ISSN:
1569-8041
Keywords:
alveolar soft part sarcoma
;
pediatric tumors
;
soft tissue sarcoma
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Abstract Background:Alveolar soft part sarcoma (ASPS) is a rare malignanttumor and little is known about its clinical features and management. Wereport on a series of 19 pediatric patients managed over 20 years. Patients and methods:Primary conservative surgery was performedin all patients and was radical in nine, non-radical in three; seven patientsunderwent biopsy alone (3 unresectable tumors, 4 metastatic disease). In twocases radical surgery was performed after primary chemotherapy. Radiotherapywas delivered to 8 patients, chemotherapy to 15. Results:After a median follow-up of 74 months, the five-yearsurvival was 80% for the whole series, 91% for patients withlocalized disease, 100% for patients with tumor ≤5 cm, and31% for those 〉5 cm; 16 of 19 patients were alive (12 of 12 withgrossly-resected tumor in first continuous remission). Chemotherapy achievedtwo partial remission among seven evaluable patients. Conclusions:Pediatric ASPS has a more favorable prognosis thanits adult counterpart. In this series, tumor size correlates with metastaticdisease at onset and is the major factor influencing survival. Surgery is themainstay of therapy. The effectiveness of adjuvant therapy remains to beestablished, though radiotherapy may be advisable in cases of inadequatesurgery.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1023/A:1026579623136
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