title: Long-term survival of an infant with an atypical teratoid/rhabdoid tumor following subtotal resection and low-cumulative dose chemotherapy: a case report.
abstract: BACKGROUND
  Atypical teratoid/rhabdoid tumor (AT/RT) is an aggressive embryonal tumor of the central nervous system with a generally dismal prognosis, especially in patients younger than 12 months.
  
  
  CONCLUSIONS
  We here describe the unusual case of an infant with AT/RT with long-term survival despite low-cumulative dose chemotherapy after subtotal resection. Due to a poor neurological situation and an unfavorable oncological prognosis, therapy was halted after two partial surgical resections and four of the nine chemotherapy courses recommended by the European Rhabdoid Registry, without the patient receiving either radiotherapy or high-dose chemotherapy. The patient is alive without evidence of disease 52 months after diagnosis.
  
  
  CONCLUSIONS
  This case report highlights that independent prognostic factors are urgently needed for optimizing treatment stratification and preventing overtreatment.
authors.affiliation: Department of Pediatric Oncology/Hematology/BMT, Charité - Universitätsmedizin Berlin, Augustenburger Platz 1, 13353, Berlin, Germany. viktor.arnhold@charite.de.
authors.forename: Viktor
authors.initials: V
authors.lastname: Arnhold
authors.affiliation: Department of Pediatric Oncology and Hematology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
authors.forename: Florian
authors.initials: F
authors.lastname: Oyen
authors.affiliation: Department of Pediatric Oncology and Hematology, University Medical Center Hamburg-Eppendorf, Hamburg, Germany.
authors.forename: Reinhard
authors.initials: R
authors.lastname: Schneppenheim
authors.affiliation: Department of Pediatric Neurosurgery, Charité - Universitätsmedizin Berlin, Berlin, Germany.
authors.forename: Hannes
authors.initials: H
authors.lastname: Haberl
authors.affiliation: Institute of Pathology, Charité - Universitätsmedizin Berlin, Berlin, Germany.
authors.forename: Arend
authors.initials: A
authors.lastname: Koch
authors.affiliation: Swabian Children's Cancer Center, Children's Hospital Augsburg, Klinikum Augsburg, Augsburg, Germany.
authors.forename: Michael C
authors.initials: MC
authors.lastname: Frühwald
authors.affiliation: Department of Pediatric Oncology/Hematology/BMT, Charité - Universitätsmedizin Berlin, Augustenburger Platz 1, 13353, Berlin, Germany.
authors.forename: Pablo
authors.initials: P
authors.lastname: Hernáiz Driever
mesh_terms: D000970:Antineoplastic Agents / Q000627:therapeutic use*; D001932:Brain Neoplasms / Q000188:drug therapy* / Q000601:surgery*; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007223:Infant; D008279:Magnetic Resonance Imaging; D019635:Neurosurgical Procedures / Q000379:methods*; D018335:Rhabdoid Tumor / Q000188:drug therapy / Q000401:mortality / Q000601:surgery
chemical_list: D000970:Antineoplastic Agents
keywords: AT/RT; Chemotherapy; Childhood cancer; SMARCB1/Ini1