Multimodal treatments combined with gamma knife surgery for primary atypical teratoid/rhabdoid tumor of the central nervous system: a single-institute experience of 18 patients.
OBJECTIVES
Atypical teratoid/rhabdoid tumor (AT/RT) is a rare, highly malignant tumor of the central nervous system with poor prognosis. Nowadays, multimodal management, including surgery, chemotherapy (CMT), and radiation therapy (RT), is advocated. However, AT/RT treatment with gamma knife surgery (GKS) was rarely reported. The aim of this study was to assess the efficacy and safety of GKS for the treatment of AT/RT.


PATIENTS AND METHODS
Medical records of AT/RT patients who underwent surgery from 2007 to 2014 at the West China Hospital were retrospectively reviewed and statistically analyzed.


RESULTS
Eighteen patients (12 males and 6 females) were presented with AT/RTs. Median age during presentation was 20.5 months (range, 4-179 months). Twelve patients were < 3 years and six patients were > 3 years. Tumor location was supratentorial in seven patients, infratentorial in ten patients, and center area of the brain in one patient. Treatments performed were as follows: surgery alone in two patients, surgery+RT in two patients, surgery+CMT in five patients, surgery+CMT+RT in two patients, and surgery+CMT+RT+GKS in seven patients. The 2-year overall survival (OS) rate and event-free survival (EFS) rate for all 18 consecutive patients were 33.3 and 27.8%, respectively. Cox regression analyses showed that multimodal management combined with GKS was an independent positive prognostic factor for OS.


CONCLUSIONS
Although AT/RTs are lethal cancer types, the OS of the disease was improved by using multimodal therapeutic strategies, including surgery, CMT, and RT, combined with GKS.
['Ren|Yan-Ming|YM|', 'Wu|Xia|X|', 'You|Chao|C|', 'Zhang|Yue-Kang|YK|', 'Li|Qiang|Q|', 'Ju|Yan|Y|']
D000293:Adolescent D000970:Antineoplastic Agents D016543:Central Nervous System Neoplasms D002648:Child D002675:Child, Preschool D003131:Combined Modality Therapy D005260:Female D006801:Humans D007223:Infant D007633:Keratins D008297:Male D018396:Mucin-1 D009419:Nerve Tissue Proteins D016634:Radiosurgery D012189:Retrospective Studies D018335:Rhabdoid Tumor D016896:Treatment Outcome D014746:Vimentin
Gamma knife surgery Atypical teratoid/rhabdoid tumors Prognosis Chemotherapy Radiation therapy INI1