To date, all surgical techniques used for reconstruction of the pelvic ring following supra-acetabular tumour resection produce high complication rates. We evaluated the clinical, oncological and functional outcomes of a cohort of 35 patients (15 men and 20 women), including 21 Ewing’s sarcomas, six chondrosarcomas, three sarcomas not otherwise specified, one osteosarcoma, two osseous malignant fibrous histiocytomas, one synovial cell sarcoma and one metastasis. The mean age of the patients was 31 years (8 to 79) and the latest follow-up was carried out at a mean of 46 months (1.9 to 139.5) post-operatively.

We undertook a functional reconstruction of the pelvic ring using polyaxial screws and titanium rods. In 31 patients (89%) the construct was encased in antibiotic-impregnated polymethylmethacrylate. Preservation of the extremities was possible for all patients. The survival rate at three years was 93.9% (95% confidence interval (CI) 77.9 to 98.4), at five years it was 82.4% (95% CI 57.6 to 93.4). For the 21 patients with Ewing’s sarcoma it was 95.2% (95% CI 70.7 to 99.3) and 81.5% (95% CI 52.0 to 93.8), respectively. Wound healing problems were observed in eight patients, deep infection in five and clinically asymptomatic breakage of the screws in six. The five-year implant survival was 93.3% (95% CI 57.8 to 95.7). Patients were mobilised at a mean of 3.5 weeks (1 to 7) post-operatively. A post-operative neurological defect occurred in 12 patients. The mean Musculoskeletal Tumor Society score at last available follow-up was 21.2 (10 to 27).

This reconstruction technique is characterised by simple and oncologically appropriate applicability, achieving high primary stability that allows early mobilisation, good functional results and relatively low complication rates.

Cite this article: Bone Joint J 2013;95-B:1410–16.

Aim

We present the greatest study of patients with proximal fibula resection. Moreover we describe a new classification system for tumour resection of the proximal fibula independent of the tumour dignity.

Sarcomas are rare malignant tumors of mesenchymal origin and primarily occur in children, adolescents and young adults. With multimodal treatment concepts survival has significantly improved and is now in the range of 60–70 %. Following relapse or metastasis, however, the prognosis still is poor as is also the case for patients presenting with primary disseminated disease. TranSaR-Net aims to develop novel treatment strategies overcoming tumor cell resistance directed against novel targets. To achieve this goal the German pediatric, adolescent and adult sarcoma research groups have formed a collaborative network linking the nationwide and European trial groups with access to over 90 % of all pediatric and adolescent sarcoma patients and a large number of adult sarcoma patients to basic and translational sarcoma research groups. Within TranSaRNet a registry for patients at relapse is established as target cohort for innovative treatment strategies as well as a biomaterial banking network in order to facilitate the availability of tumor and other biomaterial for basic and translational research. A joint bioinformatics platform will integrate existing array data, to standardize laboratory and evaluation procedures and for modeling new theoretical concepts in a joint effort. Within the basic and translational research work packages, the sarcoma research groups in Germany have coordinated their research activities in a joint effort. The basic research work package (WP1) includes projects on genomic (WP1.1) and epigenetic (WP1.2) tumor characterization as well as identification of the tumor initiating cell (WP1.3) and resistance mechanisms (WP1.3 und 1.4), and the identification of new targets in apoptotic pathways (WP1.4, 2.4) and tumor-induced angiogenesis (WP1.5). The translational research work package (WP2) is focused on innovative immunological treatment strategies including sarcoma specific T-cells (WP2.1), dendritic cells (WP2.2), NK- cells (WP2.4) and tumor imaging (WP2.3).

A brief overview of the projects will be provided.

Tumor size and metastases are known risk factors in Ewing tumors. Adequate staging is essential to stratify treatment intensity, but TNM staging is not established.

The validity of TNM staging was tested based on tumor volume (T1 ≤200 ml; T2 > 200 ml ≤500 ml; T3, > 500 ml), the presence or absence of lymph node metastases (N0, N1), and distant metastases (M0, no metastases; M1 lung/pleura metastases; M1a ≤5 nodules; M1b > 5 lesions; M2 bone metastases; M2a, 1 lesion; M2b > 1 lesion and/or microscopic bone marrow contamination; M3, multi-system metastases). 1799 Ewing sarcoma patients of the (EI)CESS/EE99 studies entered into the Muenster database from 1981–2008 were analyzed.

Ten-year event-free survival (EFS) was 0.46. EFS in patients without metastases (T1-3N0M0) was 0.56 compared to 0.22 in metastatic patients (T1-3N0,1M1-3), p< .0001. In non-metastatic patients, tumor volume discriminated EFS: T1:0.62; T2:0.43; T3:0.40, p< .0001. The rare event of lymph node metastases correlated with unfavorable prognosis (N0:0.47, N1:0.12, p< .0001). The difference in EFS between pulmonary, skeletal and multi-system dissemination was significant: M1:0.29, M2:0.23; M3:0.12, p< .0001. The discrimination of M1 subgroups (M1a/M1b) was of prognostic relevance (p=.0050); M2 subgroups (M2a/M2b) discriminated outcome less clearly (p=.0457).

TNM staging is appropriate in Ewing tumors and should be incorporated in future trials.

Background: Intensive chemotherapy in sarcoma treatment may lead to weight loss, and in turn reduce dose intensity. A possible correlation between weight loss under treatment and outcome has never been analysed in sarcoma treatment. Ewing sarcoma (ES) patients undergoing intensive non-corticosteroid-containing chemotherapy commonly experience weight loss. The German Paediatric Oncology/Haematology Society (GPOH) Ewing sarcoma trials (CESS, EICESS) registry was analysed with respect to weight loss and outcome.

Patients and Methods: Body weight (BW) both at diagnosis and after a median of 12 courses of chemotherapy was available in 837 of 1549 ES patients, excluding amputees. Changes in BW were calculated as percentage of initial BW; outcome was determined as event-free-survival (EFS) from diagnosis according to Kaplan-Meier. Correlations of BW and outcome and potential confounders like disease stage or tumour volume were estimated uni- and multivariately.

Results: Weight loss was not correlated with inferior outcome: A loss of 10%–20% of BW was associated with a slightly more favourable outcome (3-year EFS 0.64 +/−0.106, N=82) than weight gain of 10%–20% of BW (3-year EFS 0.58 +/−0.098, N=97), p=0.101. Multivariate analyses revealed no confounders interfering with these results.

Conclusions: In 837 ES-patients analysed, weight loss did not correlate with inferior outcome. This should be observed in discussions about tube feeding and/or parenteral nutrition under cytostatic therapy. Future analyses of the prognostic impact of extreme under- or overweight both at diagnosis or under treatment are warranted in order to develop appropriate guidelines. Validity of this observation should be analysed for other solid tumours.

Supported by EC Biomed and Deutsche Krebshilfe