title: Alveolar soft part sarcoma in Japan: multi-institutional study of 57 patients from the Japanese Musculoskeletal Oncology Group.
abstract: OBJECTIVE
  The clinical features and the management of alveolar soft part sarcoma (ASPS) are not well known. The efficacy of chemotherapy for soft tissue sarcoma, including high-dose ifosfamide and cisplatin, has not been established yet. Some reports suggest ASPS may occur primarily in bone.
  
  
  METHODS
  We report on a series of 57 patients with ASPS over 20 years. Their ages ranged from 7 to 75 years (mean 25).
  
  
  RESULTS
  There were 37 females and 20 males. Thirteen lesions (23%) showed bone involvement at the primary site, and 6 of them were diagnosed as bone tumors at presentation. Thirty-seven patients had distant metastases at presentation. Tumor size, bone involvement at the primary site and the presence of metastases at presentation were prognostic indicators (p < 0.05). Marginal excision with radiotherapy or wide excision without radiotherapy achieved good local control. Chemotherapy was performed in 47 patients with different regimens. Two patients treated with intra-arterial chemotherapy regimens responded partially, but intravenous chemotherapy with high-dose ifosfamide or cisplatin failed.
  
  
  CONCLUSIONS
  ASPS can present primarily as a bone tumor. No advantage of chemotherapy with high-dose ifosfamide or cisplatin could be demonstrated.
authors.affiliation: Division of Orthopedic Surgery, Graduate School of Medical and Dental Sciences, Niigata University, Niigata, Japan. aogose@med.niigata-u.ac.jp
authors.forename: Akira
authors.initials: A
authors.lastname: Ogose
authors.forename: Yasuo
authors.initials: Y
authors.lastname: Yazawa
authors.forename: Takafumi
authors.initials: T
authors.lastname: Ueda
authors.forename: Tetsuo
authors.initials: T
authors.lastname: Hotta
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Kawashima
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Hatano
authors.forename: Tetsuro
authors.initials: T
authors.lastname: Morita
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D001859:Bone Neoplasms / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000209:etiology / Q000401:mortality / Q000556:secondary / Q000628:therapy; D002648:Child; D002945:Cisplatin / Q000008:administration & dosage; D003131:Combined Modality Therapy; D005260:Female; D006801:Humans; D007069:Ifosfamide / Q000008:administration & dosage; D007261:Infusions, Intra-Arterial; D007262:Infusions, Intravenous; D007564:Japan / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D009362:Neoplasm Metastasis; D011379:Prognosis; D011859:Radiography; D018714:Radiotherapy, Adjuvant; D012189:Retrospective Studies; D012509:Sarcoma / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000209:etiology / Q000401:mortality / Q000556:secondary / Q000628:therapy; D012983:Soft Tissue Neoplasms / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000209:etiology / Q000401:mortality / Q000473:pathology / Q000628:therapy; D016019:Survival Analysis
chemical_list: D002945:Cisplatin; D007069:Ifosfamide