Atypical teratoid rhabdoid tumor: long-term survival after chemoradiotherapy.
OBJECTIVE
Atypical teratoid rhabdoid tumors (ATRTs) arise from the central nervous system largely in the pediatric population. They portend a very poor prognosis with few long-term survivors. We describe a series of five cases at our institution.


METHODS
We conducted a retrospective chart review and clinical follow-up.


RESULTS
Three patients underwent chemoradiation after surgical resection; the two patients whose caretakers declined this therapy passed away soon after diagnosis. Chemoradiation included intravenous and intrathecal chemotherapy as well as intensity-modulated radiotherapy after resection. Of the patients receiving chemoradiation, two patients had infratentorial tumors, two had gross residual tumor after resection, and two were under the age of 3 years. The three patients receiving trimodality therapy remain clinically and symptomatically disease-free with follow-up times of 44, 46, and 55 months. Two of the patients have mild neuropsychiatric sequelae after therapy.


CONCLUSIONS
Long-term, high-volume trials of ATRT are currently not published. We offer experience in successful long-term survival of this tumor treated with chemoradiotherapy.
Department of Radiation Oncology, University of Nebraska Medical Center, 987521 Nebraska Medical Center, Omaha, NE, USA, vivek333@gmail.com.
Vivek
V
Verma
Catrina P
CP
Johnson
Nathan R
NR
Bennion
Abhijeet R
AR
Bhirud
Sicong
S
Li
Rodney D
RD
McComb
Chi
C
Lin
D000293:Adolescent; D016543:Central Nervous System Neoplasms / Q000188:drug therapy*; D059248:Chemoradiotherapy / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D018572:Disease-Free Survival; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D012189:Retrospective Studies; D018335:Rhabdoid Tumor / Q000188:drug therapy*; D017741:Survivors; D013724:Teratoma / Q000188:drug therapy*