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Multicenter Study
. 2022 Feb 17;107(3):e1020-e1031.
doi: 10.1210/clinem/dgab784.

Management of Childhood-onset Craniopharyngioma in Italy: A Multicenter, 7-Year Follow-up Study of 145 Patients

Stefano Zucchini  1 , Natascia Di Iorgi  2   3 , Gabriella Pozzobon  4 , Stefania Pedicelli  5 , Maria Parpagnoli  6 , Daniela Driul  7 , Patrizia Matarazzo  8 , Federico Baronio  1 , Marco Crocco  2   9 , Giovanna Iudica  2 , Cristina Partenope  4 , Beatrice Nardini  6 , Graziamaria Ubertini  5 , Rachele Menardi  10 , Chiara Guzzetti  11 , Lorenzo Iughetti  12 , Tommaso Aversa  13 , Raffaella Di Mase  14 , Alessandra Cassio  1 ; Physiopathology of Growth Processes and Puberty Study Group of the Italian Society for Pediatric Endocrinology and Diabetology
Collaborators, Affiliations
Multicenter Study

Management of Childhood-onset Craniopharyngioma in Italy: A Multicenter, 7-Year Follow-up Study of 145 Patients

Stefano Zucchini et al. J Clin Endocrinol Metab. .

Abstract

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

Objective: This work aimed to identify patients' characteristics, type of surgical approach, complications and recurrences, number of pituitary deficits, and number of patients starting growth hormone (GH) treatment.

Methods: A retrospective multicenter collection took place of 145 patients aged 0 to 18 years who underwent surgery for CP between 2000 and 2018, and 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 transcranial (TC) in 67.5% of cases and transsphenoidal (TS) in 31.%. The TC approach was prevalent in all age groups. Postsurgery complications occurred in 53% of cases, with water-electrolyte disturbances most frequent. Radiotherapy was used in 39% of cases. All patients but one presented with at least one hormone pituitary deficiency, with thyrotropin deficiency most frequent (98.3%), followed by adrenocorticotropin (96.8%), arginine vasopressin (91.1%), and GH (77.4%). Body mass index (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.

Conclusion: CP is often diagnosed late in Italy, with TC more frequent than the TS surgical approach. Postsurgery complications were not rare, and hypopituitarism developed almost in all cases. BMI shows a tendency to increase overtime.

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

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