pédiatrique, CH André Mignot, Le Chesnay, France; mSIREDO Oncology Center (Care, Innovon and Research for Children ad AYA Cancer), Paris, France; nPSL Research University, Paris, France; oCentre de référence des Maladies Endocriniennes Rares de la Croissance (CRMERC), Villejuif, France; pInstitut Imagine (Rare Genetic diseases) Affiliate, Paris, France

Background: Brain tumours are the most frequent solid tumours during childhood. Many of these patients develop endocrine disorders.

Objective: To describe our cohort of patients with primary brain tumours, followed in the Pediatric Endocrinology Unit at Hôpital Universitaire Necker–Enfants Malades, Paris, France between 2010–2015, to assess current practice and propose recommendations.

Methods: Retrospective and prospective observational study, data collection from medical records of patients seen at least once between 2010 and 2015. Patients with pituitary adenomas, untreated fortuitously diagnosed gliomas (NF1 context) or insufficient data were excluded.

Results: 228 patients were included, 49.5% females, mean age at diagnosis: 7.1±3.9, mean follow-up time: 5.9±3.7 years. Main tumour subtypes: medulloblastoma (36.8%), craniopharyngioma (28.5%), glioma (20.6%), dysgerminoma (4%). Patients were divided into two groups: suprasellar (SS: 48.3%), involving the sellar/suprasellar region, hypothalamus or optic pathways, and non-suprasellar tumours (NSS: 51.7%), mainly involving the posterior fossa. Initial height was similar between both groups (SS: −0.3±1.6SDS Vs NSS: −0.1±1.2SDS), but BMI was significantly higher in SS (+0.8±2.0SDS, 20% >+2DS: obese) than in NSS (−0.2±1.3SDS, 5.1% obese, P<0.0001). Treatment was surgery (SS: 83.6%, NSS: 94.1%), and/or radiotherapy (SS: 58.2%, NSS: 95.8%) and/or chemotherapy (SS: 35.5%, NSS: 76.3%). GH deficiency was similar in both groups (SS: 86.8%, NSS: 83.9%), hypothyroidism was more common in SS (69%, all with TSH deficiency), than in NSS (33.9%, 2/3 TSH deficiency), as well as cortisol deficiency (SS: 69%, NSS: 4.2%) and diabetes insipidus (SS: 61.8%, NSS: 0.9%). Precocious and early puberty was noted in 16.4% SS and 11% NSS. Hypogonatropic hypogonadism was predominant in SS (47%, Vs 0.9% of NSS), and gonadal insufficiency in NSS (37.4%, Vs 1.2% of SS). Final height was available for 93 patients, with a significant difference between both groups (SS: −0.3±1.4SDS, NSS: −1.0±1.3SDS, P<0.0001). NSS had a significantly lower final height compared to initial height (P<0.0001) and to target height (-1.1 SDS P<0.0001), attributable to craniospinal radiotherapy (P=0.020). BMI increased at final visit in both groups (P=0.0001), with obesity in 46.4% of SS and 16.9% of NSS. Thyroid nodules were found in 11/45 patients, including 2 cancers.

Conclusions: This large cohort shows a high incidence of early endocrine disorders. An endocrine follow-up should be mandatory for all patients with a history of brain tumour, including nutritional evaluation.

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