Atypical teratoid rhabdoid tumors of the posterior fossa in children.
OBJECTIVE
Atypical teratoid rhabdoid tumors (AT/RT) are rare, aggressive, central nervous system neoplasms that typically affect children under 3 years of age and have a very poor prognosis. Early case series consistently demonstrated rapid recurrence with progression to death, but more recent experience has shown significant improvements in progression free and overall survival.


METHODS
A retrospective analysis of the clinical data of children diagnosed with AT/RT at the Ann & Robert H. Lurie Children's Hospital of Chicago (formerly Children's Memorial Hospital) between 2000 and 2014 was performed. Overall survival (OS) was used to describe outcome. Our small sample size and the utilization of different adjuvant regimens over the study period precluded a detailed statistical analysis.


RESULTS
Eight children with AT/RT of the posterior fossa were included in our report. Gross total resection (GTR) was achieved in five children (63 %), two children underwent subtotal resection (25 %), and there was one who underwent biopsy. Patients were treated with various combinations of chemotherapy with or without conformal radiation therapy (RT). Median overall survival was 5 months (range 1 to 107 months) with two patients achieving sustained responses to 45 and 107 months.


CONCLUSIONS
Our experience is in line with prior reports that show that children diagnosed with AT/RT of the posterior fossa have a poor prognosis, but that long-term survival is possible. These tumors provide many challenges, but contemporary series are beginning to show improvements in survival.
Department of Neurological Surgery, Northwestern University, Feinberg School of Medicine, Chicago, IL, USA. adipatri@luriechildrens.org.|Ann & Robert H. Lurie Children's Hospital of Chicago Chicago, Division of Pediatric Neurosurgery, 225 E. Chicago Avenue, Box 28, Chicago, IL, 60611-2605, USA. adipatri@luriechildrens.org.
Arthur J
AJ
DiPatri
Department of Neurological Surgery, Northwestern University, Feinberg School of Medicine, Chicago, IL, USA. ssredni@luriechildrens.org.|Ann & Robert H. Lurie Children's Hospital of Chicago Chicago, Division of Pediatric Neurosurgery, 225 E. Chicago Avenue, Box 28, Chicago, IL, 60611-2605, USA. ssredni@luriechildrens.org.|Cancer Biology and Epigenomics Program, Stanley Manne Children's Research Institute, Chicago, IL, USA. ssredni@luriechildrens.org.
Simone Treiger
ST
Sredni
Ann & Robert H. Lurie Children's Hospital of Chicago Chicago, Division of Pediatric Neurosurgery, 225 E. Chicago Avenue, Box 28, Chicago, IL, 60611-2605, USA. ggrahovac@luriechildrens.org.
Gordan
G
Grahovac
Department of Neurological Surgery, Northwestern University, Feinberg School of Medicine, Chicago, IL, USA. ttomita@luriechildrens.org.|Ann & Robert H. Lurie Children's Hospital of Chicago Chicago, Division of Pediatric Neurosurgery, 225 E. Chicago Avenue, Box 28, Chicago, IL, 60611-2605, USA. ttomita@luriechildrens.org.
Tadanori
T
Tomita
D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D015192:Infratentorial Neoplasms / Q000401:mortality / Q000628:therapy*; D008297:Male; D012189:Retrospective Studies; D018335:Rhabdoid Tumor / Q000401:mortality / Q000628:therapy*; D016019:Survival Analysis; D013724:Teratoma / Q000401:mortality / Q000628:therapy*; D055815:Young Adult
AT/RT; Atypical teratoid rhabdoid tumor; Brain tumor; Child/children; Posterior fossa; Surgery