Journal of cancer research and clinical oncology · 2026
Undifferentiated pleomorphic sarcoma of bone (UPSB) treated in the German-speaking countries. A report of 132 unselected patients from the Cooperative Osteosarcoma Study Group (COSS).
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Bielack Stefan S, Andreou Dimosthenis, Baumhoer Daniel, Blattmann Claudia, Friedel Godehard, Fröhlich Birgit, Harrabi Semi Ben, Kager Leo, von Kalle Thekla, Kessler Torsten, Kevric Matthias, Knoll Antonia, Kühne Thomas, Reichardt Peter, Sorg Benjamin, Windhager Reinhard, Hecker-Nolting Stefanie
Аннотация
PURPOSE: To describe Undifferentiated Pleomorphic Sarcoma of Bone (UPSB) treated in Germany, Austria, or Switzerland and using the same treatment-approach as for osteosarcoma.
PATIENTS AND METHODS: The database of the Cooperative Osteosarcoma Study Group (COSS) was screened for UPSB. Eligible patients were evaluated for patient, tumor, and treatment related variables, and outcomes.
RESULTS: One-hundred thirty-two eligible patients were identified (median age 41.7 (range: 10.3-74.6) years; males 58%; preceding malignancies 11%; tumor-sites extremities 86%, trunk 12%, head & neck 2%). Relative tumor size was < 1/3 of the involved bone in 83% of evaluable cases, pathologic fractures were present in 14% (limb-primaries only), and primary distant metastases in 6%. All patients received chemotherapy and 96% surgery for their primary (81% limb-salvage for extremity lesions). The response rate to preoperative chemotherapy was 38%. After a median follow-up of 3.9 (1 day-34.1) years for event-free and 5.2 (0.2-34.1) years for overall survival, the 5 year event-free and overall survival probabilities were 63% (standard error: 5%) and 70% (4%), respectively. Younger patient age, an extremity tumor, and localized disease predicted superior outcomes, pathologic fractures and limb-salvage surgery worse. The extent of tumor response to pre-operative chemotherapy seemed to have no impact on prognosis.
DISCUSSION: UPSB generally affects considerably older persons than does the more frequent osteosarcoma. Despite a lower response rate to preoperative chemotherapy, its prognosis is at least comparable, if not better. Several prognostic factors impacting outcome could be defined. These results provide a benchmark for this rare disease.
PATIENTS AND METHODS: The database of the Cooperative Osteosarcoma Study Group (COSS) was screened for UPSB. Eligible patients were evaluated for patient, tumor, and treatment related variables, and outcomes.
RESULTS: One-hundred thirty-two eligible patients were identified (median age 41.7 (range: 10.3-74.6) years; males 58%; preceding malignancies 11%; tumor-sites extremities 86%, trunk 12%, head & neck 2%). Relative tumor size was < 1/3 of the involved bone in 83% of evaluable cases, pathologic fractures were present in 14% (limb-primaries only), and primary distant metastases in 6%. All patients received chemotherapy and 96% surgery for their primary (81% limb-salvage for extremity lesions). The response rate to preoperative chemotherapy was 38%. After a median follow-up of 3.9 (1 day-34.1) years for event-free and 5.2 (0.2-34.1) years for overall survival, the 5 year event-free and overall survival probabilities were 63% (standard error: 5%) and 70% (4%), respectively. Younger patient age, an extremity tumor, and localized disease predicted superior outcomes, pathologic fractures and limb-salvage surgery worse. The extent of tumor response to pre-operative chemotherapy seemed to have no impact on prognosis.
DISCUSSION: UPSB generally affects considerably older persons than does the more frequent osteosarcoma. Despite a lower response rate to preoperative chemotherapy, its prognosis is at least comparable, if not better. Several prognostic factors impacting outcome could be defined. These results provide a benchmark for this rare disease.