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.

Management of Childhood-onset Craniopharyngioma in Italy: A Multicenter, 7-Year Follow-up Study of 145 Patients / Zucchini, S.; Di Iorgi, N.; Pozzobon, G.; Pedicelli, S.; Parpagnoli, M.; Driul, D.; Matarazzo, P.; Baronio, F.; Crocco, M.; Iudica, G.; Partenope, C.; Nardini, B.; Ubertini, G.; Menardi, R.; Guzzetti, C.; Iughetti, L.; Aversa, T.; Di Mase, R.; Cassio, A.; Cianfarani, S.; Maghnie, M.; Tuli, G.; Loche, S.; Bruzzi, P.; Wasniewska, M.; Salerno, M.; Rutigliano, I.; Iezzi, M. L.; Cherubini, V.; Grandone, A.; Faienza, M. F.; Tumini, S.; Baldoli, C.; Consales, A.; Genitori, L.; Marras, C. E.; Milanaccio, C.; Mortini, P.; Vindigni, M.; Zenga, F.; Zucchelli, M.. - In: THE JOURNAL OF CLINICAL ENDOCRINOLOGY AND METABOLISM. - ISSN 0021-972X. - 107:3(2022), pp. E1020-E1031. [10.1210/clinem/dgab784]

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

Iughetti L.;Bruzzi P.;Tumini S.;
2022

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.
2022
107
3
E1020
E1031
Management of Childhood-onset Craniopharyngioma in Italy: A Multicenter, 7-Year Follow-up Study of 145 Patients / Zucchini, S.; Di Iorgi, N.; Pozzobon, G.; Pedicelli, S.; Parpagnoli, M.; Driul, D.; Matarazzo, P.; Baronio, F.; Crocco, M.; Iudica, G.; Partenope, C.; Nardini, B.; Ubertini, G.; Menardi, R.; Guzzetti, C.; Iughetti, L.; Aversa, T.; Di Mase, R.; Cassio, A.; Cianfarani, S.; Maghnie, M.; Tuli, G.; Loche, S.; Bruzzi, P.; Wasniewska, M.; Salerno, M.; Rutigliano, I.; Iezzi, M. L.; Cherubini, V.; Grandone, A.; Faienza, M. F.; Tumini, S.; Baldoli, C.; Consales, A.; Genitori, L.; Marras, C. E.; Milanaccio, C.; Mortini, P.; Vindigni, M.; Zenga, F.; Zucchelli, M.. - In: THE JOURNAL OF CLINICAL ENDOCRINOLOGY AND METABOLISM. - ISSN 0021-972X. - 107:3(2022), pp. E1020-E1031. [10.1210/clinem/dgab784]
Zucchini, S.; Di Iorgi, N.; Pozzobon, G.; Pedicelli, S.; Parpagnoli, M.; Driul, D.; Matarazzo, P.; Baronio, F.; Crocco, M.; Iudica, G.; Partenope, C.;...espandi
File in questo prodotto:
File Dimensione Formato  
dgab784.pdf

Accesso riservato

Tipologia: Versione pubblicata dall'editore
Dimensione 672.13 kB
Formato Adobe PDF
672.13 kB Adobe PDF   Visualizza/Apri   Richiedi una copia
Pubblicazioni consigliate

Licenza Creative Commons
I metadati presenti in IRIS UNIMORE sono rilasciati con licenza Creative Commons CC0 1.0 Universal, mentre i file delle pubblicazioni sono rilasciati con licenza Attribuzione 4.0 Internazionale (CC BY 4.0), salvo diversa indicazione.
In caso di violazione di copyright, contattare Supporto Iris

Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11380/1280787
Citazioni
  • ???jsp.display-item.citation.pmc??? 4
  • Scopus 11
  • ???jsp.display-item.citation.isi??? 9
social impact