Synovial sarcoma (SS) is a malignant soft tissue sarcoma with a poor prognosis because of late local recurrence and distant metastases. To our knowledge, no studies have minimum follow-up of 10 years that evaluate long-term outcomes for survivors. Data on 62 patients who had been treated for SS from 1968 to 1999 were studied retrospectively in a multicenter study. The following parameters were examined for their potential prognostic value: age at diagnosis, sex, tumour site and size, histology, histological grade, fusion type (SYT-SSX1 vs. SYT-SSX2), and surgical margin status. Mean follow-up of living patients was 17.2 years, and of dead patients 7.7 years.Aim
Method
The development of local recurrence after multimodal treatment of osteosarcoma is associated with a very poor prognosis. The importance of clear surgical margins has been demonstrated in multiple studies, however up to date there are no studies defining which margin width is safe from an oncological perspective. The purpose of this retrospective analysis was to evaluate whether margin width or other surgical and tumour-related factors influence the development of local recurrence in osteosarcoma patients. The files of 1867 consecutive patients with high-grade central osteosarcoma of the extremities, the pelvic bones and the shoulder girdle, who had achieved a complete surgical remission during combined-modality therapy on neoadjuvant Cooperative Osteosarcoma Study Group (COSS) protocols between 1986 and 2005, were reviewed. Of those, the data required were available for 1369 patients, who were the subject of this analysis. Eighty of these patients developed a local recurrence during the course of their illness. The median surgical margin width amounted to 45 mm (range, 0 to 140 mm) in the local recurrence (LR) group and 50 mm (range, 0 to 350 mm) in the non-local recurrence (NLR) group (p=0.106). No statistically significant difference between the two groups was found regarding tumour size (mean, 10.38 cm and 9.53 cm respectively, p=0.169), T-status (p=0.225) and presence of pathological fracture (p=0.231). However infiltration of the soft tissue beyond the periosteum was documented in 58.8% of the patients with local recurrence and only in 36.9% of the rest (p=0.003). Furthermore, in 50% of the LR group the biopsy had been performed in a centre other than the one performing the definitive tumour resection, compared to 30.2% of the NRL group (p=0.001). In conclusion, the absolute metric width of surgical margins does not define oncological safety. Local recurrence is more likely to develop in patients with soft tissue infiltration beyond the periosteum or those biopsied in a centre other than the one performing the tumour resection.
Sclerosing epitheloid fibrosarcoma (SEF) is an extremely rare soft tissue sarcoma arising from connective tissue cells of mesenchymal origin. SEF mostly occurs in extraosseous sites in the soft tissue; however two cases of primary localization in the bone have been described. Despite benign cytological features the clinical course is complicated by a high local recurrence rate and late metastases. SEF represents a clinically challenging entity especially because no standardized treatment regimens are available. We report a 16-year old female patient who showed persistent load-dependent pain focused on the right proximal tibia. Radiological evaluation revealed an osteolytic lesion and the diagnosis of a benign bone cyst was consented. The tumor was surgically removed. Only after recurrence of the tumor and repeated histopathological analysis diagnosis of SEF could be established. Because of the bone localization of the tumor the patient underwent standardized neoadjuvant chemotherapy analogous to the European-American EURAMOS-1 protocol for the treatment of osteosarcoma followed by tumor resection and endoprothesis. Histopathological analysis of the resected tumor showed >
90% vital tumor cells suggesting no response to the neoadjuvant chemotherapy. Therefore, therapy was reassigned to the CWS protocol of the German Society for Pediatric Oncology and Hematology (GPOH) for treatment of soft tissue sarcoma. To date, the patient is alive and no metastases of the primary tumor can be detected. SEF represents a taunting clinical entity due to deceptive histopathological features and rare occurrence. Localization in the bone represents an additional challenge with regards to the therapeutical approach. Standardized treatment regimens are currently not available for SEF. This case report, to our knowledge, is the first outlining a therapeutic approach in detail. Our data suggest that SEF may be resistant to a chemotherapy regimen containing Cisplatin, Doxorubicin and Metho-trexate despite close association to the bone, possibly indicative of the soft tissue histogenesis of this tumor. The response to the soft tissue sarcoma targeting CWS chemotherapy remains to be determined.
