[Clinical features and prognosis of alveolar soft part sarcoma].
OBJECTIVE
To investigate the clinical feature, suitable treatment and prognosis of alveolar soft part sarcoma.


METHODS
The clinical data of 58 such patients treated at our hospital from 1967 to 2006 were retrospectively analyzed. Fifty-three patients except 6 with distant metastasis underwent surgical resection including 33 extensive resection and 19 local resection. Furthermore, 19 patients received postoperative adjuvant radiotherapy or chemotherapy.


RESULTS
Eleven of 50 patients who underwent complete resection developed local recurrence. Thirty-One (53.4%) was found to have lung metastasis. The overall 3-, 5-, 10-year survival rate was 89.5%, 74.1% and 57.7%, respectively. The median survival time of the patients was 125 months. The 3-, 5-, 10-year survival rate was 100.0%, 81.6% and 65.3% for female patients, but it was 79.6%, 67.2% and 49.7% for male patients, respectively.


CONCLUSIONS
Alveolar soft part sarcoma usually grows slowly. Though local recurrence is rarely seen, distant metastasis is frequently observed. Lung is the most common organ of metastasis. However, survival can still be long even with lung metastasis. To achieve microscopically complete resection is quite critical to localized alveolar soft part sarcoma. No survival advantage was observed when adding adjuvant radiotherapy and/or chemotherapy. The phenomena of female patient or patient with X chromosome translocation showing better prognosis than the male or other patients needs further investigation.
Xu
Li-Bin
LB
Department of Orthopaedic Surgery, Cancer Hospital, Chinese Academy of Medical Sciences, Beijing 100021, China.
Yu
Sheng-Ji
SJ
Shao
Yong-Fu
YF
Zhang
Hong-Tu
HT
Zhao
Zhen-Guo
ZG
D000293:Adolescent; D000328:Adult; D017024:Chemotherapy, Adjuvant; D002648:Child; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008175:Lung Neoplasms / Q000556:secondary*; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D009367:Neoplasm Staging; D018714:Radiotherapy, Adjuvant; D012189:Retrospective Studies; D018234:Sarcoma, Alveolar Soft Part / Q000188:drug therapy / Q000532:radiotherapy / Q000556:secondary / Q000601:surgery*; D012737:Sex Factors; D012983:Soft Tissue Neoplasms / Q000188:drug therapy / Q000473:pathology / Q000532:radiotherapy / Q000601:surgery*; D015996:Survival Rate; D055815:Young Adult