Background: In paediatric CNS tumours, germ cell neoplasms usually produce endocrine disorders helping an early diagnosis.
Objective and hypotheses: To describe presenting symptoms in paediatric CNS germ cell tumours and endocrine abnormalities on follow-up.
Methods: We reviewed the records of children and adolescents aged under 14 who were followed in our unit presenting a CNS germ cell tumour. Endocrine abnormalities at diagnosis and over the follow-up period were recorded.
Results: We studied 11 patients (ten female), aged at diagnosis 7.012.0 years. Hypothalamic origin was the most frequent (10/11) and chorionic gonadotropin was secreted in five cases. Reasons of consultation were neuro-ophthalmic signs/symptoms in eight cases and endocrine complaints only in three. Endocrine disorders, not leading to consultate, were also the presenting symptoms in other six cases. Seven children referred diabetes insipidus beginning 25 years before tumour diagnosis. Anterior hypopituitarism developed in all the hypothalamic lesions immediately after starting oncologic treatment.
Conclusions: The management of paediatric germ cell tumours involves a multidisciplinary effort. Endocrine disorders usually occurred long time before neurological and ophthalmological symptoms, so identifying them may help to earlier diagnosis. Hormonal evaluation is mandatory as well on follow-up.
01 - 03 Oct 2015
European Society for Paediatric Endocrinology