Practical approach to childhood craniopharyngioma: a role of an endocrinologist and a general paediatrician.

ABSTRACT

OBJECTIVES: The objectives of this study are to evaluate co-morbidities in patients with craniopharyngioma and to elaborate an interdisciplinary protocol of the follow-up. PATIENTS AND METHODS: The group comprised 15 children (median age at the diagnosis, 10.1; mean follow-up period, 4 years). All patients had surgical resection of the tumour gross total in seven, subtotal or partial removal in eight cases. Surgery was followed by radiotherapy in ten cases for tumour residue or progression. Sexual development and auxology were evaluated at diagnosis and during follow-up. Hormones were determined by chemiluminescent immunometric assays. Antidiuretic hormone dysfunction was diagnosed on the grounds of clinical symptoms, water-electrolyte balance, urine specific gravity, and serum osmolality. Metabolic control was monitored by levels of glucose, insulin, lipids, and transaminases; insulin resistance was expressed by homeostatic model assessment (HOMA) index. RESULTS: At diagnosis, median height standard deviation score (hSDS) was -1.6 (five children being short-statured). Median change hSDS for the whole follow-up was 1.2 (four children decelerating growth). Diabetes insipidus was diagnosed in eight (within 0-1.8 years of the follow-up), hypocorticolism in eight, and hypothyroidism in 12 subjects (within 0-3.75 years for both endocrinopathies). Four patients required sex hormone replacement therapy. At diagnosis, five children were overweight; during follow-up, only four children sustained normal body mass index. Hypertransaminasaemia was found in three, dyslipidaemia in 11, and hyperinsulinaemia in seven patients (with elevated HOMA in four cases). CONCLUSIONS: On the grounds of these observations, the management of craniopharyngioma in our institution includes repeated hormonal and metabolic assays in chosen time intervals. Early detection of co-morbidities and their management involves interdisciplinary team.