Craniopharyngiomas in Children

Abstract

Background: The modern era of pediatric craniopharyngioma treatment includes multiple modalities including microsurgical resection, irradiation, brachytherapy or chemotherapy. No clear consensus as to the best therapeutic approach has yet been established. The aim of this study was to describe the techniques and strategies for the treatment of pediatric craniopharyngiomas in light of a literature review with particular attention to the incidence of adverse postoperative effects. Methods: Twenty-seven pediatric patients (median age 9.0 years) who were surgically treated for craniopharyngiomas were evaluated. We reviewed the recent literature for clinical features of craniopharyngiomas in children, including the present cases. Results: The overall rate of radiographically complete resection of our cases was 92.6%. In the literature we reviewed, the rate of gross total resection was 33–91% (average 57.8%). According to the literature, recurrence rates range from zero to 52.8% (average 16.1%) in the gross total resection group, 51.1% in the subtotal resection without radiation therapy group, and 33.5% in the subtotal resection with radiation therapy group. In six of our most recent cases, for whom we have complete outcome analysis, radiographically complete resection is 100% with only one recurrence so far over 4 years. Conclusions: For patients in whom early postoperative MRI reveals complete craniopharyngioma removal, a very low rate of recurrence is anticipated. In the authors’ experience, radiographically total excision of even large craniopharyngiomas can be safely achieved by one or a combination of several advanced microsurgical techniques, sometimes by a staged strategy. Major morbidities can be avoided, although moderate morbidities occur and require management.

Surgical management of giant pediatric craniopharyngiomas

“…While there was no operative mortality in our series, the OS rate (69%) was lower than the reported rate of longterm survival of over 90% in the modern era 3,14,15,17,22,23,36,42,49,51,52,58,59,63,69,77,84,95 and significantly worse than the OS rate in the 60 children with smaller tumors in our series (91%). However, subgroup analysis revealed most of the difference was due to the extremely poor survival rate in children with previously treated (recurrent) giant tumors (33%).…”

Surgical treatment of ectopic recurrence of craniopharyngioma

“…Only 1 patient required RT for multiple lesions, and GTR was successful in providing disease control in 22 patients (71%), similar to reported rates of GTR for suprasellar tumors. 5,8,10,12,13,16,22,26,27,30,37,38,44,55,56,62,63 At the time of surgery in our series, there was an excellent arachnoidal plane with preservation of the pia mater; pathological investigation confirmed the absence of brain invasion in all specimens. Total extirpation was accomplished in all of our patients without new permanent neurological deficit, and no further recurrences developed during long-term follow-up.…”

“…5,8,10,12,13,16,22,26,27,37,38,44,50,55,56,60,62,63 Due to the benign nature of the epithelial rests that comprise craniopharyngiomas on histopathological studies, tumor recurrence usually occurs locally at the primary site or in regions immediately adjacent. Remote recurrence following surgery is reported as a rare phenomenon, and only 28 cases have been reported in the literature (Table 4).…”

“…39,45,46,58,60 Despite their benign histopathological characteristics, their clinical course is often rather malignant, and recurrence of craniopharyngiomas is one of the most common complications of treatment, occurring in 0-57% of patients following complete resection. 5,8,10,12,13,16,17,22,26,27,30,37,38,44,50,55,56,60,62,63 Although most recurrences occur locally-in or adjacent to the suprasellar cistern-ectopic recurrence of craniopharyngioma can occur in the presence or absence of suprasellar disease. Two common patterns of ectopia are along the surgical tract 4,7,8,18,19,23,25,[33][34][35]43,47,55 and dissemination via the CSF into the cortical subarachnoid, 3,6,7,15,21,24,29,30,42,…”

Successful surgical treatment of craniopharyngioma in very young children

“…The debate centers around those advocating radical resection for surgical cure, 9,16,20,30,35,44,49,57,86,87,90,95,95 those recommending attempted GTR only if hypothalamic morbidity is acceptably low, 1,12,15,21,29,58,61,69,74,77 and those offering limited resection with adjuvant ra diotherapy. 7,28,32,47,50 Both treatment strategies-radical re section and limited resection combined with irradiationyield similar rates of overall survival.…”