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Carbon-fiber (CF) plates are a promising alternative to metal plates. However, reported experience in orthopaedic oncology remains limited. The aim of this study was to identify complications of patients with bone tumors treated with CF plates. Between February 2015 and May 2021, 13 centers retrospectively registered patients with bone tumors that were reconstructed using CF plates. Complications were identified, and timing and etiology of complications were noted. Similar complications were tabulated and classified based on mechanical, non-mechanical and paediatric complications. Mechanical complications included: (1) aseptic loosening or graft-host non-union, and (2) structural complications. Non-mechanical complications included: (3) soft tissue complications, (4) infection and (5) tumor progression. Specific paediatric complications included (6) growth arrest resulting in longitudinal or angular deformity. Ninety-six patients were included with a median follow-up time of 35 months. In total, 22 (23%) patients had complications. Mechanical complications included: 1 (1%) aseptic loosening, 2 (2%) non-unions, and 7 (7%) structural complications. Non-mechanical complications included 1 (1%) soft tissue complication, 4 (4%) infections and 5 (5%) tumor progressions. Paediatric complications occurred in 2 (2%) patients. This study suggests CF plates are safe to use in demanding reconstructions after bone tumor resections, presenting a seemingly low complication profile.

A simple prognostic scoring system for non metastatic osteosarcoma of the extremity

Combined positor emission cronography using Gallium 68 with computer tomography and use of antibiotics containing Hydrops Apatite pellets in the treatment of bone infections

Background: According to retrospective osteosarcoma series, ABCB1/P-glycoprotein (Pgp) overexpression predicts for poor outcomes. A prospective trial to assess a risk-adapted treatment strategy using mifamurtide in Pgp+ patients was performed. Methods: This was a phase 2, multicenter, uncontrolled trial including patients 40 years old or younger with nonmetastatic extremity high-grade osteosarcoma stratified according to Pgp expression. All patients received high-dose methotrexate, doxorubicin, and cisplatin (MAP) preoperatively. In Pgp+ patients, mifamurtide was added postoperatively and combined with MAP for a good histologic response (necrosis ≥ 90%; good responders [GRs]) or with high-dose ifosfamide (HDIFO) at 3 g/m2/d on days 1 to 5 for a histologic response < 90% (poor responders [PRs]). Pgp– patients received MAP postoperatively. After an amendment, the cumulative dose of methotrexate was increased from 60 to 120 g/m2 (from 5 to 10 courses). The primary end point was event-free survival (EFS). A postamendment analysis was performed. Results: In all, 279 patients were recruited, and 194 were included in the postamendment analysis: 70 (36%) were Pgp–, and 124 (64%) were Pgp+. The median follow-up was 51 months. For Pgp+ patients, 5-year EFS after definitive surgery (null hypothesis, 40%) was 69.8% (90% confidence interval [CI], 62.2%-76.2%): 59.8% in PRs and 83.7% in GRs. For Pgp– patients, the 5-year EFS rate was 66.4% (90% CI, 55.6%-75.1%). Conclusions: This study showed that adjuvant mifamurtide, combined with HDIFO for a poor response to induction chemotherapy, could improve EFS in Pgp+ patients. Overall, the outcomes compared favorably with previous series. Mifamurtide and HDIFO as salvage chemotherapy are worth further study.

Introduction The role of chemotherapy for patients with dedifferentiated chondrosarcoma (DDCS) is still under discussion. Here, we present the outcome in patients with DDCS treated with intensive chemotherapy from the EUROpean Bone Over 40 Sarcoma Study. Materials and methods The chemotherapy regimen included doxorubicin, ifosfamide and cisplatin. Postoperative methotrexate was added in case of poor histological response. Toxicity was graded based on the National Cancer Institute expanded common toxicity criteria, version 2.0, and survival was analysed using Kaplan-Meier curves, log-rank tests and univariate Cox regression models. Results Fifty-seven patients with DDCS (localised, 34 [60%]; metastatic, 23 [40%]) aged 42–65 years were included. Surgical complete remission (SCR) was achieved in 36 (63%) patients. The median overall survival (OS) was 24 months (95% confidence interval, 22–25), and the 5-year OS was 39%. Patients with extremity localisation had a 5-year OS of 49% compared with 29% in patients with a central tumour (P = 0.08). Patients with localised disease had a 5-year OS of 46%, whereas patients with metastatic disease had a 5-year OS of 29% (P = 0.12). Patients in SCR had a 5-year OS of 49%, whereas patients not in SCR had a 5-year OS of 23% (P = 0.004). Chemotherapy toxicity was considerable but manageable. There was no treatment-related death, and 39 (70%) patients received ≥6 cycles of the planned nine chemotherapy cycles. Conclusions Adding intensive chemotherapy to surgery for treatment of DDCS is feasible and shows favourable survival data compared with previous reports. With the limitations of data from a non-controlled trial, we conclude that chemotherapy could be considered in the management of patients aged >40 years.

Background Large, malignant bone tumors and revision limb salvage procedures often result in the resection of extensive lengths of the involved bone segment, leaving a residual segment of bone that may be too short to support a standard intramedullary stem for endoprosthetic reconstruction. Telescope allografting, in which an allograft is used to augment the remaining bone segment by telescoping it into the residual bone segment, was described for situations in which residual bone stock is insufficient after tumor resection or prosthetic revision. Apart from one study that first described the procedure [15], there are no other studies reporting the outcome of this telescopic concept for restoring bone stock. Questions/purposes For patients younger than 18 years who underwent the telescopic allograft technique to augment a short segment of the proximal femur after resection of bone sarcomas who also underwent endoprosthesis reconstruction of the distal femur, we asked: (1) What is the survivorship free from removal of the telescopic allograft and the endoprosthetic stem at 7 years after surgery? (2) What proportion of these reconstructions will heal to the host bone without delayed union or nonunion? (3) What is the functional outcome based on the Musculoskeletal Tumor Society (MSTS) score? Methods We retrospectively studied our institutional database and identified 127 patients younger than 18 years who underwent surgery for a primary malignant bone tumor of the distal femur between December 2008 and October 2018. After excluding 16 patients undergoing amputation and rotationplasty and 57 patients undergoing recycled autograft/allograft reconstruction, 54 patients who underwent primary or revision distal femur endoprosthesis reconstruction were identified. Among these patients, we considered 15 patients who underwent telescopic allograft augmentation of the femur for analysis. One patient was lost to follow-up before 2 years but was not known to have died, leaving 14 for analysis at a median (range) 49 months (24 to 136 months) of follow-up. The indications for telescopic allograft augmentation of the femur in our institution were a proximal femur length of less than 120 mm after resection or resection of more than two-thirds of the total length of the femur. Ten of 14 patients underwent telescopic allograft augmentation as a revision procedure (distal femur resorption in five patients, endoprosthesis stem loosening in three patients, implant fracture in one patient, and infection in one patient), and the remaining four patients underwent telescopic allograft augmentation as a primary limb salvage procedure for large malignant bone tumors of the distal femur. The histologic diagnosis in all patients was osteosarcoma. At the time of telescopic allograft augmentation and reconstruction, the median age of the patients was 14 years (7 to 18 years). The size and the type of bone allograft to be used (femoral shaft or proximal femur) was planned before surgery, with consideration of the extent of resection, level of osteotomy, diameter of the host bone at the osteotomy site, and approximate diameter of the endoprosthesis stem to be used. The segment of the cylindrical allograft used for telescoping was thoroughly washed, prepared, and impacted onto the native femur to achieve telescoping and overlap. Serial digital radiographs were performed once a month for the first 6 months after the procedure, every 2 months until 1 year, and then every 6 months thereafter. Two surgeons in the department (at least one of which was involved in the surgery) retrieved and reviewed clinical notes and radiographs to determine the status of the telescopic allograft and endoprosthesis stem. We defined delayed union as radiological union at the osteotomy site more than 6 months after the procedure without additional surgery; we defined nonunion as no radiological evidence of callus formation at the osteotomy site 9 months after the procedure, necessitating additional surgery. The reviewers did not disagree about the definition of healing time. None of the patients missed radiographic follow-up. Kaplan-Meier survivorship free from removal of telescopic allograft and the endoprosthesis stem at 7 years after surgery was estimated. Patient function was assessed using the 1993 version of the MSTS [9], as determined by chart review of the institutional database performed by one of the surgeons from the department. Results The survivorship free from removal of the telescopic allograft and endoprosthesis stem at 7 years after surgery was 80% (95% confidence interval 22% to 96%). The allograft united with the host bone in 100% (14 of 14) of the patients. Though 21% (3 of 14) had delayed union, no nonunions were seen. The median (range) MSTS score at the final follow-up interval was 27 (22 to 30). Conclusion Although this is a small group of patients, we believe that allograft segments help augment short bone stock of the proximal femur after long-segment resections, and the telescopic technique seems to be associated with a low proportion of nonunion or delayed union, which is one of the most common complications of allografts. Maintaining an adequate length of the proximal femur is important in preserving the hip, and this technique may be especially useful for young individuals who may undergo repeated revision procedures. Level of evidence Level IV, therapeutic study.

Background: Acquired hip deformities in patients affected by hereditary multiple exostosis (HME) may incur in early hip osteoarthritis and functional limitation requiring primary total hip arthroplasty (THA). Characteristic coxo-femoral joint dysmorphisms in HME may pose a challenge for the orthopaedic surgeon. Here we report our experience in a series of patients with HME treated in our hospital with THA. Methods: With a mean follow-up of 5 years, 10 primary THAs were reviewed; proximal femur deformities, acetabular dysplasia and joint osteoarthritis has been assessed through x-rays and CT-scan evaluation. In all cases hemispheric press-fit cups were used; 4 stem had metaphyseal engagement, 5 had proximal diaphyseal engagement and 1, with anatomical geometry, had metaphyseal fixation. 2 cases required stem cementation, 3 modular neck and 1 lateralised. The clinical data, complications and clinical outcomes, were recorded and analysed. Results: The mean Harris Hip Score (HHS) increased from 34 preoperative to 86 postoperative; preoperative mean neck shaft angle (NSA) was 150°, head/neck ratio 0.6, offset 31 mm; Wiberg angle 28°, Sharp angle 38°, 1 patient had subluxation grade 4 according to Crowe, 8 hips showed osteoarthritis (Tönnis grade ⩾2 ); 5 femurs were classified as Dorr type C, 2 as type B and 3 as type A. Perioperative complications were not observed. Conclusions: Primary THA in HME significantly improved clinical and functional outcomes. Press-fit cup fixation together with metaphyseal and proximal diaphyseal stem engagement on reliable bone quality femur, represents a valid option in HME patients with normal acetabular morphology, wide broaden neck and valgus NSA.

Aims: To evaluate the diagnostic accuracy of SSX and SSX::SS18 antibodies in decalcified surgical specimens and outcome of synovial sarcomas (SS) of bone. Methods and results: Twenty-five cases were classified as bone SS (prevalence 0.32% among malignant primary bone sarcoma). Median age was 34 years (range = 9–79). Twenty-four of 25 patients presented with non-metastatic tumours, one with lung metastases. The majority of tumours involved the long bones of extremities with metaphyseal origin. Mean size of the tumour was 7.1 cm. Twenty cases (80%) were monophasic and five (20%) were biphasic. SS18::SSX fusion-specific antibody had 92% sensitivity and 99% specificity for primary bone SS, whereas SSX C-terminus antibody had 100% sensitivity and 94% specificity. Fluorescence in-situ hybridisation (FISH) analysis was feasible in nine (36%) cases and detected SS18 rearrangement in all nine cases. All patients underwent surgical removal of their primary tumour, with adequate margins in 18 (72%) patients. Chemotherapy with metothrexate, cisplatin, doxorubicin and ifosfamide was used in the seven patients. Two patients with inadequate surgical margins received radiotherapy. With a median follow-up of 80 months (range = 6–428), 5- and 10-year overall survival (OS) was 66.6% and 47.9%, respectively, and 5 and 10 years’ disease-free survival (DFS) was 36.8% [95% confidence interval (CI) = 18.0–55.7%] and 32.2% (95% CI = 14.6–51.2%), respectively. A significant improvement in 10 years’ DFS in patients undergoing chemotherapy compared with patients who did not was observed (P = 0.039). Conclusions: Our series highlights the utility of SS18::SSX fusion-specific and SSX C-terminus antibodies to support the diagnosis of SS. Adjustment chemotherapy was associated with improved prognosis in this series.

Aims The aim of this study is to assess outcomes of patients ≤12 years who undergo Stanmore noninvasive extendible endoprosthetic replacement of the distal femur (DF NIEPR). Patients and methods A total of 101 children (mean age 9.6 years) were included. All complications which required further surgery were recorded. Clinical and functional outcomes were evaluated with Musculoskeletal Tumor Society (MSTS) scores at a mean follow-up of 64 months (range 6-174). Results Thirty-one (30.7%) patients died at a mean of 33 months. Forty had prosthesis removed after a mean of 43 months (range, 7-103). Attaining of the full lengthening potential before skeletal maturity was the most frequent reason for revision surgery, particularly in those with smaller lengthening potential (P = 0.039). Implant survival rate for other causes was 61.7% at 5 years and 45.0% at 10 years. At final follow-up mean MSTS score was 26 (range, 13-29). Twenty-two (21.5%) patients had a final limb-length discrepancy (LLD) > 2 cm. Conclusions DF NIEPR produces a good functional outcome, with the prevention of major LLD at skeletal maturity in the majority of the cases. We suggest patient selection criteria to account for the stage of the disease due to the high cost of the NIEPR, and high percentage requiring revision, and a 60% mortality rate in those patients presenting with distant disease burden.

The ‘leave me alone’ bone lesions are very classical, and, as indicated by their name, do not require any further investigation. There are very typical cases, and there are also more difficult ones, and they can be especially difficult to manage if the patient has a known cancer.

Aim To determine activity and toxicity of high-dose ifosfamide (HDIFO) in recurrent or advanced Ewing sarcoma family tumors (EFT). Methods Thirty-seven EFT patients [median age 17 years (6–45 years)] previously treated with chemotherapy regimens including standard dose ifosfamide were enrolled. HDIFO was administered for metastatic recurrent disease in 33 patients and for progression during neoadjuvant chemotherapy in 4 patients. All patients who received two courses of 15 g/m2 ifosfamide were evaluable for radiographic response assessed according to RECIST criteria. Results Transient Grade 4 neutropenia and thrombocytopenia in 97% and 54% HDIFO courses respectively and severe CNS toxicity in one patient were observed. Thirty-five patients were evaluable: 12 (34%) had complete (2) or partial (10) response, 11 (32%) had stable disease, and 12 (34%) had progression. Conclusions In patients with relapsed or advanced EFT previously treated with standard dose ifosfamide HDIFO is active and it should be considered a treatment option. Pediatr Blood Cancer 2009;52:581–584. © 2009 Wiley-Liss, Inc.

This study was performed to assess the imaging findings in cases of parosteal osteosarcoma dedifferentiated into telangiectatic osteosarcoma. Parosteal osteosarcoma is a low-grade well-differentiated malignant tumor. Dedifferentiation into a more aggressive lesion is frequent and usually visible on imaging as a central lytic area in a sclerotic mass. Only one case of differentiation into a telangiectatic osteosarcoma has been reported. As it has practical consequences, with a need for aggressive chemotherapy, we looked for this rather typical imaging pattern. Review of 199 cases of surface osteosarcomas (including 86 parosteal, of which 23 were dedifferentiated) revealed lesions suggesting a possible telangiectatic osteosarcoma on imaging examinations in five cases (cavities with fluid). Histology confirmed three cases (the two other only had hematoma inside a dedifferentiated tumor). There were three males, aged 24, 28, and 32. They had radiographs and CT, and two an MR examination. Lesions involved the distal femur, proximal tibia, and proximal humerus. The parosteal osteosarcoma was a sclerotic, regular mass, attached to the cortex. A purely lytic mass, partially composed of fluid cavities was easily detected on CT and MR. It involved the medullary cavity twice, and remained outside the bone once. Histology confirmed the two components in each case. Two patients died of pulmonary metastases and one is alive. Knowledge of this highly suggestive pattern should help guide the initial biopsy to diagnose the two components of the tumor, and guide aggressive treatment.

Osteosarcoma of the pelvis. A monoinstitutional experience in patients younger than 41 year