CS-05 EXTRA-PARENCHYMAL (PERIPHERAL) ATYPICAL TERATOID / RHABDOID TUMORS

AT/RT is a malignant embryonal tumor reported by Rorke in 1996. Authors reported first AT/RT in Japan in 1998. This tumor entity was included as new malignant embryonal tumor in WHO 2000, and tumors of Japanese patients has been reported more than 60 cases in the past. This AT/RT is a tumor in the brain parenchyma that a medulloblastoma and PNET and the possibility that it has been misdiagnosed have had pointed out. On the other hand, it is reported that there is the type that we should call peripheral AT/RT which rarely occurs in extra-parenchyma. We want to propose that there is such special tumor group. In the results, age: 6 infants were main (2nd - 14 years old after birth). tumor location: 6 sellar region, 3 civuses, 2 petrous bone or cerebellum and 2 conexities. treatment: duration of survival significantly improved all macroscopic tumor resection by the operation, but, in small pontine part AT/RT, an outcome tended to be poor. On the other hand, in AT/RT which occurred in the sellar region, all cases adult woman tended to have good prognosis. It is necessary for AT/RT (central AT/RT) in the brain to recognize that there is extra-parenchymal AT/RT (peripheral AT/RT) tumor which we reported this time which came to be recognized widely.