Turkish Neurosurgery 2019 , Vol 29 , Num 5
Survival Benefit with Gross Total Resection and Adjuvant Radiotherapy in Childhood Atypical Teratoid/Rhabdoid Tumors: Results of a Single-Center Cohort of 27 Cases
1Hacettepe University, Faculty of Medicine, Department of Neurosurgery, Ankara, Turkey
2Health Sciences University, Diskapi Yildirim Beyazit Research and Training Hospital, Neurosrugery Clinic, Ankara, Turkey
3Siirt State Hospital, Neurosurgery Clinic, Siirt, Turkey
4Van Training and Research Hospital, Neurosurgery Clinic, Van, Turkey
DOI : 10.5137/1019-5149.JTN.25406-18.1 AIM: To share a single center experience with 27 atypical teratoid/rhabdoid tumor (AT/RT) cases, and to determine the effect of gross total tumor resection and other clinical characteristics on the overall survival rate of AT/RT.

MATERIAL and METHODS: We included 27 patients—with a histopathologically confirmed primary intracranial childhood AT/ RT—who were operated in our clinic between January 2000 and December 2017. Age, sex, tumor location, disseminated disease, the presence of hydrocephalus, symptom duration till diagnosis, the extent of resection, and adjuvant radiotherapy were evaluated for their influence on overall survival.

RESULTS: Median age at diagnosis for 27 patients was 19.1 months (7.2 months-5 years). Gross total resection was possible in 13 (48.72%) patients. Except for three patients who died of perioperative complications, all patients received chemotherapy and 11 received radiotherapy. In univariate analysis, male sex, older age at diagnosis (≥24 months), gross total resection, and radiotherapy were associated with overall longer survival; however, radiotherapy remained the only significant parameter in multivariate analysis.

CONCLUSION: AT/RT is a rare and dreadful brain tumor that has low survival rates despite contemporary treatment. Radiotherapy seems to prolong survival; however, large-scale studies are needed to establish prognostic factors. Keywords : Brain neoplasms, Childhood, Observational study, Rhabdoid tumor

Corresponding author : Ilkay ISIKAY, isikay@hacettepe.edu.tr