ORIGINAL ARTICLES   

Minerva Pediatrica 2007 June;59(3):219-31

Copyright © 2007 EDIZIONI MINERVA MEDICA

language: Italian

Pediatric craniopharyngioma: long-term results in 61 cases

Pancucci G., Massimi L., Caldarelli M., D’Angelo L., Sturiale C., Tamburrini G., Tufo T., Di Rocco C.

Unità di Neurochirurgia Infantile Università Cattolica del Sacro Cuore, Roma

Aim. The aim of this study was to analyze the long-term results of the surgical management of craniopharyngioma in children by reviewing a series of patients consecutively treated in a single institution, and to assess the efficacy of intratumoral chemotherapy with interferon-a.
Methods. Sixty-one paediatric patients (38 males and 23 females; mean age: 8 years) have been surgically treated in the last 20 years. The goal of surgery was to remove the tumour as much as possible. Postoperative radiotherapy was administered only to children aged more than 4 year old and with postsurgical evolutive remnant. Three patients were treated with intratumoral administration of interferon-a. The mean follow-up is 8.4 years (median: 7 years).
Results. All the 55 surviving patients enjoy a normal social life, except for 3 of them (visual and/or neurological deficits); endocrine function, compromised in 3/5 of cases, is managed by chronic hormone replacement; neuropsychological assessment is satisfactory in almost all the cases. Although obesity does not seem to be an important social limit, it represents a real management problem. Interferon-a was useful in transitorily arresting the growing cystic craniopharyngiomas.
Conclusion. The current experience confirms the still remarkable challenges in the treatment of craniopharyngioma in childhood. Surgery should aim not only at removing the tumour, but also at preserving the late neuro-endocrinological functions. Intracystic chemo-therapy with interferon-a might represent an effective option to postpone the surgical operation until the maturation of the hypotalamic-hypophyseal pathway is completed. However, it can not replace the traditional surgical management.