Craniopharyngioma in children: Marseille experience

The management of craniopharyngioma in children represents a challenging problem. If radical excision is recommended by many authors as the initial treatment, in some cases, particularly in recurrent tumours, other methods (gamma knife surgery and intracystic bleomycin) can be very useful. Even if craniopharyngioma is a benign tumour, recurrences are frequent, and the aim of our study was to analyse our results, to try to determine some prognostic factors of recurrences and to discuss about a new strategy concerning the initial management of these tumours.

Forty-seven children with craniopharyngioma were treated in the Department of Pediatric Neurosurgery. All of the patients, but five children treated by intracystic bleomycin, underwent a surgical resection of the tumour as initial treatment with the goal of achieving gross total removal (GTR) of the tumour. Two children had radiotherapy and gamma knife treatment, respectively, following surgery for a tumoural residue. All the children had a magnetic resonance imaging (MRI) study 3 months after surgery to evaluate the results of the initial treatment. Using statistical analysis, some prognostic factors (age, sex, location, aspect, size of the tumour and result of the first MRI) have been studied.

Forty-two children were operated on, but one died in the immediate postoperative period from a major stroke due to carotid spasm. GTR, defined as the absence of residue on the first MRI control, was achieved in 27 children (65.8%), but 7 patients (25.9%) presented recurrence. Subtotal removal (STR) was obtained in 14 children (34.2%), but 9 patients (64.3%) developed a recurrence defined as the growth of the residual tumour with or without clinical symptoms. Five children having a small- or moderate-size cystic craniopharyngioma were treated using one-stage (three cases) or two-stage (two cases) intracystic bleomycin and any presented recurrence. All the prognostic factors studied, except one (presence of a residue on the first MRI control), do not have a statistical significance.

Craniopharyngioma in children remains a formidable tumour, and regardless of whatever progress made in their management, the incidence of recurrences is still elevated and severe sequelae can be observed. There are no prognostic factors among those studied concerning the recurrences of these tumours except the quality of the exeresis confirmed by the first postoperative MRI.