MANAGEMENT OF RECURRENT CRANIOPHARYNGIOMA

Although histologically benign, craniopharyngioma can regrow either fr om macroscopic remnants of the tumour left behind at operation, or eve n after an apparently gross total removal. Recurrence rates vary signi ficantly in the literature, depending on the efficacy of surgical trea tment and also on the growth potential of the tumour itself. The main factor influencing tumour regrowth is obviously the extent of surgical resection, as total removal carries a much lesser risk of recurrence compared to subtotal or partial resections (although in such cases rad iation therapy can lower this risk significantly). Other factors invol ved are the duration of follow-up and patient's age at operation, as c hildren tend to relapse more frequently than adults. Even in the ''mic rosurgery'' era, characterized by high percentages of total resections , recurrences remain high and continue to represent a major problem of craniopharyngioma treatment. Twenty-seven children and adolescents we re operated on for craniopharyngioma at the Department of Neurosurgery , Section of Pediatric Neurosurgery, Catholic University Medical Schoo l, Pome, between June 1985 and June 1997. Total tumour resection was a chieved in 18 cases, subtotal in 7 and partial in 2 instances. One pat ient died post-operatively. Post-operative neuroradiological investiga tions confirmed the operative findings, although 3 children with an ap parently gross total removal showed a residual nonenhancing calcium fl eck adherent to the hypothalamus (which remained stable at the followi ng examinations). Three of the 9 patients with less than total removal underwent post-operative radiation therapy. Out of the 26 surviving p atients 6 presented a recurrence of their craniopharyngioma, 2 after a n apparently gross total removal and 4 after a subtotal or partial res ection (one of them had received radiation therapy). The diagnosis was merely neuroradiological in 5 cases, as only one child presented a cl inical picture suggestive of tumour regrowth. Surgery was the first th erapeutic option in all the cases. Total tumour resection was accompli shed in 3 cases, subtotal in 2 and partial in the last one. One child died post-operatively. Four of the 5 survivors received radiation ther apy. All the patients are presently alive and stable (mean follow-up: 5.6 yrs). The authors conclude that surgery should be the first therap eutic option in case of recurrent craniopharyngioma and that radiation therapy should also be considered but only as adjuvant therapy.