title: Primary extracranial rhabdoid tumors. Clinicopathologic features and response to ifosfamide.
abstract: BACKGROUND
  Malignant rhabdoid tumor (MRT) is an aggressive, invariably lethal tumor that is resistant to multimodal therapy.
  
  
  METHODS
  The authors reviewed the clinicopathologic features, treatment, and outcome of 13 children (7 boys and 6 girls) with diagnoses of primary extracranial MRT at St. Jude Children's Research Hospital between 1981 and 1990.
  
  
  RESULTS
  The median age at diagnosis was 8 months (range, 10 weeks-18 years). Primary sites included the kidney (seven patients), liver (three patients), soft tissue of scapula, posterior mediastinum, and retroperitoneum. Seven patients had metastatic disease (lungs, six patients; cutaneous hemangioma, one patient). Ten patients had surgical resection of primary tumor (complete, nine patients; incomplete, one patient). Eleven patients had chemotherapy with multiple agents. Three of four chemotherapy responses observed were with regimens containing ifosfamide. Partial responses (PR, > 50% reduction in tumor size) were obtained in one patient who received single-agent ifosfamide during disease relapse (PR lasting 2 months), one patient who received a combination of ifosfamide, carboplatin, and etoposide at diagnosis (PR lasting 5 months), and one patient who was treated with bleomycin, cyclophosphamide, doxorubicin, and vincristine at diagnosis (PR lasting 5 months) and subsequently with ifosfamide in combination with carboplatin and etoposide during disease relapse (PR lasting 4 months). All patients died at a median period of 5 months (range, 0.5-30 months) after diagnosis.
  
  
  CONCLUSIONS
  Based on this review, the authors recommend using ifosfamide alone or in combination with carboplatin and etoposide in front-line therapy for malignant rhabdoid tumor.
authors.affiliation: Department of Hematology/Oncology, St. Jude Children's Research Hospital, Memphis, Tennessee 38101-0318.
authors.forename: S
authors.initials: S
authors.lastname: Gururangan
authors.forename: L C
authors.initials: LC
authors.lastname: Bowman
authors.forename: D M
authors.initials: DM
authors.lastname: Parham
authors.forename: J A
authors.initials: JA
authors.lastname: Wilimas
authors.forename: B
authors.initials: B
authors.lastname: Rao
authors.forename: C B
authors.initials: CB
authors.lastname: Pratt
authors.forename: E C
authors.initials: EC
authors.lastname: Douglass
mesh_terms: D000293:Adolescent; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001859:Bone Neoplasms / Q000150:complications / Q000188:drug therapy; D016190:Carboplatin / Q000008:administration & dosage; D002648:Child; D002675:Child, Preschool; D004334:Drug Administration Schedule; D005260:Female; D006801:Humans; D007069:Ifosfamide / Q000008:administration & dosage; D007223:Infant; D007680:Kidney Neoplasms / Q000150:complications / Q000188:drug therapy; D008113:Liver Neoplasms / Q000150:complications / Q000188:drug therapy; D008297:Male; D008479:Mediastinal Neoplasms / Q000150:complications / Q000188:drug therapy; D012186:Retroperitoneal Neoplasms / Q000150:complications / Q000188:drug therapy; D012540:Scapula
chemical_list: D016190:Carboplatin; D007069:Ifosfamide