Synovial sarcoma of childhood and adolescence. Report of the German CWS-81 study.
BACKGROUND
Synovial sarcoma is the third most common pediatric soft tissue tumor. It requires an aggressive approach to achieve a cure. However, optimal treatment modalities adapted to disease extension and histologic variants have not been determined because there is little information about prospectively treated patients.


METHODS
A multicenter trial for soft tissue sarcomas (Protocol CWS 81) was conducted in West Germany between 1981-1985, and 31 patients with synovial sarcoma were registered. Treatment included multiagent chemotherapy and irradiation after initial tumor excision or biopsy. The male-female ratio in this group was 1:1.6 with a median age of 14 years (range, 1-19 years). The median follow-up time after diagnosis was 101 months (range, 77-131 months).


RESULTS
The overall event-free survival (EFS) for patients with synovial sarcoma was 74.2% at 5 years. Group I-II tumors had a significantly better prognosis than those in Group III-IV (EFS at 5 years 84.4% and 58.3%, respectively; P = 0.024). Small tumors (< 5 cm) responded better than larger tumors (> or = 5 cm; EFS, 93% versus 58%; P = 0.029). Synovial sarcoma involved the extremities in 28 patients who had a better outcome compared with those with extremity rhabdomyosarcoma in this study (EFS for Group I-IV was 82% versus 24%, P = 0.001).


CONCLUSIONS
The results appeared superior to previous experience using radical surgery alone and suggested that after initial, nonmutilating surgery, adjuvant chemotherapy, and irradiation contributed to the improved long-term survival.
St. Anna Children's Hospital, Vienna, Austria.
R
R
Ladenstein
J
J
Treuner
E
E
Koscielniak
F
F
d'Oleire
M
M
Keim
H
H
Gadner
H
H
Jürgens
D
D
Niethammer
J
J
Ritter
D
D
Schmidt
D000293:Adolescent; D000328:Adult; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D017024:Chemotherapy, Adjuvant; D002648:Child; D003131:Combined Modality Therapy; D003520:Cyclophosphamide / Q000008:administration & dosage; D003609:Dactinomycin / Q000008:administration & dosage; D004317:Doxorubicin / Q000008:administration & dosage; D005121:Extremities; D005260:Female; D005500:Follow-Up Studies; D005860:Germany, West / Q000453:epidemiology; D006801:Humans; D007223:Infant; D008297:Male; D011379:Prognosis; D011879:Radiotherapy Dosage; D012086:Reoperation; D013584:Sarcoma, Synovial / Q000188:drug therapy / Q000401:mortality / Q000473:pathology / Q000601:surgery / Q000628:therapy*; D014750:Vincristine / Q000008:administration & dosage
D003609:Dactinomycin; D014750:Vincristine; D004317:Doxorubicin; D003520:Cyclophosphamide