We evaluated the risk of
Clear cell sarcoma (CCS) of soft-tissue is a rare melanocytic subtype of mesenchymal malignancy. The aim of this study was to investigate the clinical and therapeutic factors associated with increased survival, stratified by clinical stage, in order to determine the optimal treatment. The study was a retrospective analysis involving 117 patients with histologically confirmed CCS, between July 2016 and November 2017, who were enrolled in the Bone and Soft Tissue Tumour Registry in Japan.Aims
Methods
Aim. Ten years ago at the EMSOS 2000 meeting we have presented our experience concerning the non-surgical treatment of stage IIB osteosarcoma of extremities. The purpose of study was to evaluate long-term results and complications related to this non-standard and controversial treatment modality. Methods. Since 1988 definitive radiation gamma therapy (RT) in the median dose of 60 Gy was administrated to 36 patients who refused surgery after preoperative chemotherapy. Until 1999 induction consisted in 3-5 cycles of intra-arterial doxorubicin (DOX) 75 mg/m2 or cis-platin (CDDP) 120 mg/m2. Since 2000 induction comprised 4 cycles of DOX 90 mg/m2+CDDP 120 mg/m2 intra-arterially. Tumour response before local treatment was assessed non-invasively by means of X-ray, CT, MRI, angiography, 3-phase bone scans and alkaline phosphatase measurement. The end-points were overall (OS), metastases free (MFS) and local progression free (LPFS) survival at 10 years. Results. OS, MFS and LPFS by Kaplan-Meier method were 48±9%, 51±9%, and 45±11% respectively. The outcome was significantly related to response assessed before RT. In 12 good responders OS, MFS and LPFS were 74±13%, 91±9%, and 69±19% respectively compared with 29±11%, 27±11%, and 34±13% in 24 non-responders, p- value 0.03, 0.003, 0.009, respectively. In 14 patients 5 years survival after local progression and delayed surgery was 38±10%. Median survival time after local relapse was 36 months. Two types of major complications were seen: 7 pathological fractures and one osteomyelitis. 21 patients (58%) remained with a functional limb until the last examination or death. Conclusions. Long-term results were higher than expected in this radioresistant tumour. In good responders to induction chemotherapy RT could be an option for local treatment. Majority of patients had a functional limb during and long time after treatment. Isolated