Radical resection of craniopharyngioma.
BACKGROUND
The best management of craniopharyngioma in children remains a controversial topic among neurosurgeons. The two treatments for craniopharyngioma most commonly discussed in the literature are primary total resection and limited resection followed by radiotherapy. Without ignoring the challenging behavior of these tumors, we strongly believe that the first approach in a child with a craniopharyngioma is to attempt total removal. Trying to remove a craniopharyngioma that has been treated previously with other methods is, in our experience, much more dangerous because of adherences of the tumor to vascular and neural structures.


METHODS
Between 1988 and 2004, we operated on 153 patients with craniapharyngioma (40% female and 60% male), whose ages at the time of surgery ranged from 15 days to 21 years (mean 10.5 years). Eighty-seven percent of the patients were found to have some visual disturbance and 42% endocrinological alterations. Fifty-four percent of the patients presented hydrocephalus, but only 18% had shunting. Gross total removal was attempted in all patients. Among the 153 patients, the tumor was prechiasmatic in 35 and retrochiasmatic in 112; in ten, these were considered giant forms, and eight had a posterior fossa extension. We performed 84 single and 69 combined approaches.


RESULTS
We achieved total removal in 69% of our patients. None of our patients regarded as having undergone total tumor resection disclosed recurrence after a follow-up of 1-16 years. Radiation therapy was administered in children with subtotal removal. All children underwent total removal, but only 62% of those who underwent subtotal removal had good outcomes. After surgery, endocrinological status worsened in almost all patients, but visual status improved markedly.


CONCLUSIONS
The treatment of choice in craniopharyngioma in childhood is total resection in order to avoid radiation therapy and recurrence. When total resection is not possible, subtotal resection plus radiation therapy is the alternative.
Zuccaro
Graciela
G
Department of Neurosurgery, Hospital Nacional de Pediatria Juan P. Garrahan, Buenos Aires, Argentina. gnzuccaro@fibertel.com.ar
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D003397:Craniopharyngioma / Q000628:therapy*; D004522:Educational Status; D004700:Endocrine System Diseases / Q000209:etiology; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D006849:Hydrocephalus / Q000209:etiology; D007016:Hypophysectomy / Q000379:methods*; D007223:Infant; D007231:Infant, Newborn; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D010910:Pituitary Irradiation; D010911:Pituitary Neoplasms / Q000628:therapy*; D011183:Postoperative Complications; D012189:Retrospective Studies; D014057:Tomography, X-Ray Computed / Q000379:methods; D016896:Treatment Outcome