The descriptive epidemiology of atypical teratoid/rhabdoid tumors in the United States, 2001-2010.
BACKGROUND
Atypical teratoid/rhabdoid tumor is a rare malignant CNS tumor that most often affects children ≤ 3 years old. The Central Brain Tumor Registry of the United States contains the largest aggregation of population-based incidence data for primary CNS tumors in the US. Its data were used to describe the incidence, associated trends, and relative survival after diagnosis of atypical teratoid/rhabdoid tumor.


METHODS
Using data from 50 cancer registries between 2001 and 2010, age-adjusted incidence rates per 100 000 and 95% CIs were calculated by sex, race, Hispanic ethnicity, age at diagnosis, and location of tumor in the CNS for children aged 0 to 19 years. Relative survival rates and 95% CIs were also calculated.


RESULTS
The average annual age-adjusted incidence rate was 0.07 (95% CI: 0.07, 0.08). Incidence rates did not significantly vary by sex, race, or ethnicity. Age had a strong effect on incidence rate, with highest incidence among children <1 year, and decreasing incidence with increasing age. The 6-month, 1-year, and 5-year relative survival rates for all ages were 65.0%, 46.8%, and 28.3%, respectively. Atypical teratoid/rhabdoid tumor can occur anywhere in the CNS, but supratentorial tumors were more common with increasing age.


CONCLUSIONS
We confirm differences in survival by age at diagnosis, treatment pattern, and location of tumor in the brain. This contributes to our understanding of these tumors and may stimulate research leading to improved treatment of this devastating childhood disease.
Case Comprehensive Cancer Center, Case Western Reserve University School of Medicine, Cleveland, Ohio (Q.T.O., Y.C., H.G., Y.W., J.S.B.-S.); Central Brain Tumor Registry of the United States, Hinsdale, Illinois (Q.T.O., Y.C., H.G., Y.W., C.K., J.S.B.-S.); Division of Pediatric Hematology-Oncology, Department of Pediatrics, Case Western Reserve University School of Medicine, Cleveland, Ohio (P.M.d.B.); The Ohio State University, Columbus, Ohio (A.O.); Case Western Reserve University, Cleveland, Ohio (P.F.); Department of Pathology, University Hospitals Case Medical Center, Cleveland, Ohio (M.L.C.); Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Emory University Hospital, Atlanta, Georgia (D.J.B.).
Quinn T
QT
Ostrom
Case Comprehensive Cancer Center, Case Western Reserve University School of Medicine, Cleveland, Ohio (Q.T.O., Y.C., H.G., Y.W., J.S.B.-S.); Central Brain Tumor Registry of the United States, Hinsdale, Illinois (Q.T.O., Y.C., H.G., Y.W., C.K., J.S.B.-S.); Division of Pediatric Hematology-Oncology, Department of Pediatrics, Case Western Reserve University School of Medicine, Cleveland, Ohio (P.M.d.B.); The Ohio State University, Columbus, Ohio (A.O.); Case Western Reserve University, Cleveland, Ohio (P.F.); Department of Pathology, University Hospitals Case Medical Center, Cleveland, Ohio (M.L.C.); Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Emory University Hospital, Atlanta, Georgia (D.J.B.).
Yanwen
Y
Chen
Case Comprehensive Cancer Center, Case Western Reserve University School of Medicine, Cleveland, Ohio (Q.T.O., Y.C., H.G., Y.W., J.S.B.-S.); Central Brain Tumor Registry of the United States, Hinsdale, Illinois (Q.T.O., Y.C., H.G., Y.W., C.K., J.S.B.-S.); Division of Pediatric Hematology-Oncology, Department of Pediatrics, Case Western Reserve University School of Medicine, Cleveland, Ohio (P.M.d.B.); The Ohio State University, Columbus, Ohio (A.O.); Case Western Reserve University, Cleveland, Ohio (P.F.); Department of Pathology, University Hospitals Case Medical Center, Cleveland, Ohio (M.L.C.); Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Emory University Hospital, Atlanta, Georgia (D.J.B.).
Peter
P
M de Blank
Case Comprehensive Cancer Center, Case Western Reserve University School of Medicine, Cleveland, Ohio (Q.T.O., Y.C., H.G., Y.W., J.S.B.-S.); Central Brain Tumor Registry of the United States, Hinsdale, Illinois (Q.T.O., Y.C., H.G., Y.W., C.K., J.S.B.-S.); Division of Pediatric Hematology-Oncology, Department of Pediatrics, Case Western Reserve University School of Medicine, Cleveland, Ohio (P.M.d.B.); The Ohio State University, Columbus, Ohio (A.O.); Case Western Reserve University, Cleveland, Ohio (P.F.); Department of Pathology, University Hospitals Case Medical Center, Cleveland, Ohio (M.L.C.); Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Emory University Hospital, Atlanta, Georgia (D.J.B.).
Annie
A
Ondracek
Case Comprehensive Cancer Center, Case Western Reserve University School of Medicine, Cleveland, Ohio (Q.T.O., Y.C., H.G., Y.W., J.S.B.-S.); Central Brain Tumor Registry of the United States, Hinsdale, Illinois (Q.T.O., Y.C., H.G., Y.W., C.K., J.S.B.-S.); Division of Pediatric Hematology-Oncology, Department of Pediatrics, Case Western Reserve University School of Medicine, Cleveland, Ohio (P.M.d.B.); The Ohio State University, Columbus, Ohio (A.O.); Case Western Reserve University, Cleveland, Ohio (P.F.); Department of Pathology, University Hospitals Case Medical Center, Cleveland, Ohio (M.L.C.); Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Emory University Hospital, Atlanta, Georgia (D.J.B.).
Paul
P
Farah
Case Comprehensive Cancer Center, Case Western Reserve University School of Medicine, Cleveland, Ohio (Q.T.O., Y.C., H.G., Y.W., J.S.B.-S.); Central Brain Tumor Registry of the United States, Hinsdale, Illinois (Q.T.O., Y.C., H.G., Y.W., C.K., J.S.B.-S.); Division of Pediatric Hematology-Oncology, Department of Pediatrics, Case Western Reserve University School of Medicine, Cleveland, Ohio (P.M.d.B.); The Ohio State University, Columbus, Ohio (A.O.); Case Western Reserve University, Cleveland, Ohio (P.F.); Department of Pathology, University Hospitals Case Medical Center, Cleveland, Ohio (M.L.C.); Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Emory University Hospital, Atlanta, Georgia (D.J.B.).
Haley
H
Gittleman
Case Comprehensive Cancer Center, Case Western Reserve University School of Medicine, Cleveland, Ohio (Q.T.O., Y.C., H.G., Y.W., J.S.B.-S.); Central Brain Tumor Registry of the United States, Hinsdale, Illinois (Q.T.O., Y.C., H.G., Y.W., C.K., J.S.B.-S.); Division of Pediatric Hematology-Oncology, Department of Pediatrics, Case Western Reserve University School of Medicine, Cleveland, Ohio (P.M.d.B.); The Ohio State University, Columbus, Ohio (A.O.); Case Western Reserve University, Cleveland, Ohio (P.F.); Department of Pathology, University Hospitals Case Medical Center, Cleveland, Ohio (M.L.C.); Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Emory University Hospital, Atlanta, Georgia (D.J.B.).
Yingli
Y
Wolinsky
Case Comprehensive Cancer Center, Case Western Reserve University School of Medicine, Cleveland, Ohio (Q.T.O., Y.C., H.G., Y.W., J.S.B.-S.); Central Brain Tumor Registry of the United States, Hinsdale, Illinois (Q.T.O., Y.C., H.G., Y.W., C.K., J.S.B.-S.); Division of Pediatric Hematology-Oncology, Department of Pediatrics, Case Western Reserve University School of Medicine, Cleveland, Ohio (P.M.d.B.); The Ohio State University, Columbus, Ohio (A.O.); Case Western Reserve University, Cleveland, Ohio (P.F.); Department of Pathology, University Hospitals Case Medical Center, Cleveland, Ohio (M.L.C.); Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Emory University Hospital, Atlanta, Georgia (D.J.B.).
Carol
C
Kruchko
Case Comprehensive Cancer Center, Case Western Reserve University School of Medicine, Cleveland, Ohio (Q.T.O., Y.C., H.G., Y.W., J.S.B.-S.); Central Brain Tumor Registry of the United States, Hinsdale, Illinois (Q.T.O., Y.C., H.G., Y.W., C.K., J.S.B.-S.); Division of Pediatric Hematology-Oncology, Department of Pediatrics, Case Western Reserve University School of Medicine, Cleveland, Ohio (P.M.d.B.); The Ohio State University, Columbus, Ohio (A.O.); Case Western Reserve University, Cleveland, Ohio (P.F.); Department of Pathology, University Hospitals Case Medical Center, Cleveland, Ohio (M.L.C.); Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Emory University Hospital, Atlanta, Georgia (D.J.B.).
Mark L
ML
Cohen
Case Comprehensive Cancer Center, Case Western Reserve University School of Medicine, Cleveland, Ohio (Q.T.O., Y.C., H.G., Y.W., J.S.B.-S.); Central Brain Tumor Registry of the United States, Hinsdale, Illinois (Q.T.O., Y.C., H.G., Y.W., C.K., J.S.B.-S.); Division of Pediatric Hematology-Oncology, Department of Pediatrics, Case Western Reserve University School of Medicine, Cleveland, Ohio (P.M.d.B.); The Ohio State University, Columbus, Ohio (A.O.); Case Western Reserve University, Cleveland, Ohio (P.F.); Department of Pathology, University Hospitals Case Medical Center, Cleveland, Ohio (M.L.C.); Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Emory University Hospital, Atlanta, Georgia (D.J.B.).
Daniel J
DJ
Brat
Case Comprehensive Cancer Center, Case Western Reserve University School of Medicine, Cleveland, Ohio (Q.T.O., Y.C., H.G., Y.W., J.S.B.-S.); Central Brain Tumor Registry of the United States, Hinsdale, Illinois (Q.T.O., Y.C., H.G., Y.W., C.K., J.S.B.-S.); Division of Pediatric Hematology-Oncology, Department of Pediatrics, Case Western Reserve University School of Medicine, Cleveland, Ohio (P.M.d.B.); The Ohio State University, Columbus, Ohio (A.O.); Case Western Reserve University, Cleveland, Ohio (P.F.); Department of Pathology, University Hospitals Case Medical Center, Cleveland, Ohio (M.L.C.); Department of Pathology and Laboratory Medicine, Emory University School of Medicine, Emory University Hospital, Atlanta, Georgia (D.J.B.).
Jill S
JS
Barnholtz-Sloan
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D001932:Brain Neoplasms / Q000453:epidemiology* / Q000401:mortality; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D012042:Registries; D018335:Rhabdoid Tumor / Q000453:epidemiology* / Q000401:mortality; D016019:Survival Analysis; D013724:Teratoma / Q000453:epidemiology* / Q000401:mortality; D014481:United States / Q000453:epidemiology; D055815:Young Adult
atypical teratoid/rhabdoid tumors; brain tumors; incidence; pediatric brain tumors; survival