E. Koscielniak

Mesenchymal Chondrosarcoma of Soft Tissues and Bone in Children, Adolescents, and Young Adults : Experiences of the CWS and COSS Study Groups

Mesenchymal chondrosarcoma (MCS) is a rare tumor with a strong tendency toward late recurrences leading to reported 10‐year survival rates below 50%. The recommended treatment is resection with wide margins; the effectiveness of chemo‐ and radiotherapy remain poorly defined. As reports about MCS in young patients are scarce, treatment and outcomes of children/adolescents/young adults in the CWS and COSS studies were investigated.

Comparison of the rates of response to ifosfamide and cyclophosphamide in primary unresectable rhabdomyosarcomas.

SummaryIn the 1981 cooperative soft-tissue sarcoma (CWS-81) study, a clear correlation between the degree of response to initial chemotherapy comprising vincristine, actinomycin D, cyclophosphamide, and Adriamycin (VACA) and the survival of patients with rhabdomyosarcoma was found. In the subsequent CWS-86 study, cyclophosphamide was replaced by ifosfamide (VAIA) in the expectation that the combination VAIA might be more effective than VACA. In both studies, the initial cytostatic response for primary unresectable tumors was evaluated after the first cycle of chemotherapy at weeks 7–9. The reduction in tumor volume was measured by computerized axial tomographic (CAT) scan or sonography, and the patients were categorized as complete responders, patients with a tumor regression of >2/3 albeit incomplete, patients with a tumor regression of <2/3 but >1/3, and nonresponders, who underwent either a tumor regression of <1/3 or tumor progression. We compared the response rate obtained with VACA chemotherapy and that resulting from VAIA chemotherapy. The preliminary data from this comparison show a tendency for a higher rate of good responders (complete and >2/3 tumor regression) to be induced by VAIA therapy (71%) than that obtained using the VACA combination (55%). From the response-prognosis relationship, we confidently expect that the final outcome for patients in the present study will be better than that in the previous study.

Embryonal rhabdomyosarcoma with metastases confined to the lungs: Report from the CWS Study Group†‡§

Embryonal rhabdomyosarcoma [RME] is the most common pediatric soft tissue sarcoma. Whereas the prognosis of localized rhabdomyosarcoma has improved, it remains poor for metastatic disease.

Tumour volume reduction after neoadjuvant chemotherapy impacts outcome in localised embryonal rhabdomyosarcoma

Response (tumour volume reduction) to induction chemotherapy has been used to stratify secondary local and systemic treatment of Intergroup Rhabdomyosarcoma Study Group III (IRSG‐III) embryonal rhabdomyosarcoma (RME) in consecutive CWS‐trials. To evaluate its actual impact we studied response‐related treatment and outcomes.

Malignant ectomesenchymoma in children and adolescents: Report from the Cooperative Weichteilsarkom Studiengruppe (CWS)

Malignant ectomesenchymoma (MEM) is a soft tissue tumor with heterologous rhabdomyoblastic components believed to arise from pluripotent migratory neural crest cells. To date merely 50 cases have been published and the knowledge about the course of disease and optimal treatment is limited.

Primary Metastatic Synovial Sarcoma: Experience of the CWS Study Group.

Prognostic factors for localized synovial sarcoma are well defined. However, few data exist regarding patients with metastases at diagnosis. Poor outcome is described but the optimal therapeutic regimen remains unclear. Our aim was to assess the outcome, identify prognostic factors, and analyze treatment strategies.