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Management of Childhood-onset Craniopharyngioma in Italy: A Multicenter, 7-Year Follow-up Study of 145 Patients
- Source :
- The Journal of Clinical Endocrinology & Metabolism. 107:e1020-e1031
- Publication Year :
- 2021
- Publisher :
- The Endocrine Society, 2021.
-
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.
- Subjects :
- Male
Pediatrics
Neoplasm, Residual
Endocrinology, Diabetes and Metabolism
medicine.medical_treatment
Clinical Biochemistry
Hypopituitarism
Biochemistry
Craniopharyngioma
Postoperative Complications
Endocrinology
Pituitary deficiency
Recurrence
Age of Onset
Child
Human Growth Hormone
Follow up studies
Treatment Outcome
Italy
Child, Preschool
Residual
Pituitary Gland
Female
medicine.symptom
craniopharyngioma
GH therapy
children
hypothalamic obesity
intracranial tumor
pituitary deficiency
Follow-Up Studies
Humans
Hypophysectomy
Pituitary Neoplasms
Retrospective Studies
medicine.medical_specialty
Pediatric endocrinology
Context (language use)
Lesion
Internal medicine
medicine
Preschool
business.industry
Biochemistry (medical)
medicine.disease
Radiation therapy
Neoplasm
business
Subjects
Details
- ISSN :
- 19457197 and 0021972X
- Volume :
- 107
- Database :
- OpenAIRE
- Journal :
- The Journal of Clinical Endocrinology & Metabolism
- Accession number :
- edsair.doi.dedup.....7b5e90dab1ede08c7aa5dad17288a2be