Craniopharyngioma is a rare, embryonic malformation of the sellar/parasellar region with low histological grade. In childhood-onset craniopharyngioma (CoCR), endocrine dysfunctions, severe obesity and metabolic syndrome (MetS), neurological impairment and reduced quality of life have been described as consequences of both localization and treatment. To characterize a population with CoCR and to correlate endocrine/metabolic sequelae with different surgery approach we performed a retrospective longitudinal study on a single-centre cohort of 66 children (36 males) with CoCR, followed from 1990 to 2017. Clinical evaluations were performed yearly. Age at diagnosis (7.24±4.92 yrs), established in 34/66 patients, was not statistical different between sex. Clinical presentation was heterogeneous: neurological symptoms (55.2%), growth retardation (13.8%), diabetes insipidus (DI) (13.8%), pubertal disorders (6.9%) and other symptoms (10.3%); in 1 case severe obesity was the unique presentation symptom. Only patients with pubertal disorders were older than others. Surgery technique was defined for 32 patients: in 90.6% tumour was removed (by craniotomy in 82.8% and by transsphenoidal/transventricular approach in 17.2%); partial resection was found in 85.7%. Patients who underwent craniotomy were significant younger (P=0.009), probably reflecting the easier feasibility of this technique. Pituitary deficiencies developed in majority of cases after diagnosis: hypothyroidism in 92.9%, central adrenal insufficiency (cAI) in 91%, DI in 78.6%, growth hormone deficiency in 74.5% and hypogonadism in 73.1%. The first disorders that appeared were hypothyroidism, cAI and DI. Only DI was correlated with surgery approach, with a significant prevalence in craniotomy technique (P=0.001), even in case of partial resection. Obesity at the end of follow-up showed no correlation with surgery approach/extension. For 8 patients (4 males) we had data about body mass index (BMI) both at diagnosis and at the end of follow-up: all patients had normal BMI at diagnosis, but 2 patients (1 male) were obese at the last visit. BMI SDS at last control was significant correlated with BMI SDS at diagnosis; no significant differences were found in age at diagnosis, duration of follow-up, surgical approach/extension. In our cohort of patients with a long follow-up, cAI was present in >90%, indicating the need of frequent assessment of adrenal axis function to avoid adrenal crisis. We also found that BMI during follow-up was related to BMI at diagnosis. We suggest that in every patient, especially in those with initial overweight/obesity, criteria of MetS (waist circumference, blood pressure, glucose and lipid profile) should be strictly evaluated.
27 - 29 Sep 2018
European Society for Paediatric Endocrinology