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

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

Full description

Saved in:

Bibliographic Details
Published in:	The journal of clinical endocrinology and metabolism Vol. 107; no. 3; pp. e1020 - e1031
Main Authors:	Zucchini, Stefano, Di Iorgi, Natascia, Pozzobon, Gabriella, Pedicelli, Stefania, Parpagnoli, Maria, Driul, Daniela, Matarazzo, Patrizia, Baronio, Federico, Crocco, Marco, Iudica, Giovanna, Partenope, Cristina, Nardini, Beatrice, Ubertini, Graziamaria, Menardi, Rachele, Guzzetti, Chiara, Iughetti, Lorenzo, Aversa, Tommaso, Di Mase, Raffaella, Cassio, Alessandra
Format:	Journal Article
Language:	English
Published:	United States Oxford University Press 17-02-2022
Subjects:	Age of Onset Child Child, Preschool Craniopharyngioma - complications Craniopharyngioma - diagnosis Craniopharyngioma - pathology Craniopharyngioma - therapy Female Follow-Up Studies Health aspects Human Growth Hormone - therapeutic use Humans Hypophysectomy - adverse effects Hypopituitarism - diagnosis Hypopituitarism - etiology Hypopituitarism - therapy Italy - epidemiology Male Medical research Medicin och hälsovetenskap Medicine, Experimental Neoplasm, Residual Neuropeptides Pediatrics Pituitary Gland - pathology Pituitary Gland - surgery Pituitary gland tumors Pituitary hormones Pituitary Neoplasms - complications Pituitary Neoplasms - diagnosis Pituitary Neoplasms - pathology Pituitary Neoplasms - therapy Postoperative Complications - epidemiology Postoperative Complications - etiology Recurrence Retrospective Studies Treatment Outcome Italy GH therapy intracranial tumor children hypothalamic obesity craniopharyngioma pituitary deficiency
Online Access:	Get full text
Tags:	Add Tag No Tags, Be the first to tag this record!

Description
Summary:	Nationwide data on children diagnosed with craniopharyngioma (CP) are not available in Italy. 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. 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. 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. 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.
Bibliography:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 content type line 23
ISSN:	0021-972X 1945-7197 1945-7197
DOI:	10.1210/clinem/dgab784