Aims. Surgical limb sparing for knee-bearing paediatric bone sarcoma is considered to have a clinically significant influence on postoperative function due to complications and leg-length discrepancies. However, researchers have not fully evaluated the long-term postoperative functional outcomes. Therefore, in this study, we aimed to elucidate the risk factors and long-term functional prognosis associated with paediatric limb-sparing surgery. Methods. We reviewed 40 patients aged under 14 years who underwent limb-sparing surgery for knee bone sarcoma (15 cases in the proximal tibia and 25 in the distal femur) between January 2000 and December 2013, and were followed up for a minimum of five years. A total of 35 patients underwent reconstruction using artificial materials, and five underwent biological reconstruction. We evaluated the patients’ postoperative complications, survival rate of reconstruction material, and limb, limb function, and leg-length discrepancy at the final follow-up, as well as the risk factors for each. Results. Complications were observed in 55% (22/40) of patients. The limb survival and reconstruction material rates at five and ten years were 95% and 91%, and 88% and 66%, respectively. Infection was the only risk factor in both survivals (p < 0.001, p = 0.019). In the 35 patients with limb preservation, the median International Society of Limb Salvage (ISOLS) score at the final follow-up was 80 (47% to 97%). Younger age (p = 0.027) and complications (p = 0.005) were poor prognostic factors. A negative correlation was found between age and leg-length discrepancy (R = −0.426; p = 0.011). The ISOLS scores were significantly lower in patients with a leg-length discrepancy of more than 5 cm (p = 0.005). Conclusion. Young age and complications were linked to an unfavourable functional prognosis. Leg-length correction was insufficient, especially in very young children, resulting in decreased function of the affected limb. Limb-sparing surgery for these children remains a considerable challenge. Cite this article: Bone Jt Open 2024;5(10):868–878

Aim. To determine whether delayed diagnosis (lapse from initial symptoms to the beginning of treatment) has influence on the possibilities of crossing the physis by the tumour, and/or on the outcome in pediatric patients with high grade metaphyseal osteosarcoma. Patients and methods. The clinical records, image methods and the histology reports of 157 metaphyseal paediatric osteosarcomas were reviewed. The mean follow-up time was 102 months. Location, histological subtype, time from initial symptoms to start of treatment, major diameter, % of necrosis, physis crossed by the tumour or not, and outcome (recurrence, metastases and status) were recorded in a SPSS v15.0 file. Results. The tumour crossed the physis in 58% of patients. There were statistically significant differences in the age, time from initial symptoms to start of treatment, presence of metastases at diagnosis and outcome between patients affected by tumours crossing the physis and those which did not. Major diameter had no relationship with survival nor delay in diagnosis. Conclusion. The crossing of the physis by a metaphyseal paediatric OS is a matter of time. The outcome of tumours which crossed the physis was worse than that of those which did not. Delay (>2months) between initial symptoms and start of treatment is a poor prognostic factor. The younger the patient, the higher the possibilities of epiphyseal preservation. A major effort should be done to achieve early diagnosis

Aim. The recent discovery of small non-coding RNAs, so-called micro RNAs (miRNAs), has provided new insights into cancer diagnosis. Several studies have shown that profiles of miRNA expression differ between normal tissue and tumour tissue and vary among tumour types. To exploit this difference, we evaluated the feasibility of using muscle-specific miRNAs (miR-1, 133a, 133b, 206) as biomarkers of rhabdomyosarcoma (RMS). Method. Total RNA was extracted from 16 cell lines (7 RMS, 4 neuroblastoma, 3 Ewing sarcoma and 2 malignant rhabdoid tumour) and 21 tumour specimens (7 RMS, 1 Ewing sarcoma, 4 undifferentiated sarcoma, 1 osteosarcoma, 1 alveolar soft part sarcoma, 2 neuroblastoma, 2 Wilms tumour, 1 malignant rhabdoid tumour, 1 adrenal carcinoma and 1 retinoblastoma). miRNA was quantified by real-time RT-PCR. The expression levels of miRNAs were calculated utilizing the delta-delta Ct method, normalised to the level of miR-16, and compared using the Mann-Whitney U test. Results. The expression levels of muscle-specific miRNAs in the RMS cell lines were significantly higher (p<0.01) than those in neuroblastoma, Ewing sarcoma and malignant rhabdoid tumour cell lines. miR-206 was most abundantly expressed and miR-1 was least abundantly expressed among muscle-specific miRNAs in RMS cell lines. The expression levels of musclespecific miRNAs in RMS tumour specimens were significantly higher (p<0.01) than those in other pediatric tumours. The difference in the expression levels between RMS and other tumours was largest in miR-206. Conclusion. The expression levels of muscle-specific miRNAs were significantly elevated in RMS cell lines and tumour specimens. Muscle-specific miRNAs, especially miR-206, can be potential biomarkers for RMS diagnosis

In skeletally immature patients, resection of bone tumours and reconstruction of the lower limb often results in leg-length discrepancy. The Stanmore non-invasive extendible endoprosthesis, which uses electromagnetic induction, allows post-operative lengthening without anaesthesia. Between 2002 and 2009, 55 children with a mean age of 11.4 years (5 to 16) underwent reconstruction with this prosthesis; ten patients (18.2%) died of disseminated disease and one child underwent amputation due to infection. We reviewed 44 patients after a mean follow-up of 41.2 months (22 to 104). The mean Musculoskeletal Tumor Society score was 24.7 (8 to 30) and the Toronto Extremity Salvage score was 92.3% (55.2% to 99.0%). There was no local recurrence of tumour. Complications developed in 16 patients (29.1%) and ten (18.2%) underwent revision.

The mean length gained per patient was 38.6 mm (3.5 to 161.5), requiring a mean of 11.3 extensions (1 to 40), and ten component exchanges were performed in nine patients (16.4%) after attaining the maximum lengthening capacity of the implant. There were 11 patients (20%) who were skeletally mature at follow-up, ten of whom had equal leg lengths and nine had a full range of movement of the hip and knee.

This is the largest reported series using non-invasive extendible endoprostheses after excision of primary bone tumours in skeletally immature patients. The technique produces a good functional outcome, with prevention of limb-length discrepancy at skeletal maturity.

The use of allografts for the treatment of bone tumours in children is limited by nonunion and the difficulty of finding a suitable graft. Furthermore, appositional growth can’t be expected of an allograft.

We used an overlapping allograft in 11 children, with a mean age of ten years (4 to 15), with a mean follow-up of 24.1 months (20 to 33). There were five intercalary and six intra-articular resections, and the tumours were in the femur in six cases and the humerus in five. Rates of union, times to union, remodelling patterns and allograft-associated complications were evaluated.

No allograft was removed due to a complication. Of the 16 junctional sites, 15 (94%) showed union at a mean of 3.1 months (2 to 5). Remodelling between host and allograft was seen at 14 junctions at a mean of five months (4 to 7). The mean Musculoskeletal Tumor Society score was 26.5 of 30 (88.3%). One case of nonunion and another with screw protrusion required re-operation.

Overlapping allografts have the potential to shorten time to union, decrease rates of nonunion and have positive appositional growth effect.

We report our early experience with the use of a non-invasive distal femoral expandable endoprosthesis in seven skeletally immature patients with osteosarcoma of the distal femur. The patients had a mean age of 12.1 years (9 to 15) at the time of surgery. The prosthesis was lengthened at appropriate intervals in outpatient clinics, without anaesthesia, using the principle of electromagnetic induction. The patients were functionally evaluated using the Musculoskeletal Tumour Society scoring system. The mean follow-up was 20.2 months (14 to 30). The prostheses were lengthened by a mean of 25 mm (4.25 to 55) and maintained a mean knee flexion of 110° (100° to 120°). The mean Musculoskeletal Tumour Society score was 68% (11 to 29). Complications developed in two patients; one developed a flexion deformity of 25° at the knee joint, which was subsequently overcome and one died of disseminated disease. The early results from patients treated with this device have been encouraging. The implant avoids multiple surgical procedures, general anaesthesia and assists in maintaining leg-length equality.

Introduction

Following bone tumour resection, lower limb reconstruction results in leg-length discrepancy in skeletally immature patients. Previously, minimally invasive endoprostheses have been associated with a high risk of complications including joint stiffness, nerve injury, aseptic loosening and infection. The purpose of this study was to examine the outcome of the Stanmore non-invasive extendible endoprostheses used in our institution between 2002 and 2009 and compare them with implants used in the past.

We reviewed our initial seven-year experience with a non-invasive extendible prosthesis in 34 children with primary bone tumours. The distal femur was replaced in 25 cases, total femur in five, proximal femur in one and proximal tibia in three. The mean follow-up was 44 months (15 to 86) and 27 patients (79%) remain alive. The prostheses were lengthened by an electromagnetic induction mechanism in an outpatient setting and a mean extension of 32 mm (4 to 80) was achieved without anaesthesia. There were lengthening complications in two children: failed lengthening in one and the formation of scar tissue in the other. Deep infection developed in six patients (18%) and local recurrence in three. A total of 11 patients required further surgery to the leg. Amputation was necessary in five patients (20%) and a two-stage revision in another. There were no cases of loosening, but two patients had implant breakage and required revision. The mean Musculoskeletal Tumor Society functional score was 85% (60% to 100%) at last known follow-up. These early results demonstrate that the non-invasive extendible prosthesis allows successful lengthening without surgical intervention, but the high incidence of infection is a cause for concern.

Aims. For paediatric and adolescent patients with growth potential, preservation of the physiological joint by transepiphyseal resection (TER) of the femur confers definite advantages over arthroplasty procedures. We hypothesized that the extent of the tumour and changes in its extent after neoadjuvant chemotherapy are essential factors in the selection of this procedure, and can be assessed with MRI. The oncological and functional outcomes of the procedure were reviewed to confirm its safety and efficacy. Methods. We retrospectively reviewed 16 patients (seven male and nine female, mean age 12.2 years (7 to 16)) with osteosarcoma of the knee who had been treated by TER. We evaluated the MRI scans before and after neoadjuvant chemotherapy for all patients to assess the extent of the disease and the response to treatment. Results. The mean follow-up period was 64.3 months (25 to 148) after surgery and no patients were lost to follow-up. On MRI evaluation, 13 tumours were near but not in contact with the physes and three tumours were partially in contact with the physes before neoadjuvant chemotherapy. Bone oedema in the epiphysis was observed in eight patients. After neoadjuvant chemotherapy, bone oedema in the epiphysis disappeared in all patients. In total, 11 tumours were not in contact and five tumours were in partial contact with the physes. The postoperative pathological margin was negative in all patients. At the last follow-up, 12 patients were continuously disease-free and three had no evidence of disease. One patient died due to the disease. Functionally, the patients with retained allograft or recycled autograft had a mean knee range of flexion of 126° (90° to 150°). The mean Musculoskeletal Tumor Society functional score was 27.6 (23 to 30). Conclusion. TER is an effective limb-salvage technique for treating malignant metaphyseal bone tumours in paediatric and young osteosarcoma patients with open physes when a good response to chemotherapy and no progression of the tumour to the epiphysis have been confirmed by MRI. Cite this article: Bone Joint J 2020;102-B(6):772–778

Previous classification systems of failure of limb salvage focused primarily on endoprosthetic failures and lacked sufficient depth for the effective study of the causes of failure. In order to address these inadequacies, the International Society of Limb Salvage (ISOLS) formed a committee to recommend revisions of the previous systems. The purpose of this study was to report on their recommendations. The modifications were prepared using an earlier, evidence-based model with subclassification based on the existing medical literature. Subclassification for all five primary types of failure of limb salvage following endoprosthetic reconstruction were formulated and a complementary system was derived for the failure of biological reconstruction. An additional classification of failure in paediatric patients was also described. . Limb salvage surgery presents a complex array of potential mechanisms of failure, and a complete and precise classification of types of failure is required. Earlier classification systems lacked specificity, and the evidence-based system outlined here is designed to correct these weaknesses and to provide a means of reporting failures of limb salvage in order to allow the interpretation of outcome following reconstructive surgery. Cite this article: Bone Joint J 2014;96-B:1436–40

Aims

The ulna is an extremely rare location for primary bone tumours of the elbow in paediatrics. Although several reconstruction options are available, the optimal reconstruction method is still unknown due to the rarity of proximal ulna tumours. In this study, we report the outcomes of osteoarticular ulna allograft for the reconstruction of proximal ulna tumours.

Aims

The epiphyseal approach to a chondroblastoma of the intercondylar notch of a child’s distal femur does not provide adequate exposure, thereby necessitating the removal of a substantial amount of unaffected bone to expose the lesion. In this study, we compared the functional outcomes, local recurrence, and surgical complications of treating a chondroblastoma of the distal femoral epiphysis by either an intercondylar or an epiphyseal approach.

Aims

The aim of this study was to investigate the safety and efficacy of 3D-printed modular prostheses in patients who underwent joint-sparing limb salvage surgery (JSLSS) for malignant femoral diaphyseal bone tumours.

Aims

Intra-articular (IA) tumours around the knee are treated with extra-articular (EA) resection, which is associated with poor functional outcomes. We aim to evaluate the accuracy of MRI in predicting IA involvement around the knee.

Aims

Osteoarticular reconstruction of the distal femur in childhood has the advantage of preserving the tibial physis. However, due to the small size of the distal femur, matching the host bone with an osteoarticular allograft is challenging. In this study, we compared the outcomes and complications of a resurfaced allograft-prosthesis composite (rAPC) with those of an osteoarticular allograft to reconstruct the distal femur in children.

Early diagnosis is assumed to improve patient survival, but whether symptom interval (SI i.e. the period between the onset of the first symptoms signs of the disease and its definitive diagnosis) has significant impact on outcome or not remain unclear. Methods. In a series of 575 patients < 21 years of age with soft tissue sarcomas (STS) we investigated the association patterns between SI patient/tumour characteristics or disease outcome. The analysis was based on multivariate models (linear for association's patient/tumour characteristics and Cox's for survival). Results. The SI ranged between one week and 60 months (median 2 months) and tended to be longer the older the patient (i.e. the interval was longer in adolescents than in children) and the larger tumour's size and for tumours located at the extremities and for “non rhabmomio sarcoma” STS (as opposed to rhabmomio sarcomas). A longer SI unfavourably influenced survival (p=0.002): for SI of 1, 12 and 24 months, the 5-year survival for rhabmomio sarcoma was 65%, 46% and 19% respectively. A different pattern of association between SI and survival emerged for different types of STS histology. Conclusion. Our study shows that sarcoma-specific mortality increased with longer SI. This was far from obvious or expected. The independent prognostic effect of SI cannot be explained by its associations with other factors, such as patient's age (Adolescents having the longest diagnostic delays) or site, size (large tumour may be indicative, to some degree at least, of a late diagnosis) stage and histology of the tumour. Future studies should focus more on the possible cause of SI in pediatric STS populations to enable corrective measures to be implemented to reduce the diagnostic delay

Aim. The percentage of adolescents and young adults with sarcoma enrolled in multicenter clinical trials is reportedly much lower than that of younger children. We intended to determine if this remained true despite the availability of a study open to patients up to the age of 40 years. Method. Review of interim recruitment and randomization rates in a large randomized multinational trial for resectable osteosarcoma, EURAMOS-1, jointly performed by COG, COSS, EOI, and SSG, within ECT-EUROCORES. Randomization scheduled after preoperative chemotherapy and definitive surgery of the primary tumour. Results. Between Apr-2005 and Jan-2010, 1,682 patients were recruited (870 COG, 390 COSS, 338 EOI, 85 SSG). To date, 979 have been randomized. When normalizing for population-based, age-dependent osteosarcoma incidence rates according to SEER data (Mirabello et al., Cancer 2009;115:1531–43) and arbitrarily defining observed/expected recruitment at age 10-14 years as 1, there was age-appropriate recruitment at age 5-9 (1.06 patients recruited per patient expected), but under recruiting for patients aged 15 and above, particularly above the age of 19 (15-19yr: 0.88, 20-24yr: 0.52; 25-29yr: 0.17, 30-34yr: 0.09, 35-39yr: 0.22 patients recruited per patient expected) (Fig. 1). If age groups 15yr and above had recruited with the same incidence-based ratio as younger ages, up to 592 additional patients might have been recruited, an increment of 35%. Uptake of randomization was similar across all age groups. Fig. 1: EURAMOS recruitment in comparison to epidemiological data (SEER). Recruitment ratio at age 10-14 arbitrarily defined as 1. Conclusion. Recruitment and randomization into large, multinational osteosarcoma trials might be increased by approximately one third if older adolescents and young adults were recruited at similar incidence-related rates as younger pediatric patients. Supported by the European Science Foundation (ESF), under the EUROCORES Program European Clinical Trials (ECT), through contract number ERASCT-2003-980409 of the European Commission, DG Research, FP6

Aims

Aneurysmal bone cysts (ABCs) are locally aggressive lesions typically found in the long bones of children and adolescents. A variety of management strategies have been reported to be effective in the treatment of these lesions. The purpose of this review was to assess the effectiveness of current strategies for the management of primary ABCs of the long bones.

Aims

Limb salvage for pelvic sarcomas involving the acetabulum is a major surgical challenge. There remains no consensus about what is the optimum type of reconstruction after resection of the tumour. The aim of this study was to evaluate the surgical outcomes in these patients according to the methods of periacetabular reconstruction.

Aims

Urgent referral to a specialist centre for patients with a soft-tissue sarcoma (STS) has been recommended by the National Institute for Health and Care Excellence (NICE) in the UK since 2006. However, the impact of this recommendation on the prognosis for these patients remains unclear. We aimed to determine the impact of the NICE guidelines on the disease-specific survival (DSS) of patients with an STS.