Sclerosing Epithelioid Fibrosarcoma (SEF) versus Low Grade Fibromyxoid Sarcoma (LGFMS): Presentation and outcome in the nationwide NETSARC+ series of 330 patients over 13 years.

Sclerosing Epithelioid Fibrosarcoma (SEF) and Low Grade Fibromyxoid Sarcoma (LGFMS) are ultrarare sarcomas sharing common translocations whose natural history are not well known. We report on the nationwide exhaustive series of 330 patients with SEF or LGFMS in NETSARC+ since 2010. PATIENTS AND METHODS: NETSARC (netsarc.org) is a network of 26 reference sarcoma centers with specialized multidisciplinary tumor boards (MDTB). Since 2010, (i) pathological review has been mandatory for sarcoma,and (ii) tumour/patients' characteristics have been collected in the NETSARC+ nationwide database. The characteristics of patients with SEF and LGFMS and their outcome are compared. RESULTS: 35/73 (48%) and 125/257(49%) of patients with SEF and LGFMS were female. More visceral, bone and trunk primary sites were observed in SEF (p < 0.001). 30% of SEF vs 4% of LGFMS patients had metastasis at diagnosis (p < 0.0001). Median size of the primary tumor was 51 mm (range 10-90) for LGFMS vs 80 (20-320) for SEF (p < 0.001). Median age for LGFMS patients was 12 years younger than that of SEF patients (43 [range 4-98] vs 55 [range 10-91], p < 0.001). Neoadjuvant treatment was more often given to SEF (16% vs 9%, p = 0.05). More patients with LGFMS were operated first in reference centers (51% vs 26%, p < 0.001). The R0 rate on the operative specimen was 41% in LGFMS vs 16% in SEF (p < 0.001). Median event-free survival (EFS) of patients with SEF and LGFMS were 32 vs 136 months (p < 0.0001). The median overall survival (OS) was not reached. Fifty-months OS was 93% vs 81% for LGFMS vs SEF (p = 0.05). Median OS was 77 months after first relapse, similar for SEF and LGFMS. In multivariate analysis, age, tumor size, metastasis at diagnosis were independent prognostic factors for OS in LGFMS. CONCLUSIONS: Although sharing close molecular alterations, SEF and LGFMS have a different natural history, clinical presentation and outcome, with a higher risk of metastatic relapse in SEF. Survival after relapse is longer than with other sarcomas, and similar for SEF and LGFMS.

The indications and donor-site morbidity of tibial cortical strut autografts in the management of defects in long bones.

Aims: The primary aim of this study was to determine the morbidity of a tibial strut autograft and characterize the rate of bony union following its use. Patients and Methods: We retrospectively assessed a series of 104 patients from a single centre who were treated with a tibial strut autograft of > 5 cm in length. A total of 30 had a segmental reconstruction with continuity of bone, 27 had a segmental reconstruction without continuity of bone, 29 had an arthrodesis and 18 had a nonunion. Donor-site morbidity was defined as any event that required a modification of the postoperative management. Union was assessed clinically and radiologically at a median of 36 months (IQR, 14 to 74). Results: Donor-site morbidity occurred in four patients (4%; 95% confidence interval (CI) 1 to 10). One patient had a stress fracture of the tibia, which healed with a varus deformity, requiring an osteotomy. Two patients required evacuation of a haematoma and one developed anterior compartment syndrome which required fasciotomies. The cumulative probability of union was 90% (95% CI 80 to 96) at five years. The type of reconstruction (p = 0.018), continuity of bone (p = 0.006) and length of tibial graft (p = 0.037) were associated with the time to union. Conclusion: The tibial strut autograft has a low risk of morbidity and provides adequate bone stock for treating various defects of long bones. Cite this article: Bone Joint J 2018;100-B:667-74.

Local recurrence after curettage treatment of giant cell tumors in peripheral bones: retrospective study by the GSF-GETO (French Sarcoma and Bone Tumor Study Groups).

BACKGROUND: Curettage is a well-established treatment modality for giant cell tumors of bone. The purpose of this retrospective study by the French Sarcoma and Bone Tumor Study Groups (GSF-GETO) was to analyze various tumor-specific and surgery-specific factors that could influence the rate of local recurrence. PATIENTS AND METHOD: Data was collected from patients with giant cells tumors of the appendicular skeletal who were treated by intralesional curettage. The hazard ratio for tumor recurrence was calculated for the different variables collected and a multifactorial analysis carried out. RESULTS: One hundred and ninety-three surgical procedures were included from nine centers. One hundred and seventy-one (89%) were primary tumors and 22 had been referred after one or more recurrences. The mean follow-up was 6 years and 11 months. The distal femur and proximal tibia were the most common locations: 42.5 and 34.2% of cases, respectively. The bone defect after curettage was filled in 176 cases (91.2%) and left empty in 16 cases. Local adjuvant treatment (phenol, alcohol, cryotherapy or combination treatment) was used in 39 cases (20.2%) and systemic adjuvant treatment used in 24 cases (calcitonin 11 and zoledronic acid 13). Local recurrence occurred in 71 cases (36.8%). Risk factors for local recurrence were an empty defect, a defect filled with autograft, and patients treated before 2005. Multivariate analysis showed that the only risk factors for local recurrence were a surgical procedure before 2005 (odds ratio 3.6 (95% CI: 1.2, 7.9) P=0.017) and a bone defect filled with autograft (odds ratio 3.9 [95% CI: 1.3, 11.6] P=0.013) CONCLUSION: Neither tumor-specific nor surgery-specific factors such as adjuvant treatment were found to be as risk factors for local recurrence after curettage of giant cell tumors in the appendicular skeleton. As recently reported, high-quality local curettage is probably the most effective technique to prevent local recurrence. The current study suggests that two factors associated with more recent management of these tumors in France, high-speed burring and centralization to skilled surgical teams, can improve the quality of curettage. LEVEL OF EVIDENCE: 4, retrospective cohort study.

Measles immunity and measles vaccine acceptance among healthcare workers in Paris, France.

BACKGROUND: In Europe, including France, a measles outbreak has been ongoing since 2008. Unprotected healthcare workers (HCWs) may contract and spread the infection to patients. AIM: The objective of this study was to evaluate HCWs' measles immunity and vaccine acceptance in our setting. METHODS: In a survey-based study conducted in three university hospitals in Paris, 351 HCWs were included between April and June 2011. The following data were collected at enrolment: age, hospital unit, occupation, history of measles infection and vaccination, previous measles serology and acceptance of a measles vaccination in case of seronegativity. Sera were tested for the presence of specific anti-measles IgG antibodies using the CAPTIA(®) measles enzyme-linked immunosorbent assay. FINDINGS: The mean age of the participating HCWs was 36 years (range: 18-67) and 278 (79.2%) were female. In all, 104 four persons (29.6%) declared a history of measles, and 90 (25.6%) declared never having received a measles vaccination. Among the 351 HCWs included in the study, 322 (91.7%) were immunized against measles (IgG >90 mIU/mL). The risk factors for not being protected were age [18-29 years, adjusted odds ratio: 2.7 (95% confidence interval: 1.1-6.9) compared with ≥30 years], no history of measles infection or vaccination. The global acceptance rate for a measles vaccination, before knowing their results, was 78.6%. CONCLUSION: In this cohort of HCWs, 8.3% were susceptible to measles; the group most represented were aged <30 years. Acceptance of the measles vaccine was high. A vaccination campaign in healthcare settings should target specifically healthcare students and junior HCWs.

Monitoring the quality of total hip replacement in a tertiary care department using a cumulative summation statistical method (CUSUM).

The purpose of this study was to define immediate post-operative 'quality' in total hip replacements and to study prospectively the occurrence of failure based on these definitions of quality. The evaluation and assessment of failure were based on ten radiological and clinical criteria. The cumulative summation (CUSUM) test was used to study 200 procedures over a one-year period. Technical criteria defined failure in 17 cases (8.5%), those related to the femoral component in nine (4.5%), the acetabular component in 32 (16%) and those relating to discharge from hospital in five (2.5%). Overall, the procedure was considered to have failed in 57 of the 200 total hip replacements (28.5%). The use of a new design of acetabular component was associated with more failures. For the CUSUM test, the level of adequate performance was set at a rate of failure of 20% and the level of inadequate performance set at a failure rate of 40%; no alarm was raised by the test, indicating that there was no evidence of inadequate performance. The use of a continuous monitoring statistical method is useful to ensure that the quality of total hip replacement is maintained, especially as newer implants are introduced.

Intercalary defects reconstruction of the femur and tibia after primary malignant bone tumour resection. A series of 13 cases.

INTRODUCTION: Performing intercalary segment reconstruction after malignant bone tumour resection results in both mechanical and biological challenges. Fixation must be solid enough to avoid short-term or mid-term mechanical failure. The use of an allograft or autograft must ensure long-term survival of the reconstruction. The goal of this study was to analyse the clinical and radiological outcomes of these reconstructions. PATIENTS AND METHODS: Thirteen patients were operated on eight femurs and five tibias. The median age was 20 years old (range 14-50). The most common diagnosis was osteosarcoma. The median resection length was 15cm (Q1-Q3: 6-26). A plate was used for fixation in nine cases and an intramedullary locked nail in four cases. An isolated bone autograft was used in two cases, an isolated bone allograft in one case, a dual autograft-allograft composite in six cases, and vascularised fibula and allograft combination in four cases. RESULTS: The cumulative probability of union was 46% (95% CI: 0-99%) at 1 year; at the final follow-up, union was achieved in 12 patients (92%). Because of non-unions, 13 iterative procedures were needed to obtain these results. A non-displaced fracture of a cuboid-shaped tibial graft occurred in one patient, which was treated conservatively. Three infections occurred. DISCUSSION: The results of intercalary segmental defects reconstruction after bone tumour resection were good, both from an oncologic and radiological point-of-view. One or more iterative procedures are sometimes needed to finally obtain bone union. We prefer to use a free rectangular cuboidal tibial graft since reconstruction with a vascularised autograft is technically more difficult. The choice of fixation methods is still controversial and no approach was found to be superior. LEVEL OF EVIDENCE: Level IV. Retrospective study.

Pelvic chondrosarcomas: surgical treatment options.

INTRODUCTION: Chondrosarcoma (CS) is a primary malignant bone tumor with cartilaginous differentiation. The only available treatment is carcinological surgical resection since the usual adjuvant treatments are ineffective. The pelvic location creates specific technical difficulties both for exeresis and reconstruction. Our objective was to evaluate the carcinological and functional outcomes of inter-ilioabdominal amputation and conservative surgery. MATERIALS AND METHODS: We retrospectively studied 59 cases of pelvis chondrosarcoma managed in our department between 1968 and 2003. Demographic, anatomopathological, surgical and survival data were analyzed. Survival was estimated by the Kaplan-Meier curves and the cumulative incidence method. Multivariate analysis was used to identify all possible independent prognostic variables. RESULTS: There were 33 men and 26 women, with an average age of 48 years. The average follow-up duration was 94 months. Eleven patients had a grade 1 chondrosarcoma, 36 a grade 2 chondrosarcoma, five were grade 3, and seven were dedifferentiated chondrosarcoma. Eleven patients underwent an inter-ilioabdominal disarticulation, and 48 had a more conservative surgery. Resection margins proved healthy in 46 patients (78%). Eighteen patients (31%) had a local recurrence, and 12 (20%) had metastases. At last follow-up, 30 patients (51%) were still alive without any sign of recurrence. Twenty-three patients (39%) died from the disease. Multivariate analysis showed that margin invasion was associated with a definitely increased local recurrence rate. A high tumoral grade was correlated with a greater risk of metastases occurrence. These two last factors (margin status and tumor grade) as well as acetabulum involvement were correlated with a reduced survival rate. Function was better among patients treated by conservative surgery, and among them, even better when the peri-acetabular area remained intact. Our study confirmed that resection margins quality is a major prognostic factor both for local control and for survival. On the other hand, local recurrence is an adverse survival prognosis factor and is itself correlated with resection margins quality. Peri-acetabular chondrosarcoma location (in zone 2) appears to be a poor oncological prognosis factor since, in this location, obtaining healthy margins appears particularly difficult. Compared to resection, inter-ilioabdominal amputation did not prove its superiority concerning resection margins quality or survival. However, resection guaranteed a better functional outcome. CONCLUSION: Chondrosarcoma of the pelvic girdle remains of worse prognosis than peripheral bones chondrosarcoma since the critical prognosis factor is the resection margins quality. This location, and especially the peri-acetabular zone, poses difficult specific technical problems when conservative surgery is selected. Various imaging techniques should help better envision tumor resection extent. Inter-ilioabdominal amputation should only be resorted to in non-metastatic patients, when the tumor does not seem to be removable with sufficient healthy margins guarantee, or when local conditions make it impossible to hope for a good quality reconstruction. LEVEL OF EVIDENCE: Level IV; therapeutic retrospective study.

Surgical management of pelvic primary bone tumors involving the sacroiliac joint.

INTRODUCTION: Pelvic primary malignant bone tumours, especially when involving the sacroiliac joint are difficult to treat. Abdominoperineal amputations are today used, only in life-threatening situations. HYPOTHESIS: A precisely planed surgical technique can save the affected extremity without compromising the resection quality and subsequent patient survival. OBJECTIVE: To assess the procedures used for resection and reconstruction of bone tumours invading the sacroiliac joint as well as their effects on cancer outcome and functional results. MATERIALS AND METHODS: This is a continuous and retrospective analysis of 24 patients treated between 1986 and 2003. Six tumours affected the sacral body and 18 tumours involved the wing of the ilium. The joint articular surface was invaded in only six cases. Seventeen patients received neoadjuvant chemotherapy. The procedure was performed through an enlarged iliac crest incision, giving access to two sections of the pelvic ring. Six cases required neurological sacrifice. Initial tumour grading was based on the Enneking classification, and the functional results, on the Musculoskeletal Tumour Society (MSTS) scoring system. RESULTS: The average operation lasted 5.27 hours. Reconstruction was performed with bone autograft and instrumentation. Resection was large with adequate margins 11 times, marginal 12 times, and contaminated once. Average follow-up was 4.77 years. The 5-year survival rate was 50%. Twelve patients either died from their disease or were in the metastatic stage at final follow-up. Survival was linked to the quality of resection and initial tumour staging. Hemisacrectomy did not affect patient survival. Local recurrences had a poor prognosis with eight cases of secondary metastases out of 11. Bone healing occurred in 13 patients, 10 of whom survived. Of the 12 patients who survived and were in complete remission at final follow-up, the average MSTS score was 61%. The score was at 38.6% in cases involving neurological sacrifice, and at 77.1% for the rest of the group. It was at 64% in healed cases and 13% in nonunion cases. DISCUSSION: The survival of patients presenting with a sacroiliac joint tumour is substantially related to both tumour histology and resection quality. Local recurrences carry a poor prognosis with a high rate of secondary metastatic dissemination. In situations where disease control can be achieved, the proposed method of reconstruction allows, satisfactory bone healing and fair functional recovery, provided no major neurological sacrifice has taken place. LEVEL OF EVIDENCE: level IV: Retrospective Therapeutic Study.

[Surveillance of operative site infections in an orthopedic and traumatology surgery department: an example of methodology]. / Proposition d'un protocole de suivi des infections avérées de site opératoire en chirurgie orthopédique et traumatologique.

PURPOSE OF THE STUDY: A program for the prevention of nosocomial infections, including operative site infections (OSI) is a legal obligation in France. According to the CDC, in orthopedic surgery, nosocomial infection is defined as any infection occurring within 30 days of operation, or within one year in the event of material implantation. No surveillance system has been validated and the rate of OSI is unknown in orthopedic surgery. We report the number of OSI observed during a three year period in our unit and describe the characteristic features. MATERIAL AND METHODS: Data were collected from the bacteriology reports on operative site samples with a positive culture. A group of specialists determined the infective nature of the germ and the nosocomial nature of the OSI. Clinical and bacteriological data were noted on a standard datasheet used for prospective follow-up of the number of cases and data processing. During a three-year period (2000, 2001, 2002), among 9397 orthopedic and traumatology operations performed, 86 OSI were identified. Mean patient age was 58 years and mean body mass index was 25.7. The ASA score was >or=II for 72% of patients. RESULTS: The OSI involved an arthroplasty in 23 cases, a traumatology procedure in 21, and tumor treatment in 24. The diagnosis was established within 30 days of operation for 75% and after discharge from hospital in 65.4%. Single-germ infections predominated (n=59). Staphylococcus aureus was isolated in 80.23% of infections. For tumor surgery, the statistically more frequent multiple-germ infections associated coagulase negative Staphylococcus and Gram-negative bacilli. There were six OSI-related deaths. DISCUSSION: Two criticisms can be formulated concerning our surveillance system. First, infections with no identified germ could be missed. The frequency of such infections has been estimated at 2.8 to 19% by different authors. Although patients are automatically recalled for consultation, we were unable to determine the number of patients lost to follow-up at one year. It was thus not possible to determine a precise rate of OSI. Data in the literature have not demonstrated any system providing an exhaustive surveillance, particularly because of the long postoperative period after material implantation. Excepting tumor surgery, Staphylococcus aureus infections predominated. Factors of risk of OSI include the patient's general status, particularly for arthroplasty. We had a mortality rate of 7% for our OSI, corroborating earlier studies and illustrating the severity of such infections. CONCLUSION: Surveillance of OSI in orthopedic surgery requires the development of a system responding to the problem of a long observation period. It would be important to know the precise number of OSI and their characteristic features in order to develop comparison tools.

[Twenty nine shoulder reconstructions after resection of the proximal humerus for neoplasm with mean 7-year follow-up]. / Les reconstructions après résection tumorale de l'extrémité supérieure de l'humérus.

PURPOSE OF THE STUDY: Techniques available for shoulder reconstruction after resection of a tumor of the proximal humerus include scapulohumeral arthrodesis, humerus prosthesis with or without an allograft, inverted prostheses, and massive allografts. The purpose of this study was to review clinical and radiological outcomes in a series of 29 patients (20 men and 9 women) who underwent resection-reconstruction of the proximal humerus and to establish from these cases a decision making algorithm for therapeutic indications as a function of tumor invasion. MATERIAL AND METHODS: The tumors were 20 chondrosarcomas, five osteosarcomas, two Ewing sarcomas and one malignant hemangiopericytoma. In 17 patients epiphyso-metaphyseal or epiphyso-metaphyso-diaphyseal resection was performed with preservation of the abductor muscles (type S34A or S345A according to the Musculoskeletal Tumor Society classification). For 12 patients epiphyso-metaphyseal or epiphyso-metaphyso-diaphyseal resection was performed without preservation of the abductor muscles (type S34B or S345B). Reconstruction was achieved using a centromedullary cemented nail in one patient, scapulohumeral arthrodesis in three, a massive humerus prosthesis in 15, and composite humerus prosthesis in three and an inverted prosthesis in seven. The functional score of the Musculoskeletal Tumor Society (MSTS) and standard x-rays were used to assess outcome. RESULTS: Mean follow-up was 85 months (range 16-300). The mean MSTS score was 88% for inverted prostheses, 76% for composite prostheses, 72.6% for massive prostheses, 75% for scapulohumeral arthrodeses, 67% for massive prostheses, and 80% for cemented centromendullary nail. Five patients died from their malignant disease and one from another cause. Four patients are alive but with active disease after a mean follow-up of 108 months and 19 patients (65.5%) are alive and free of locoregional recurrence or metastasis after a mean 83.5 months. We had 28 complications. Glenohumeral instability was the most frequent (11 cases). DISCUSSION: Resection of the upper portion of the humerus should be performed to achieve cancerologically satisfactory tumor resection and enable shoulder resection, if possible, with preservation of a viable and functional abductor system. The functional outcome after such reconstruction depends on the type of bony resection, but also on the sacrifice of the rotator cuff and the deltoid muscle. In light of our experience and results in the literature, we advocate, despite the small number of cases for the different reconstructions, the following decision-making algorithm after resection of the proximal humerus without joint invasion: when the resection removes the rotator cuff and the deltoid (or the axillary nerve), there are two options: scapulohumeral arthrodesis or massive humerus prosthesis for patients who do not desire a complex therapy with a long postoperative period; when the resection preserves the rotator cuff and/or the deltoid muscle, reconstruction can be achieved with a composite (inverted or not) prosthesis with suture of the cuff tendons. We prefer the inverted composite prosthesis; if the deltoid muscle can be preserved but not the rotator cuff, the composite inverted prosthesis appears to be the most logical solution, but scapulohumeral arthrodesis can be proposed in selected cases.