Background: Pediatric Central Nervous System (CNS) neoplasms are the most frequent solid tumors in children. Since the increase in survival, the patients are in high risk of developing long term sequelae. Endocrinological sequelae may be due to the oncological disease itself but usually derived from the treatment received, and they affect 2050% of patients long-term. We aimed to review our experience from 2005 to 2015.
Objective and hypotheses: Describe the actual situation/outcomes in our tertiary unit.
Method: An observational retrospective study of clinical diagnosis of oncological disease and subsequent monitoring endocrinological data; collected both at diagnosis and follow-up, including anthropometric variables, oncological treatment received and endocrinological pathology diagnosed. Statistical analysis SPSS22.
Results: We studied 103 patients (48% female) diagnosed at the age of 6.8±4.2 years. The most frequent anatomical location was posterior (48%) followed by midline fossa (35%). The most prevalent histological types were astrocytomas (29%), medulloblastoma/PNET (17%) and Craniopharyngiomas (7%). Global mortality was 42%. Subgroup of survivors (n=59): The age at diagnosis was 7.6±4.5 (vs. 5.8±3.6; P=0.036), the complete surgical resection was 67% (vs. 29%; P=0.03), and the posterior fossa involvement was 40% (vs. 60%; P=0.03). Survival was higher in astrocytomas and craniopharyngiomas. Chemotherapy and radiotherapy were given in 39% and 29%, respectively. 23% of survivors developed transient postoperative neurohypophyseal deficiency (SIADH, diabetes insipidus or both). Permanent sequelae subgroup (n=17/59): The most frequent endocrine sequel was Panhypopituitarism (64%). The axes involved in descending order were: thyroid, GH, ACTH, ADH and FSH/LH.
Conclusion: In our review, permanent endocrinological deficits were mostly influenced by location and surgery in craniopharyngiomas. Almost one-third develop short-term endocrine pathology. Panhypopituitarism incidence is high. Increased risk tumors are craneofaringiomas and meduloblastomas.
10 - 12 Sep 2016
European Society for Paediatric Endocrinology