Alveolar soft part sarcoma in Japan: multi-institutional study of 57 patients from the Japanese Musculoskeletal Oncology Group.
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.
Ogose
Akira
A
Division of Orthopedic Surgery, Graduate School of Medical and Dental Sciences, Niigata University, Niigata, Japan. aogose@med.niigata-u.ac.jp
Yazawa
Yasuo
Y
Ueda
Takafumi
T
Hotta
Tetsuo
T
Kawashima
Hiroyuki
H
Hatano
Hiroshi
H
Morita
Tetsuro
T
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
D002945:Cisplatin; D007069:Ifosfamide