title: Extra-cranial Malignant Rhabdoid Tumor in Children: A Single Institute Experience.
abstract: OBJECTIVE
  Malignant rhabdoid tumor (MRT) is a rare and highly aggressive tumor that affects young children. Due to its extreme rarity, most of the available data are based on retrospective case series. To add to the current knowledge of this disease, we reviewed the patients treated for extra-cranial MRT in our institute.
  
  
  METHODS
  A retrospective medical record review was conducted on children treated for pathologically confirmed extra-cranial MRT at Seoul National University Children's Hospital between January 2003 and May 2013.
  
  
  RESULTS
  Eleven patients (7 boys, 4 girls) were diagnosed with extra-cranial MRT at a median age of 9 months old. INI1 staining was important in the pathological confirmation. Six patients (55%) had renal MRT and five (45%) had soft tissue MRT. Five patients (45%) had metastases at diagnosis. All patients underwent chemotherapy, eight patients (73%) underwent surgery, six patients (55%) received therapeutic radiotherapy, and four patients (36%) underwent high dose chemotherapy with autologous stem cell rescue (HDCT/ASCR) with melphalan, etoposide, and carboplatin. Five patients (45%) died of disease following progression (n=3) or relapse (n=2), however, there was no treatment related mortality. The overall survival of the cohort was 53.0% and the event-free survival was 54.5% with a median follow-up duration of 17.8 months (range, 2.3 to 112.3 months).
  
  
  CONCLUSIONS
  Extra-cranial MRT is still a highly aggressive tumor in young children. However, the improved survival of our cohort is promising and HDCT/ASCR with melphalan, etoposide, and carboplatin may be a promising treatment option.
authors: Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.|Cancer Research Institute, Seoul National University College of Medicine, Seoul, Korea.
authors: Che Ry
authors: CR
authors: Hong
authors: Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.|Cancer Research Institute, Seoul National University College of Medicine, Seoul, Korea.
authors: Hyoung Jin
authors: HJ
authors: Kang
authors: Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.|Cancer Research Institute, Seoul National University College of Medicine, Seoul, Korea.
authors: Hee Young
authors: HY
authors: Ju
authors: Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.|Cancer Research Institute, Seoul National University College of Medicine, Seoul, Korea.
authors: Ji Won
authors: JW
authors: Lee
authors: Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.|Cancer Research Institute, Seoul National University College of Medicine, Seoul, Korea.
authors: Hyery
authors: H
authors: Kim
authors: Department of Pathology, Seoul National University College of Medicine, Seoul, Korea.
authors: Sung-Hye
authors: SH
authors: Park
authors: Department of Radiation Oncology, Seoul National University College of Medicine, Seoul, Korea.
authors: Il Han
authors: IH
authors: Kim
authors: Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.|Cancer Research Institute, Seoul National University College of Medicine, Seoul, Korea.
authors: Kyung Duk
authors: KD
authors: Park
authors: Department of Pediatrics, Seoul National University Children's Hospital, Seoul, Korea.|Cancer Research Institute, Seoul National University College of Medicine, Seoul, Korea.
authors: Hee Young
authors: HY
authors: Shin
mesh_terms: D005260:Female; D006801:Humans; D007223:Infant; D007680:Kidney Neoplasms / Q000175:diagnosis* / Q000628:therapy; D008297:Male; D012189:Retrospective Studies; D018335:Rhabdoid Tumor / Q000175:diagnosis* / Q000628:therapy; D012983:Soft Tissue Neoplasms / Q000175:diagnosis* / Q000628:therapy
keywords: Kidney neoplasms; Neoplasm metastasis; Rhabdoid tumor; Soft tissue neoplasms