title: Long-term outcome of patients with spinal myxopapillary ependymoma: treatment results from the MD Anderson Cancer Center and institutions from the Rare Cancer Network.
abstract: BACKGROUND
  Spinal myxopapillary ependymomas (MPEs) are slowly growing ependymal gliomas with preferential manifestation in young adults. The aim of this study was to assess the outcome of patients with MPE treated with surgery, radiotherapy (RT), and/or chemotherapy.
  
  
  METHODS
  The medical records of 183 MPE patients (male: 59%) treated at the MD Anderson Cancer Center and 11 institutions from the Rare Cancer Network were retrospectively reviewed. Mean patient' age at diagnosis was 35.5 ± 15.8 years. Ninety-seven (53.0%) patients underwent surgery without RT, and 86 (47.0%) were treated with surgery and/or RT. Median RT dose was 50.4 Gy. Median follow-up was 83.9 months.
  
  
  RESULTS
  Fifteen (8.2%) patients died, 7 of unrelated cause. The estimated 10-year overall survival was 92.4% (95% CI: 87.7-97.1). Treatment failure was observed in 58 (31.7%) patients. Local failure, distant spinal relapse, and brain failure were observed in 49 (26.8%), 17 (9.3%), and 11 (6.0%) patients, respectively. The estimated 10-year progression-free survival was 61.2% (95% CI: 52.8-69.6). Age (<36 vs ≥36 y), treatment modality (surgery alone vs surgery and RT), and extent of surgery were prognostic factors for local control and progression-free survival on univariate and multivariate analysis.
  
  
  CONCLUSIONS
  In this series, treatment failure of MPE occurred in approximately one third of patients. The observed recurrence pattern of primary spinal MPE was mainly local, but a substantial number of patients failed nonlocally. Younger patients and those not treated initially with adjuvant RT or not undergoing gross total resection were significantly more likely to present with tumor recurrence/progression.
authors.affiliation: Center of Proton Therapy, Paul Scherrer Institute, Villigen/Würenlingen, Switzerland (D.C.W.); Department of Radiation Oncology, MD Anderson Cancer Center, Houston, Texas (Y.W., J.L.); Department of Radiation Oncology, Mayo Clinic, Rochester, Minnesota (R.M.); Department of Radiation Oncology, Catalan Institute of Oncology, Badalona, Spain (S.V.); Department of Oncology and Radiotherapy, Medical University of Gdansk, Gdansk, Poland (R.Z.); Department of Radiation Oncology, Inselspital, Bern, Switzerland (A.P.); Department of Radiation Oncology, Dr Bernard Verbeeten Instituut, Tilburg, the Netherlands (P.P.); Department of Radiation Oncology, Ege University Medical School, Izmir, Turkey (Y.A.); Department of Radiation Oncology, Faculty of Medicine, Hacettepe University, Ankara, Turkey (G.O.); Department of Robert-Janker-Klinik in Germany, Bonn, Germany (B.B.); Department of Clinical Epidemiology Unit, Department of Community Medicine, Geneva University Hospital, Geneva, Switzerland (G.H.); Department of Medicine I & Comprehensive Cancer Center, Medical University of Vienna, Vienna, Austria (M.P.).
authors.forename: Damien C
authors.initials: DC
authors.lastname: Weber
authors.affiliation: Center of Proton Therapy, Paul Scherrer Institute, Villigen/Würenlingen, Switzerland (D.C.W.); Department of Radiation Oncology, MD Anderson Cancer Center, Houston, Texas (Y.W., J.L.); Department of Radiation Oncology, Mayo Clinic, Rochester, Minnesota (R.M.); Department of Radiation Oncology, Catalan Institute of Oncology, Badalona, Spain (S.V.); Department of Oncology and Radiotherapy, Medical University of Gdansk, Gdansk, Poland (R.Z.); Department of Radiation Oncology, Inselspital, Bern, Switzerland (A.P.); Department of Radiation Oncology, Dr Bernard Verbeeten Instituut, Tilburg, the Netherlands (P.P.); Department of Radiation Oncology, Ege University Medical School, Izmir, Turkey (Y.A.); Department of Radiation Oncology, Faculty of Medicine, Hacettepe University, Ankara, Turkey (G.O.); Department of Robert-Janker-Klinik in Germany, Bonn, Germany (B.B.); Department of Clinical Epidemiology Unit, Department of Community Medicine, Geneva University Hospital, Geneva, Switzerland (G.H.); Department of Medicine I & Comprehensive Cancer Center, Medical University of Vienna, Vienna, Austria (M.P.).
authors.forename: Yucai
authors.initials: Y
authors.lastname: Wang
authors.affiliation: Center of Proton Therapy, Paul Scherrer Institute, Villigen/Würenlingen, Switzerland (D.C.W.); Department of Radiation Oncology, MD Anderson Cancer Center, Houston, Texas (Y.W., J.L.); Department of Radiation Oncology, Mayo Clinic, Rochester, Minnesota (R.M.); Department of Radiation Oncology, Catalan Institute of Oncology, Badalona, Spain (S.V.); Department of Oncology and Radiotherapy, Medical University of Gdansk, Gdansk, Poland (R.Z.); Department of Radiation Oncology, Inselspital, Bern, Switzerland (A.P.); Department of Radiation Oncology, Dr Bernard Verbeeten Instituut, Tilburg, the Netherlands (P.P.); Department of Radiation Oncology, Ege University Medical School, Izmir, Turkey (Y.A.); Department of Radiation Oncology, Faculty of Medicine, Hacettepe University, Ankara, Turkey (G.O.); Department of Robert-Janker-Klinik in Germany, Bonn, Germany (B.B.); Department of Clinical Epidemiology Unit, Department of Community Medicine, Geneva University Hospital, Geneva, Switzerland (G.H.); Department of Medicine I & Comprehensive Cancer Center, Medical University of Vienna, Vienna, Austria (M.P.).
authors.forename: Robert
authors.initials: R
authors.lastname: Miller
authors.affiliation: Center of Proton Therapy, Paul Scherrer Institute, Villigen/Würenlingen, Switzerland (D.C.W.); Department of Radiation Oncology, MD Anderson Cancer Center, Houston, Texas (Y.W., J.L.); Department of Radiation Oncology, Mayo Clinic, Rochester, Minnesota (R.M.); Department of Radiation Oncology, Catalan Institute of Oncology, Badalona, Spain (S.V.); Department of Oncology and Radiotherapy, Medical University of Gdansk, Gdansk, Poland (R.Z.); Department of Radiation Oncology, Inselspital, Bern, Switzerland (A.P.); Department of Radiation Oncology, Dr Bernard Verbeeten Instituut, Tilburg, the Netherlands (P.P.); Department of Radiation Oncology, Ege University Medical School, Izmir, Turkey (Y.A.); Department of Radiation Oncology, Faculty of Medicine, Hacettepe University, Ankara, Turkey (G.O.); Department of Robert-Janker-Klinik in Germany, Bonn, Germany (B.B.); Department of Clinical Epidemiology Unit, Department of Community Medicine, Geneva University Hospital, Geneva, Switzerland (G.H.); Department of Medicine I & Comprehensive Cancer Center, Medical University of Vienna, Vienna, Austria (M.P.).
authors.forename: Salvador
authors.initials: S
authors.lastname: Villà
authors.affiliation: Center of Proton Therapy, Paul Scherrer Institute, Villigen/Würenlingen, Switzerland (D.C.W.); Department of Radiation Oncology, MD Anderson Cancer Center, Houston, Texas (Y.W., J.L.); Department of Radiation Oncology, Mayo Clinic, Rochester, Minnesota (R.M.); Department of Radiation Oncology, Catalan Institute of Oncology, Badalona, Spain (S.V.); Department of Oncology and Radiotherapy, Medical University of Gdansk, Gdansk, Poland (R.Z.); Department of Radiation Oncology, Inselspital, Bern, Switzerland (A.P.); Department of Radiation Oncology, Dr Bernard Verbeeten Instituut, Tilburg, the Netherlands (P.P.); Department of Radiation Oncology, Ege University Medical School, Izmir, Turkey (Y.A.); Department of Radiation Oncology, Faculty of Medicine, Hacettepe University, Ankara, Turkey (G.O.); Department of Robert-Janker-Klinik in Germany, Bonn, Germany (B.B.); Department of Clinical Epidemiology Unit, Department of Community Medicine, Geneva University Hospital, Geneva, Switzerland (G.H.); Department of Medicine I & Comprehensive Cancer Center, Medical University of Vienna, Vienna, Austria (M.P.).
authors.forename: Renata
authors.initials: R
authors.lastname: Zaucha
authors.affiliation: Center of Proton Therapy, Paul Scherrer Institute, Villigen/Würenlingen, Switzerland (D.C.W.); Department of Radiation Oncology, MD Anderson Cancer Center, Houston, Texas (Y.W., J.L.); Department of Radiation Oncology, Mayo Clinic, Rochester, Minnesota (R.M.); Department of Radiation Oncology, Catalan Institute of Oncology, Badalona, Spain (S.V.); Department of Oncology and Radiotherapy, Medical University of Gdansk, Gdansk, Poland (R.Z.); Department of Radiation Oncology, Inselspital, Bern, Switzerland (A.P.); Department of Radiation Oncology, Dr Bernard Verbeeten Instituut, Tilburg, the Netherlands (P.P.); Department of Radiation Oncology, Ege University Medical School, Izmir, Turkey (Y.A.); Department of Radiation Oncology, Faculty of Medicine, Hacettepe University, Ankara, Turkey (G.O.); Department of Robert-Janker-Klinik in Germany, Bonn, Germany (B.B.); Department of Clinical Epidemiology Unit, Department of Community Medicine, Geneva University Hospital, Geneva, Switzerland (G.H.); Department of Medicine I & Comprehensive Cancer Center, Medical University of Vienna, Vienna, Austria (M.P.).
authors.forename: Alessia
authors.initials: A
authors.lastname: Pica
authors.affiliation: Center of Proton Therapy, Paul Scherrer Institute, Villigen/Würenlingen, Switzerland (D.C.W.); Department of Radiation Oncology, MD Anderson Cancer Center, Houston, Texas (Y.W., J.L.); Department of Radiation Oncology, Mayo Clinic, Rochester, Minnesota (R.M.); Department of Radiation Oncology, Catalan Institute of Oncology, Badalona, Spain (S.V.); Department of Oncology and Radiotherapy, Medical University of Gdansk, Gdansk, Poland (R.Z.); Department of Radiation Oncology, Inselspital, Bern, Switzerland (A.P.); Department of Radiation Oncology, Dr Bernard Verbeeten Instituut, Tilburg, the Netherlands (P.P.); Department of Radiation Oncology, Ege University Medical School, Izmir, Turkey (Y.A.); Department of Radiation Oncology, Faculty of Medicine, Hacettepe University, Ankara, Turkey (G.O.); Department of Robert-Janker-Klinik in Germany, Bonn, Germany (B.B.); Department of Clinical Epidemiology Unit, Department of Community Medicine, Geneva University Hospital, Geneva, Switzerland (G.H.); Department of Medicine I & Comprehensive Cancer Center, Medical University of Vienna, Vienna, Austria (M.P.).
authors.forename: Philip
authors.initials: P
authors.lastname: Poortmans
authors.affiliation: Center of Proton Therapy, Paul Scherrer Institute, Villigen/Würenlingen, Switzerland (D.C.W.); Department of Radiation Oncology, MD Anderson Cancer Center, Houston, Texas (Y.W., J.L.); Department of Radiation Oncology, Mayo Clinic, Rochester, Minnesota (R.M.); Department of Radiation Oncology, Catalan Institute of Oncology, Badalona, Spain (S.V.); Department of Oncology and Radiotherapy, Medical University of Gdansk, Gdansk, Poland (R.Z.); Department of Radiation Oncology, Inselspital, Bern, Switzerland (A.P.); Department of Radiation Oncology, Dr Bernard Verbeeten Instituut, Tilburg, the Netherlands (P.P.); Department of Radiation Oncology, Ege University Medical School, Izmir, Turkey (Y.A.); Department of Radiation Oncology, Faculty of Medicine, Hacettepe University, Ankara, Turkey (G.O.); Department of Robert-Janker-Klinik in Germany, Bonn, Germany (B.B.); Department of Clinical Epidemiology Unit, Department of Community Medicine, Geneva University Hospital, Geneva, Switzerland (G.H.); Department of Medicine I & Comprehensive Cancer Center, Medical University of Vienna, Vienna, Austria (M.P.).
authors.forename: Yavuz
authors.initials: Y
authors.lastname: Anacak
authors.affiliation: Center of Proton Therapy, Paul Scherrer Institute, Villigen/Würenlingen, Switzerland (D.C.W.); Department of Radiation Oncology, MD Anderson Cancer Center, Houston, Texas (Y.W., J.L.); Department of Radiation Oncology, Mayo Clinic, Rochester, Minnesota (R.M.); Department of Radiation Oncology, Catalan Institute of Oncology, Badalona, Spain (S.V.); Department of Oncology and Radiotherapy, Medical University of Gdansk, Gdansk, Poland (R.Z.); Department of Radiation Oncology, Inselspital, Bern, Switzerland (A.P.); Department of Radiation Oncology, Dr Bernard Verbeeten Instituut, Tilburg, the Netherlands (P.P.); Department of Radiation Oncology, Ege University Medical School, Izmir, Turkey (Y.A.); Department of Radiation Oncology, Faculty of Medicine, Hacettepe University, Ankara, Turkey (G.O.); Department of Robert-Janker-Klinik in Germany, Bonn, Germany (B.B.); Department of Clinical Epidemiology Unit, Department of Community Medicine, Geneva University Hospital, Geneva, Switzerland (G.H.); Department of Medicine I & Comprehensive Cancer Center, Medical University of Vienna, Vienna, Austria (M.P.).
authors.forename: Gokhan
authors.initials: G
authors.lastname: Ozygit
authors.affiliation: Center of Proton Therapy, Paul Scherrer Institute, Villigen/Würenlingen, Switzerland (D.C.W.); Department of Radiation Oncology, MD Anderson Cancer Center, Houston, Texas (Y.W., J.L.); Department of Radiation Oncology, Mayo Clinic, Rochester, Minnesota (R.M.); Department of Radiation Oncology, Catalan Institute of Oncology, Badalona, Spain (S.V.); Department of Oncology and Radiotherapy, Medical University of Gdansk, Gdansk, Poland (R.Z.); Department of Radiation Oncology, Inselspital, Bern, Switzerland (A.P.); Department of Radiation Oncology, Dr Bernard Verbeeten Instituut, Tilburg, the Netherlands (P.P.); Department of Radiation Oncology, Ege University Medical School, Izmir, Turkey (Y.A.); Department of Radiation Oncology, Faculty of Medicine, Hacettepe University, Ankara, Turkey (G.O.); Department of Robert-Janker-Klinik in Germany, Bonn, Germany (B.B.); Department of Clinical Epidemiology Unit, Department of Community Medicine, Geneva University Hospital, Geneva, Switzerland (G.H.); Department of Medicine I & Comprehensive Cancer Center, Medical University of Vienna, Vienna, Austria (M.P.).
authors.forename: Birgitta
authors.initials: B
authors.lastname: Baumert
authors.affiliation: Center of Proton Therapy, Paul Scherrer Institute, Villigen/Würenlingen, Switzerland (D.C.W.); Department of Radiation Oncology, MD Anderson Cancer Center, Houston, Texas (Y.W., J.L.); Department of Radiation Oncology, Mayo Clinic, Rochester, Minnesota (R.M.); Department of Radiation Oncology, Catalan Institute of Oncology, Badalona, Spain (S.V.); Department of Oncology and Radiotherapy, Medical University of Gdansk, Gdansk, Poland (R.Z.); Department of Radiation Oncology, Inselspital, Bern, Switzerland (A.P.); Department of Radiation Oncology, Dr Bernard Verbeeten Instituut, Tilburg, the Netherlands (P.P.); Department of Radiation Oncology, Ege University Medical School, Izmir, Turkey (Y.A.); Department of Radiation Oncology, Faculty of Medicine, Hacettepe University, Ankara, Turkey (G.O.); Department of Robert-Janker-Klinik in Germany, Bonn, Germany (B.B.); Department of Clinical Epidemiology Unit, Department of Community Medicine, Geneva University Hospital, Geneva, Switzerland (G.H.); Department of Medicine I & Comprehensive Cancer Center, Medical University of Vienna, Vienna, Austria (M.P.).
authors.forename: Guy
authors.initials: G
authors.lastname: Haller
authors.affiliation: Center of Proton Therapy, Paul Scherrer Institute, Villigen/Würenlingen, Switzerland (D.C.W.); Department of Radiation Oncology, MD Anderson Cancer Center, Houston, Texas (Y.W., J.L.); Department of Radiation Oncology, Mayo Clinic, Rochester, Minnesota (R.M.); Department of Radiation Oncology, Catalan Institute of Oncology, Badalona, Spain (S.V.); Department of Oncology and Radiotherapy, Medical University of Gdansk, Gdansk, Poland (R.Z.); Department of Radiation Oncology, Inselspital, Bern, Switzerland (A.P.); Department of Radiation Oncology, Dr Bernard Verbeeten Instituut, Tilburg, the Netherlands (P.P.); Department of Radiation Oncology, Ege University Medical School, Izmir, Turkey (Y.A.); Department of Radiation Oncology, Faculty of Medicine, Hacettepe University, Ankara, Turkey (G.O.); Department of Robert-Janker-Klinik in Germany, Bonn, Germany (B.B.); Department of Clinical Epidemiology Unit, Department of Community Medicine, Geneva University Hospital, Geneva, Switzerland (G.H.); Department of Medicine I & Comprehensive Cancer Center, Medical University of Vienna, Vienna, Austria (M.P.).
authors.forename: Matthias
authors.initials: M
authors.lastname: Preusser
authors.affiliation: Center of Proton Therapy, Paul Scherrer Institute, Villigen/Würenlingen, Switzerland (D.C.W.); Department of Radiation Oncology, MD Anderson Cancer Center, Houston, Texas (Y.W., J.L.); Department of Radiation Oncology, Mayo Clinic, Rochester, Minnesota (R.M.); Department of Radiation Oncology, Catalan Institute of Oncology, Badalona, Spain (S.V.); Department of Oncology and Radiotherapy, Medical University of Gdansk, Gdansk, Poland (R.Z.); Department of Radiation Oncology, Inselspital, Bern, Switzerland (A.P.); Department of Radiation Oncology, Dr Bernard Verbeeten Instituut, Tilburg, the Netherlands (P.P.); Department of Radiation Oncology, Ege University Medical School, Izmir, Turkey (Y.A.); Department of Radiation Oncology, Faculty of Medicine, Hacettepe University, Ankara, Turkey (G.O.); Department of Robert-Janker-Klinik in Germany, Bonn, Germany (B.B.); Department of Clinical Epidemiology Unit, Department of Community Medicine, Geneva University Hospital, Geneva, Switzerland (G.H.); Department of Medicine I & Comprehensive Cancer Center, Medical University of Vienna, Vienna, Austria (M.P.).
authors.forename: Jing
authors.initials: J
authors.lastname: Li
mesh_terms: D000328:Adult; D017024:Chemotherapy, Adjuvant; D018572:Disease-Free Survival; D004806:Ependymoma / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery / Q000628:therapy*; D005260:Female; D006801:Humans; D008297:Male; D013125:Spinal Neoplasms / Q000188:drug therapy / Q000532:radiotherapy / Q000601:surgery / Q000628:therapy*; D016896:Treatment Outcome
keywords: myxopapillary ependymoma; radiotherapy; spinal failures; spinal tumors