Brainstem Low-Grade Gliomas in Children-Excellent Outcomes With Multimodality Therapy.
Safe maximal surgical resection is the initial treatment of choice for pediatric brainstem low-grade gliomas. Optimal therapy for incompletely resected tumors or that progress after surgery is uncertain. We reviewed the clinical characteristics, therapy, and outcomes of all children with nontectal brainstem low-grade gliomas treated at the University of Michigan between 1993 and 2013. Median age at diagnosis was 6 years; histology was confirmed in 23 of 25 tumors, 64% were pilocytic astrocytoma. Nineteen patients underwent initial tumor resection; 14/19 received no upfront adjuvant therapy. Eight patients in the study had progressive disease; 5 initially resected tumors received chemotherapy at tumor relapse, all with partial or complete radiographic responses. Ten-year progression-free survival is 71% and overall survival, 100%. This single-institution retrospective study demonstrates excellent survival rates for children with brainstem low-grade gliomas. The efficacy of the well-tolerated chemotherapy in this series supports its role in the treatment of unresectable or progressive brainstem low-grade gliomas.
1 Department of Oncology, Division of Neuro-Oncology, St. Jude Children's Research Hospital, Memphis, TN, USA.
Santhosh A
SA
Upadhyaya
2 Department of Pediatrics, Division of Pediatric Hematology Oncology, C. S. Mott Children's Hospital, University of Michigan, Ann Arbor, MI, USA.
Carl
C
Koschmann
3 Department of Neurosurgery, C. S. Mott Children's Hospital, University of Michigan, Ann Arbor, MI, USA.
Karin
K
Muraszko
4 Department of Pathology, University of Michigan, Ann Arbor, MI, USA.
Sriram
S
Venneti
3 Department of Neurosurgery, C. S. Mott Children's Hospital, University of Michigan, Ann Arbor, MI, USA.
Hugh J
HJ
Garton
5 Texas Center for Proton Therapy, Irving, TX, USA.
Daniel A
DA
Hamstra
3 Department of Neurosurgery, C. S. Mott Children's Hospital, University of Michigan, Ann Arbor, MI, USA.
Cormac O
CO
Maher
4 Department of Pathology, University of Michigan, Ann Arbor, MI, USA.
Bryan L
BL
Betz
4 Department of Pathology, University of Michigan, Ann Arbor, MI, USA.
Noah A
NA
Brown
6 Department of Radiation Oncology, University of Michigan, Ann Arbor, MI, USA.
Daniel
D
Wahl
4 Department of Pathology, University of Michigan, Ann Arbor, MI, USA.
Helmut C
HC
Weigelin
4 Department of Pathology, University of Michigan, Ann Arbor, MI, USA.
Kathleen E
KE
DuRoss
4 Department of Pathology, University of Michigan, Ann Arbor, MI, USA.
Annette S
AS
Leonard
2 Department of Pediatrics, Division of Pediatric Hematology Oncology, C. S. Mott Children's Hospital, University of Michigan, Ann Arbor, MI, USA.|7 Departments of Pediatrics and Neurology, Division of Pediatric Neurology, C. S. Mott Children's Hospital, University of Michigan, Ann Arbor, MI, USA.
Patricia L
PL
Robertson
D000293:Adolescent; D020295:Brain Stem Neoplasms / Q000000981:diagnostic imaging / Q000235:genetics / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D003131:Combined Modality Therapy; D018572:Disease-Free Survival; D005260:Female; D005500:Follow-Up Studies; D005910:Glioma / Q000000981:diagnostic imaging / Q000235:genetics / Q000628:therapy*; D006801:Humans; D007223:Infant; D008297:Male; D048493:Proto-Oncogene Proteins B-raf / Q000235:genetics; D012189:Retrospective Studies; D016896:Treatment Outcome
C482119:BRAF protein, human; D048493:Proto-Oncogene Proteins B-raf
brainstem tumors; chemotherapy; low-grade glioma; overall survival; progressive tumors