CNS germinoma: Disease control and long-term functional outcome for 12 children treated with craniospinal irradiation

Purpose: To provide evidence that radiation therapy alone in the form of cr aniospinal irradiation (CSI) and a boost to the primary site of disease pro vides effective disease control and limited additional morbidity for patien ts with CNS germinoma. Methods and Materials: Twelve patients with a median age of It rears (range 9-16 Sears) with CNS germinoma were treated with CSI (median 25.6 Gy, rang e 23.4-32 Gy) and a boost to the primary site of disease (50.4 Gy, range 45 -54 Gy) between January 1987 and June 1998, All patients were biopsied prio r to radiation therapy and none received chemotherapy, No patients were los t to follow-up and the majority had long-term (> 45 month) pre- and postirr adiation endocrine and psychology assessment. Results: All 12 patients are alive and no failures have occurred with a med ian follow-up of 69 months (range 14-143 months). Preirradiation endocrine deficiencies were present in 6 of 6 suprasellar tumors and 1 of 6 pineal tu mors; with follow-up there was no substantial difference between age and ge nder adjusted pre- and postirradiation stature and weight. With long-term f ollow-up, there were no significant differences between pre- and postirradi ation full-scale, verbal, and performance IQ scores. Conclusions: This study confirms the ability of radiation therapy alone to achieve disease control with a high rate of success in pediatric patients a nd demonstrates that the treatment toxicity faced by these patients may be less than anticipated. Because these patients present with substantial pree xisting morbidity at diagnosis and may be of an age where the potential for radiation-related side effects is relatively small, the superiority of tre atment alternatives may be difficult to prove. (C) 2000 Elsevier Science In c.