Craniopharyngioma in children

Craniopharyngioma of childhood is a different entity than the tumor found in adulthood. Microscopically adamantinomata tissue is found in children whereas a squamous epithelial origin is more predominant in adults. Partial resection is of little benefit in children because adamantinomous tumors grow at a fast rate. Confidence in a prebiopsy diagnosis of craniopharyngioma provided by modern diagnostic imaging allows the surgeon to choose either radical surgical resection or limited surgery combined with radiotherapy. As aggressive surgery followed by radiotherapy compounds the deleterious effects of the two treatments without any added benefit, this course of action should be avoided when possible. The neurosurgeon should be clear in making a recommendation and plan of action to patients and families. Radical surgery is recommended by the vast majority of pediatric neurosurgeons at academic centers. The long-term survival achieved following radical surgery performed by a highly experienced surgeon may approach the results achieved by limited resection and radiotherapy, but the quality of life issue is left unresolved. Unfortunately the children in series reporting the results of aggressive surgery have not had the benefit of detailed psychologic, IQ, and endocrinologic testing. The major question regarding quality of life with limited resection plus radiotherapy regards the delayed effects of irradiation. Are the complications cumulative with time? The only series with long-term follow-up reporting both treatments overwhelmingly favors limited resection followed by irradiation. This polarization of opinion by the experts needs to be resolved. A true multi-institutional, cooperative study is needed. Unfortunately the bias of potential participants prohibits a randomized study at this time. It is hoped that a prospective, natural history study with detailed psychologic and physiologic testing will give a clearer evaluation of the outcome and standard of care in this country.