Case Report

Journal of Neuro-Oncology

, Volume 103, Issue 3, pp 797-801

First online:

mTORC1 activation in childhood ependymoma and response to sirolimus

  • Daniel C. BowersAffiliated withDepartment of Pediatrics, University of Texas Southwestern Medical School at DallasSimmons Comprehensive Cancer Center, University of Texas Southwestern Medical School at DallasDepartment of Neuro-Oncology, Center for Cancer and Blood Disorders, Children’s Medical Center – DallasDivision of Pediatric Hematology-Oncology, University of Texas Southwestern Medical School at Dallas Email author 
  • , Blanka KucejovaAffiliated withDepartment of Internal Medicine, University of Texas Southwestern Medical School at DallasDepartment of Developmental Biology, University of Texas Southwestern Medical School at DallasSimmons Comprehensive Cancer Center, University of Texas Southwestern Medical School at Dallas
  • , Linda MargrafAffiliated withDepartment of Pathology, University of Texas Southwestern Medical School at Dallas
  • , Lynn GarganAffiliated withDepartment of Neuro-Oncology, Center for Cancer and Blood Disorders, Children’s Medical Center – Dallas
  • , James BrugarolasAffiliated withDepartment of Developmental Biology, University of Texas Southwestern Medical School at DallasSimmons Comprehensive Cancer Center, University of Texas Southwestern Medical School at DallasDepartment of Pathology, University of Texas Southwestern Medical School at Dallas

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Abstract

Recurrent ependymomas are considered rarely responsive to chemotherapy and often have a dismal prognosis after tumor progression. Below is a brief report of a 6 year old child with a multiply progressive ependymoma whose tumor had a near complete response to sirolimus that was durable for 18 months. Immunohistochemistry for phosphorylated S6, which has been reported to be associated with tumor sensitivity to mTORC1 inhibitors, was positive in this patient’s tumor.

Keywords

Ependymoma Sirolimus Rapamycin mTORC1