Management of childhood-onset craniopharyngioma in Italy: A multicenter seven year follow-up study of 145 patients

J Clin Endocrinol Metab. 2021 Oct 29;dgab784. doi: 10.1210/clinem/dgab784. Online ahead of print.


Context: Nationwide data on children diagnosed with craniopharyngioma (CP) are not available in Italy.

Objective: Identify patients' characteristics, type of surgical approach, complications and recurrences, number of pituitary deficits and number of patients starting GH treatment.

Methods: Retrospective multicenter collection of 145 patients aged 0-18 years underwenting surgery for CP between 2000-2018, followed-up in 17 Italian centers of Pediatric Endocrinology.

Results: Age at diagnosis was 8.4±4.1 years. Duration of symptoms was 10.8±12.5 months and headache was most frequent (54%), followed by impaired growth (48%) and visual disturbances (44%). Most lesions were suprasellar (85%) and histology was adamantinomatous in all cases but two. Surgical approach was transcranic (TC) in 67.5% of cases and transphenoidal (TS) in 31.%. TC approach was prevalent in all age groups. Post-surgery complications occurred in 53% of cases with water-electrolyte disturbances most frequent. Radiotherapy was used in 39% of cases. All patients but one presented at least one hormone pituitary deficiency, with TSH deficit as most frequent (98.3%), followed by ACTH (96.8%), AVP (91.1%) and GH (77.4%). BMI significantly increased over time. A hypothalamic disturbance was present in 55% of cases. GH therapy was started during follow-up in 112 patients at a mean age of 10.6 years and 54 developed a recurrence or regrowth of the residual lesion.

Conclusions: CP is often diagnosed late also in Italy with TC more frequent than TS surgical approach. Post-surgery complications were not rare and hypopituitarism developed almost in all cases. BMI shows a tendency to increase overtime.

Keywords: Craniopharyngioma; GH therapy; children; hypothalamic obesity; intracranial tumor; pituitary deficiency.