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Background: We investigated the impact of the implementation of a network of reference centers for sarcomas (NETSARC) on the care and survival of sarcoma patients in France since 2010., Patients and Methods: NETSARC (netsarc.org) is a network of 26 reference sarcoma centers with specialized multidisciplinary tumor boards (MDTBs), funded by the French National Cancer Institute (INCa) since 2010. Its aims are to improve the quality of diagnosis and care of sarcoma patients. Patients' characteristics, treatments, and outcomes are collected in a nationwide database. The objective of this analysis was to compare the survival of patients in three periods: 2010-2012 (non-exhaustive), 2013-2015, and 2016-2020., Results: A total of 43 975 patients with sarcomas, gastrointestinal stromal tumors (GISTs), or connective tissue tumors of intermediate malignancy were included in the NETSARC+ database since 2010 (n = 9266 before 2013, n = 12 274 between 2013 and 2015, n = 22 435 in 2016-2020). Median age was 56 years, 50.5% were women, and 13.2% had metastasis at diagnosis. Overall survival was significantly superior in the period 2016-2020 versus 2013-2015 versus 2010-2012 for the entire population, for patients >18 years of age, and for both metastatic and non-metastatic patients in univariate and multivariate analyses (P < 0.0001). Over the three periods, we observed a significantly improved compliance to clinical practice guidelines (CPGs) nationwide: the proportion of patients biopsied before surgery increased from 62.9% to 72.6%; the percentage of patients presented to NETSARC MDTBs before first surgery increased from 31.7% to 44.4% (P < 0.0001). The proportion of patients with R0 resection on first surgery increased (from 36.1% to 46.6%), while R2 resection rate decreased (from 10.9% to 7.9%), with a better compliance and improvement in NETSARC centers., Conclusions: The implementation of the national reference network for sarcoma was associated with an improvement of overall survival and compliance to guidelines nationwide in sarcoma patients. Referral to expert networks for sarcoma patients should be encouraged, though a better compliance to CPGs can still be achieved., (Copyright © 2024 The Author(s). Published by Elsevier Ltd.. All rights reserved.)

Background: Pelvic bone and/or soft tissue sarcoma removal surgeries are associated with a high rate of surgical site infection (SSI). The recommended antibiotic prophylaxis (ABP) duration is 24-48 h. We aimed to assess the impact of extended ABP (5 days) on the SSI rate and describe the microbiology of SSI in bone and/or soft tissue pelvic sarcomas., Methods: We retrospectively included all consecutive patients who underwent pelvic bone and/or soft tissue sarcoma removal surgery between January 2010 and June 2020., Results: We analyzed 146 patients with pelvic bone (45, 31%) or soft tissue (101, 69%). Sixty patients (41%) developed SSI. SSI occurred in 13/28 (46.4%) in the extended ABP group versus 47/118 (39.8%) in the standard group (p = 0.53). In multivariable analysis, risk factors for SSI were surgery duration (OR: 1.94 [1.41-2.92] per h), stay in postoperative ICU for more than 2 days (12.0 [2.8-61.3]), and shred or autologous skin flap (39.3 [5.8-409.5]). Extended ABP was not associated with SSI. SSI were mainly polymicrobial with Enterobacterales (57.4%) and Enterococcus (45%)., Conclusions and Discussion: Pelvic bone and/or soft tissue sarcoma removal surgery is highly prone to postoperative infection. Extending the ABP to 5 days does not reduce the level of SSI., (© 2023 Wiley Periodicals LLC.)

Background: The initial management of patients with sarcoma is a critical issue. We used the nationwide French National Cancer Institute-funded prospective sarcoma database NETSARC to report the management and oncologic outcomes in adolescents and young adults (AYAs) patients with sarcoma at the national level., Patients and Methods: NETSARC database gathers regularly monitored and updated data from patients with sarcoma. NETSARC was queried for patients (15-30 years) with sarcoma diagnosed from 2010 to 2017 for whom tumor resection had been performed. We reported management, locoregional recurrence-free survival (LRFS), progression-free survival (PFS), and overall survival (OS) in AYA treated in French reference sarcoma centers (RSC) and outside RSC (non-RSC) and conducted multivariable survival analyses adjusted for classical prognostic factors., Results: Among 3,227 patients aged 15-30 years with sarcoma diagnosed between 2010 and 2017, the study included 2,227 patients with surgery data available, among whom 1,290 AYAs had been operated in RSC, and 937 AYAs in non-RSC. Significant differences in compliance to guidelines were observed including pre-treatment biopsy (RSC: 85.9%; non-RSC 48.1%), pre-treatment imaging (RSC: 86.8%; non-RSC: 56.5%) and R0 margins (RSC 57.6%; non-RSC: 20.2%) (p < 0.001). 3y-OS rates were 81.1% (95%CI 78.3-83.6) in AYA in RSC and 82.7% (95%CI 79.4-85.5) in AYA in non-RSC, respectively. Whereas no significant differences in OS was observed in AYAs treated in RSC and in non-RSC, LRFS and PFS were improved in AYAs treated in RSC compared to AYAs treated in non-RSC (Hazard Ratios (HR): 0.58 and 0.83, respectively)., Conclusions: This study highlights the importance for AYA patients with sarcoma to be managed in national sarcoma reference centers involving multidisciplinary medical teams with paediatric and adult oncologists., (© 2023. The Author(s).)

Background: This French nationwide NETSARC exhaustive prospective cohort aims to explore the impact of systematic re-excision (RE) as adjuvant care on overall survival (OS), local recurrence free survival (LRFS), and local and distant control (RFS) in patients with soft tissue sarcoma (STS) with positive microscopic margins (R1) after initial resection performed outside of a reference center., Methods: Eligible patients had experienced STS surgery outside a reference center from 2010 to 2017, and had R1 margins after initial surgery. Characteristics and treatment comparisons used chi-square for categorical variables and Kruskall-Wallis test for continuous data. Survival distributions were compared in patients reexcised (RE) or not (No-RE) using a log-rank test. A Cox proportional hazard model was used for subgroup analysis., Results: A total of 1,284 patients had experienced initial STS surgery outside NETSARC with R1 margins, including 1,029 patients with second operation documented. Among the latter, 698 patients experienced re-excision, and 331 were not re-excised. Characteristics were significantly different regarding patient age, tumor site, tumor size, tumor depth, and histotype in the population of patients re-excised (RE) or not (No-RE). The study identified RE as an independent favorable factor for OS (HR 0.36, 95%CI 0.23-0.56, p<0.0001), for LRFS (HR 0.45, 95%CI 0.36-0.56, p<0.0001), and for RFS (HR 0.35, 95%CI 0.26-0.46, p<0.0001)., Conclusion: This large nationwide series shows that RE improved overall survival in patients with STS of extremities and trunk wall, with prior R1 resection performed outside of a reference center. RE as part of adjuvant care should be systematically considered., (© 2022. The Author(s).)

Background: The incidence of different soft tissue sarcoma (STS) histotypes among ethnic and geographic populations has not been comprehensively investigated., Methods: Data from 2013 to 2016 were obtained from national cancer registry databases in France and Taiwan. Liposarcoma (LPS), leiomyosarcoma (LMS), angiosarcoma (AS), synovial sarcoma (SS), and malignant peripheral nerve sheath tumor (MPNST) were selected as index STSs to estimate the age-standardized incidence rates (ASRs) and other clinical features between patients., Results: In total, 9398 patients (7148 from France and 2250 from Taiwan) were included. The ASRs of AS (5.4 vs. 2.8) and MPNST (2.0 vs. 1.0) were significantly higher in Taiwan; France had significantly higher ASRs for LPS (12.0 vs. 10.0), LMS (9.7 vs. 7.6), and SS (1.7 vs. 1.2). Patients in Taiwan with LMS or LPS were younger than their French counterparts. With regard to the distribution according to primary anatomic site, French patients had higher odds for extremity and truncal LMS (odds ratio [OR], 2.84; p < .001), AS (OR, 2.67; p < .001), MPNST (OR, 1.55; p = .027), and LPS (OR, 1.38; p < .001) and for breast AS (OR, 10.58; p < .001). Taiwanese patients had higher odds for liver AS (OR, 10.72; p < .001) and uterine LMS (OR, 3.21; p < .001). SS age and distribution according to primary anatomic site did not differ significantly between the French and Taiwanese populations., Conclusions: Significant differences in the incidence and clinical characteristics of index STS suggested that geographic (environmental) and ethnicity factors likely play a vital role in the pathogenesis of STS., (© 2022 American Cancer Society.)

The main issues pertaining to oncological surgery of the musculoskeletal system are tumour resection with sufficient margins, and the coverage of inherent defects. We report the use of a pedicled cutaneous groin flap as coverage for a trochanteric defect following an oncological resection of a grade III pleomorphic soft tissue sarcoma in a 75-year-old patient. This flap is not routinely used in the coverage of a defect in this region. The interest of this original process lies in its technical ease, and in the possibility of lifting the flap directly after resection, without changing the set-up., (Copyright © 2021 Elsevier Masson SAS. All rights reserved.)

Background: Hand sarcomas frequently suffer from a delayed diagnosis, and the current guidelines for their management are often not followed., Methods: The objectives of our study were to determine: (1) the rate of inadequate initial treatments; (2) the rates of mortality, recurrence, and complementary excision in a cohort of patients with a sarcoma of the hand who were treated at our reference center between 2000 and 2015., Results: The series comprised 26 patients (mean age 40 years). Of the 20 patients not initially treated at a reference center, 17 had inadequate initial treatment. Of the six patients treated at our center, one had inadequate initial care. Significantly more patients had inadequate initial care outside a reference center (p=0.0045). The cumulative probabilities of recurrence or metastases at 5 years were 15% and 30%, respectively. Survival by cumulative incidence was 71% at 5 years and 56% at 10 years., Conclusions: Sarcomas of the hand are a deadly pathology. All diagnostic uncertainty warrants referral of the patient to a reference center., Level of Proof: IV., (Copyright © 2021 Elsevier Masson SAS. All rights reserved.)

Background: The management of local sarcoma recurrence about the knee (at the distal femur or proximal tibia) is challenging. The objective of this study was to describe the locations of local sarcoma recurrences about knee megaprostheses, the treatments used for each type of recurrence, and the outcomes according to the treatment used., Hypothesis: The strategy for treating a local sarcoma recurrence about the knee must be based on the topography of tumour involvement., Materials and Methods: We included 13 patients who had local sarcoma recurrence about a knee megaprosthesis and were followed up for at least 2 years. The diagnosis was osteosarcoma in 10 patients and chondrosarcoma in 3 patients. The distal femur was involved in 11 patients and the proximal tibia in 2 patients. We classified the patients into two groups based on whether the tumour involved important structures or was at a distance within the soft tissues., Results: The recurrence was a nodule at a distance of important structures in 8 (57%) patients, in whom the mean time to recurrence was 15 months (range, 2-34 months). The remaining 5 (38.5%) patients had involvement of important structures in the popliteal fossa and a mean time to recurrence of 42 months (range, 16-80 months). Surgery was performed in 10 patients; the other 3 patients received palliative care due to rapid metastatic dissemination. Of the 6 patients who had surgery for a recurrence at a distance from important structures, 5 (83.3%) underwent isolated tumour resection with the goal of salvaging the limb and 1 (16.7%) underwent transfemoral amputation. Transfemoral amputation was performed in all 3 patients who had surgery for popliteal recurrences. Lung metastases were detected in 10 (77%) patients, 2 at the time of initial sarcoma diagnosis and 8 at the time of the local recurrence. At last follow-up, 5 patients were alive, including 2 (33.3%) of the 6 patients with recurrences at a distance from important structures and all 3 patients with popliteal recurrences. Of the 5 survivors at last follow-up, 2 had metastases., Conclusion: In patients with local recurrence of knee sarcoma, three presentations can be distinguished. When the local recurrence coincides with distant recurrence, failure to control the disease is likely and the local treatment should be designed as a component of palliative care. When no metastases are detected but the local recurrence is in contact with important structures, amputation is advisable at present given the complexity of limb-salvage surgery and high risk of further local recurrence. Finally, when no metastases are detected and the local recurrence is at a distance from important structures, limb-salvage surgery deserves consideration., Level of Evidence: IV, retrospective observational study., (Copyright © 2022 Elsevier Masson SAS. All rights reserved.)

The benefit of chemotherapy (CT) in rare bone sarcomas is not documented in prospective studies. Our retrospective study from the French sarcoma network for bone tumors ResOs was performed in adult patients (pts) from 1976 to 2014, with histologically verified diagnosis of leiomyosarcomas (LMS), undifferentiated pleomorphic sarcoma (UPS) or radiation-associated sarcomas of bone. The median follow-up was 4.7 years (95% CI: 3.7-6.5). Clinical features, treatment modalities and outcomes were recorded and analyzed from 145 pts (median age 53 years [range 20-87]). Site of disease was extremities (66%) or axial skeleton (34%), 111 (77%) presented with localized and potentially resectable disease. The most common histological subtypes were UPS (58%) and LMS (33%); 58% were high-grade tumors. Surgery was performed in 127 pts. In the 111 localized pts, 28 pts (25%) underwent upfront surgery or exclusive radiotherapy (RT; >50 Gy) without CT, whereas 83 pts (75%) received either neoadjuvant (n = 26) or adjuvant CT (n = 13) or both (n = 44). Neoadjuvant and adjuvant CT was mostly doxorubicin-based (95%/86%) and cisplatin-based (67%/63%). R0 resection was achieved in 59 pts, and a good histological response in 15 patients (25%). Adjuvant RT was performed in 24 (22%) pts. For the whole cohort (n = 145), the 5-year overall survival (OS) rate was 53% [42; 62]. In univariate analysis, age ≤ 60 was associated with a longer disease-free survival (DFS) (P = .0436). Neoadjuvant and adjuvant CT tended to be associated with better DFS (P = .056) with no significant impact on OS in this retrospective series., (© 2021 UICC.)

Soft tissue sarcomas (STS) are rare tumors accounting for less than 1% of human cancers. While the highest incidence of sarcomas is observed in elderly, this population is often excluded or poorly represented in clinical trials. The present study reports on clinicopathological presentation, and outcome of sarcoma patients over 90 recorded in the Netsarc.org French national database. NETSARC (netsarc.org) is a network of 26 reference sarcoma centers with specialized multidisciplinary tumor board (MDTB), funded by the French National Cancer Institute to improve the outcome of sarcoma patients. Since 2010, presentation to an MDTB, second pathological review, and collection of sarcoma patient characteristics and follow-up are collected in a database Information of patients registered from January 1, 2010, to December 31, 2016, in NETSARC were collected, analyzed and compared to the younger population. Patients with sarcomas aged >90 have almost exclusively sarcomas with complex genomics (92.0% vs. 66.3%), are less frequently metastatic (5.3% vs. 14·7%) at diagnosis, have more often superficial tumors (39.8% vs. 14.7%), as well as limbs and head and neck sites (75.2% vs. 38.7%) (all p < 0.001). Optimal diagnostic procedures and surgery were less frequently performed in patients over 90 (p < 0.001). These patients were less frequently operated in NETSARC centers, as compared to those of younger age groups including aged 80-90. However, local relapse-free survival, metastatic relapse-free survival and relapse-free survival were not significantly different from those of younger patients, in the whole cohort, as well as in the subgroup of operated patients. As expected overall survival was worse in patients over 90 (p < 0.001). Patients over 90 who were not operated had worse overall survival than younger patients (9.9 vs. 27.3 months, p < 0.001). Patients with STS diagnosed after 90 have distinct clinicopathological features, but comparable relapse-free survival, unless clinical practice guidelines recommendations are not applied. Standard management should be proposed to these patients if oncogeriatric status allows., (© 2019 The Authors. International Journal of Cancer published by John Wiley & Sons Ltd on behalf of UICC.)

Background: NETSARC (netsarc.org) is a network of 26 sarcoma reference centers with specialized multidisciplinary tumor boards (MDTB) aiming to improve the outcome of sarcoma patients. Since 2010, presentation to an MDTB and expert pathological review are mandatory for sarcoma patients nationwide. In the present work, the impact of surgery in a reference center on the survival of sarcoma patients investigated using this national NETSARC registry., Patients and Methods: Patients' characteristics and follow-up are prospectively collected and data monitored. Descriptive, uni- and multivariate analysis of prognostic factors were conducted in the entire series (N = 35 784) and in the subgroup of incident patient population (N = 29 497)., Results: Among the 35 784 patients, 155 different histological subtypes were reported. 4310 (11.6%) patients were metastatic at diagnosis. Previous cancer, previous radiotherapy, neurofibromatosis type 1 (NF1), and Li-Fraumeni syndrome were reported in 12.5%, 3.6%, 0.7%, and 0.1% of patients respectively. Among the 29 497 incident patients, 25 851 (87.6%) patients had surgical removal of the sarcoma, including 9949 (33.7%) operated in a NETSARC center. Location, grade, age, size, depth, histotypes, gender, NF1, and surgery outside a NETSARC center all correlated to overall survival (OS), local relapse free survival (LRFS), and event-free survival (EFS) in the incident patient population. NF1 history was one of the strongest adverse prognostic factors for LRFS, EFS, and OS. Presentation to an MDTB was associated with an improved LRFS and EFS, but was an adverse prognostic factor for OS if surgery was not carried out in a reference center. In multivariate analysis, surgery in a NETSARC center was positively correlated with LRFS, EFS, and OS [P < 0.001 for all, with a hazard ratio of 0.681 (95% CI 0.618-0.749) for OS]., Conclusion: This nationwide registry of sarcoma patients shows that surgical treatment in a reference center reduces the risk of relapse and death., (© The Author(s) 2019. Published by Oxford University Press on behalf of the European Society for Medical Oncology.)

Purpose: The use of adjuvant radiation in the treatment of soft-tissue sarcoma (STS) is equivocal in selected cases. Our objective was to compare the short-term outcomes in patients operated on for a local recurrence who had radiation for the primary tumour to those who were spared radiation., Methods: This was a retrospective study of 103 patients treated for a local recurrence: 48 (47%) with previous radiation and 55 (53%) without. Our primary outcome criterion was to identify the differences in the local treatment provided. Secondary outcomes were the cumulative incidence of a surgical site infection/wound complication (SSI/WC), variables associated with SSI/WC, and local recurrence., Results: Amputation and the incidence of re-operation were significantly more frequent in patients who received previous radiation compared to patients without previous radiation (27% vs 9%, p = 0.02, for amputation; 26% vs 36% at 2 years for SSI/WC, p = 0.049). Multivariable regression models found previous radiation (p = 0.049), arteriopathy (p = 0.012), location at lower limb (p = 0.09), and use of a flap (0.0048) associated with the risk of SSI/WC., Conclusions: Previous radiation is associated with an increased risk of amputation and reoperation for SSI/WC when treating a local recurrence. This information should be accounted for when deciding for the use of radiation.

We report the case of a patient with a BRCA2 germline mutation who developed a localized pleomorphic soft tissue sarcoma of the leg with poor prognostic features. BRCA2 germline mutations were not previously reported to be associated with pleomorphic sarcoma. BRCA2 loss-of-heterozygosity was found in the tumor, resulting in a complete BRCA2 loss-of-function. BRCA2 deficiency is associated with sensitivity to cisplatin-based chemotherapy in breast and ovarian cancer patients. We used a cisplatin-based chemotherapy. A rapid major partial response was obtained, which allowed a curative and conservative surgical resection of the sarcoma followed by adjuvant irradiation. This case illustrates that sarcoma patients may present unexpected but targetable genetic abnormalities and that BRCA2 loss-of-function may be targetable in sarcoma as it is associated with enhanced sensitivity to cisplatin. Our observation emphasizes the input of genomic medicine in clinical practice, its importance for treatment decisions, and the overlap between constitutional and somatic genetics., (Copyright © 2018 Southern Society for Clinical Investigation. Published by Elsevier Inc. All rights reserved.)

We asked whether there would be any difference between primary and revision modern cemented fixed hinge megaprosthesis of the distal femur in function and activity-related outcomes following treatment of a bone tumor. An identical custom-made fixed hinge cemented megaprosthesis with a hydroxyapatite collar was used in all cases. The main outcomes were joint-specific function, disease-specific activity, and health-related quality of life. Implant survival was also evaluated. Patients in the revision group performed slightly better than patients in the primary group on disease-specific (Toronto Extremity Salvage Score, p  = 0.033; Musculoskeletal Tumor Society, p  = 0.072) and health-related outcomes (Short Form 36 [SF-36] physical component, p  = 0.085; SF-36 mental component, p  = 0.069) but not on joint-specific outcomes (Knee Society Score, p  = 0.94). The cumulative probabilities of revision for any reason were 14.5% (7-25%) at 5 years with no statistically significant difference between primary and revision procedures ( p  = 0.77). In conclusion, patients undergoing a revision have similar joint-specific functional outcome but improved disease-specific and health-related outcomes. Implant survival are similar between groups., Competing Interests: None., (Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.)

Background: Sarcomas are rare but aggressive diseases. Specialized multidisciplinary management is not implemented for all patients in most countries. We investigated the impact of a multidisciplinary tumor board (MDTB) presentation before treatment in a nationwide study over 5 years., Patients and Methods: NETSARC (netsarc.org) is a network of 26 reference sarcoma centers with specialized MDTB, funded by the French National Cancer Institute to improve the outcome of sarcoma patients. Since 2010, presentation to an MDTB and second pathological review are mandatory for sarcoma patients in France. Patients' characteristics and follow-up are collected in a database regularly monitored and updated. The management and survival of patients presented to these MDTB before versus after initial treatment were analyzed., Results: Out of the 12 528 patients aged ≥15 years, with a first diagnosis of soft tissue and visceral sarcoma obtained between 1 January 2010 and 31 December 2014, 5281 (42.2%) and 7247 (57.8%) were presented to the MDTB before and after the initiation of treatment, respectively. The former group had generally worse prognostic characteristics. Presentation to a MDTB before treatment was associated with a better compliance to clinical practice guidelines, for example, biopsy before surgery, imaging, quality of initial surgery, and less reoperations (all P < 0.001). Local relapse-free survival and relapse-free survival were significantly better in patients presented to a MDTB before initiation of treatment, both in univariate and multivariate analysis., Conclusion: The compliance to clinical practice guidelines and relapse-free survival of sarcoma patients are significantly better when the initial treatment is guided by a pre-therapeutic specialized MDTB., (© The Author 2017. Published by Oxford University Press on behalf of the European Society for Medical Oncology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.)

Purpose: Local control of soft tissue sarcomas frequently involves adjuvant radiation to the surgical resection. When opting for post-operative radiation, care should be taken that radiation is started within some reasonable time after the surgery. We were interested to the proportion of patients who did not recieve optimal post-operative radiation and the variables associated., Methods: We retrospectively analyzed a series of 77 patients operated on for an extremity soft-tissue sarcoma and due for post-operative radiotherapy. Patients were considered to have received radiation optimally if radiation was started within 12 weeks of surgery. Variables associated with not receiving radiation optimally were looked for using univariable and multivariable regression models., Results: Overall, 26 patients (34%; 95% CI: 23-45%) did not receive radiation optimally. Twenty (26%) did not start radiation within the 12-weeks mark and six (8%) could not have radiation at all. The main reason identified for not receiving radiation on time was the occurrence of a wound complication (14 (54%) patients). An increased body mass index (OR: 1.14; 95% CI: 1.02-1.26; p = 0.02) and an older age (OR: 1.55; 95% CI: 1.18-2.14; p = 0.04; of note, the OR are for a 10 year change) were significantly associated with not receiving radiation optimally. Patients with a social fragility (p = 0.04), metastatic spread at presentation (p = 0.04), and increased duration of surgery (p = 0.02) were more likely to develop a wound complication., Conclusions: About 34% of patients do not receive optimal post-operative radiation treatment. Older and obese patients have a higher risk of not receiving radiation optimally. The decision for pre- or post-operative radiation should account for these findings.

Purpose: Ifosfamide is a cornerstone of chemotherapy in bone and soft-tissue sarcoma. Results of pharmacokinetic studies indicate that the optimal schedule of ifosfamide should be repeated doses over several days. With the development of 5-day infusion devices, we developed and evaluated a 5-day infusion regimen of ifosfamide in sarcoma patients in the outpatient setting., Methods: Sarcoma patients requiring chemotherapy after at least one doxorubicin-based line were enrolled in this study. Ifosfamide+mesna was administered as 1:1 concentration for a total of 6 g/m2 of each over 5 days (i.e. 1.2 g/m2 per day as continuous infusion) every 3 weeks. Patients were treated until progression or limiting toxicity, and salvage surgery was attempted when possible. An economic study was run comparing ifosfamide plus mesna as a 5-day infusion regimen and conventional Ifosfamide regimen., Results: Thirteen sarcoma patients were evaluable. The median number of cycles per patient was 6 (range, 1-8), for a total of 69 cycles. No acute encephalopathy or aggravation of renal function was noted. Acute grade 3 and 4 haematological toxicities were observed in 11.6 and 1.4% of patients, respectively without febrile neutropenia. Median time to progression survival and overall survival were 8.7 and 21.5 months, respectively. Total cost per cycle for a 2-m2 patient body surface area was ambulatory infusion=1,891 euros and conventional ifosfamide=6,256 euros., Conclusion: The combination of ifosfamide and mesna as a continuous infusion over 5 days is feasible and well tolerated in the outpatient setting using infusion device. Its very favourable cost-effectiveness invites to further develop this approach.

Background: The use of a megaprosthesis has become the method of choice for reconstruction after bone tumor resection at the knee. However, the long-term survival of megaprostheses is poor. In this study, we sought to identify factors that were associated with implant failure and amenable to interventions designed to improve implant survival., Methods: A retrospective review of the charts of ninety-one patients who had undergone resection of a tumor of the knee followed by reconstruction with a custom-made megaprosthesis was performed. The distal part of the femur was resected in fifty-six patients and the proximal part of the tibia, in thirty-five patients. The reconstruction was performed with an allograft-prosthesis composite in thirty-three patients and with metal or plastic sleeves in fifty-eight patients. Reconstruction of the extensor mechanism was necessary in all thirty-five patients with a tibial tumor., Results: The median duration of follow-up was sixty-two months. The extensor mechanism was significantly less likely to rupture when partial continuity had been preserved at the time of the resection. Intra-axial laxity (an arc of motion of >5 degrees in the frontal plane) was significantly more common when the prosthesis had an antirotation pin than when it did not have an antirotation pin (p = 0.0023). There was mechanical failure of ten allograft-prosthesis composites and ten sleeve reconstructions. Thirty-six patients had removal of at least one component of the prosthesis. When revision due to local tumor recurrence was excluded, the median duration of prosthetic survival was 130 months following the distal femoral resections and 117 months following the proximal tibial resections. The median duration of survival was 117 months for the allograft-prosthesis composites and 138 months for the sleeve reconstructions. Body weight and activity level were independent predictors of early revision., Conclusions: The long-term survival of the knee megaprostheses in this study was poor. Mechanical failure was multifactorial and the leading cause of revision. Use of allograft-prosthesis composites and use of bushings or an antirotation pin appeared to have no mechanical benefits. We recommend that weight control programs and advice about adapting their activity level be offered to patients preoperatively.

DNA double-strand break (DSB) repair pathways are implicated in the maintenance of genomic stability. However the alterations of these pathways, as may occur in human tumor cells with strong genomic instability, remain poorly characterized. We analyzed the loss of heterozygosity (LOH) and the presence of mutations for a series of genes implicated in DSB repair by non-homologous end-joining in five radiation-induced sarcomas devoid of both active Tp53 and Rb1. LOH was recurrently observed for 8 of the 9 studied genes (KU70, KU80, XRCC4, LIG4, Artemis, MRE11, RAD50, NBS1) but not for DNA-PKcs. No mutation was found in the remaining allele of the genes with LOH and the mRNA expression did not correlate with the allelic status. Our findings suggest that non-homologous end-joining repair pathway alteration is unlikely to be involved in the high genomic instability observed in these tumors.

A massive prosthesis and medial gastrocnemius muscle transfer were used to reconstruct the knee after extracapsular en bloc excision for bone sarcoma. Magnetic resonance images showed intraarticular involvement. This technique was used in nine patients, six men and three women aged 18 to 51 years, with primary malignant bone tumors of the knee. Extraarticular resection of the knee, including the patella, was done in every case. A knee prosthesis was implanted, and the extensor mechanism was reconstructed by transfer of the medial gastrocnemius muscle and pes anserinus tendons. All resections had negative margins. There were no local recurrences, but metastases occurred in two patients. Infection was the only major complication and was seen in two patients. The mean postoperative Musculoskeletal Tumor Society score was 61% (range, 36%-100%). The mean postoperative range of flexion was 62 degrees (range, 30 degrees-90 degrees), and the mean extensor lag was 12 degrees (range, 0 degrees-40 degrees). Three patients required a crutch to walk. The functional outcome was poor in the two patients whose proximal tibia was removed with the joint, suggesting that arthrodesis may be best in this situation. In properly selected patients, prosthesis and muscle flap reconstruction provides acceptable function and a good cosmetic result.

A case of epithelioid sarcoma localized in the palm of the left hand in a 32-year-old woman is reported. The tumor evolved for many years, clinically and microscopically simulating palmar fibromatosis. Epithelioid sarcoma is an uncommon malignant tumor, often misdiagnosed by surgeons and pathologists. It occurs in young patients and is often localized at the upper distal extremity. Microscopically, epithelioid sarcoma shows nodules manifesting fibrous hyaline cores with central necrosis. It contains epithelioid and spindle cells immunoreactive to keratin, epithelial membrane antigen and vimentin. Recurrences, lymph node metastases and lung metastases are frequent. Surgical literature tends to recommend wide "en bloc" excision or amputation, combined or not with adjuvant radiation therapy.

Abstract Background The initial management of patients with sarcoma is a critical issue. We used the nationwide French National Cancer Institute-funded prospective sarcoma database NETSARC to report the management and oncologic outcomes in adolescents and young adults (AYAs) patients with sarcoma at the national level. Patients and methods NETSARC database gathers regularly monitored and updated data from patients with sarcoma. NETSARC was queried for patients (15–30 years) with sarcoma diagnosed from 2010 to 2017 for whom tumor resection had been performed. We reported management, locoregional recurrence-free survival (LRFS), progression-free survival (PFS), and overall survival (OS) in AYA treated in French reference sarcoma centers (RSC) and outside RSC (non-RSC) and conducted multivariable survival analyses adjusted for classical prognostic factors. Results Among 3,227 patients aged 15–30 years with sarcoma diagnosed between 2010 and 2017, the study included 2,227 patients with surgery data available, among whom 1,290 AYAs had been operated in RSC, and 937 AYAs in non-RSC. Significant differences in compliance to guidelines were observed including pre-treatment biopsy (RSC: 85.9%; non-RSC 48.1%), pre-treatment imaging (RSC: 86.8%; non-RSC: 56.5%) and R0 margins (RSC 57.6%; non-RSC: 20.2%) (p