6.P.02 SYNOVIAL SARCOMA OF CHILDHOOD – SURGICAL LESSONS

Synovial sarcoma (SS) of childhood is considered chemoresponsive, what frequently leads to conservative surgical approach. Aim of the report is to assess whether that approach is sufficient and what are other clinical factors influencing the outcome. Patients: 23 children (aged 3 months – 17 years) treated in 5 cooperating centres for nonmetastatic SS located in the limbs(Fu>36 months). Treatment: primary or secondary resections, chemotherapy and radiotherapy (in case of resection R1 or R2 or relapse). Locations of tumours: thigh/6, shank/1, popliteal fossa/4, cubital fossa/3, axilla/2, forearm/2, foot/3, arm/1, hand/1. Primary R0 achieved in 3, R1/5, R2/3. Twelve after initial biopsy and 3 after primary R2 were submitted to chemotherapy and secondary surgery. 12/15 those resections were R0, 3/15 R1. Two of them underwent mutilating resections. Results: 11 pts are in CR. 12 relapsed (7 local, 5 metastatic). Initial locations of the relapsing tumours were around joints/4, foot/hand 4, thigh 3, arm 1. Seven of them died despite aggressive re-treatment, 2 are alive with disease, 3 are in second CR. Quality of resections (primary or secondary) in 7 locally relapsing pts were R0 in 3, R1 in 3 and biopsy only /1 (CR after CHT alone, refused local treatment). Of 2 submitted to mutilating resections, 1 relapsed in the lungs (2nd CR after re-treatment and metastasectomy). Summary: R0 doesn’t prevent from local relapse (3 of 7 pts relapsed after R0 vs 4/7 with R1). Mutilating resections (amputations) were unfrequent (2) and were not followed by local relapses. One metastatic relapse occurred and was finally cured. Locations at risk seemed joints and distal parts of limbs (8/12 of the relapsing pts). Relapsed implied weak chance for long-term second CR (3/12).