Pineal tumors in children and adolescents. Treatment by CSF shunting and radiotherapy.

Tumors of the pineal region account for 3% to 8% of pediatric intracranial tumors. The treatment of such tumors has been in a state of flux between conservative therapy (cerebrospinal fluid shunting and radiotherapy) and direct surgical removal. A brief history and review of the literature with analysis of both approaches is given, and the Mayo Clinic's experience with conservative treatment of tumors in the pineal region in patients 20 years old and younger (27 cases) is studied and analyzed. The series comprises 21 boys and six girls, with an age range of 1 to 20 years (mean 13.7 years). Follow-up examinations are complete and range from 1 to 24 years, with a mean follow-up period of 7.8 years. The median survival time for these patients treated with shunt and radiotherapy is 17.7 years. There was no mortality from treatment and complications were rare. The details of the clinical presentation, diagnostic findings, pathology, therapy, recurrence, and survival are presented. All patients under 6 years of age (six cases) had recurrences, 50% in other areas in the brain and 50% in the spinal cord, perhaps pointing to the need for whole-brain and spinal-axis irradiation in patients in this age group. The results of this study of the conservative approach form a standard against which results of any other type of therapy may be compared.