Background

Extendable partial femoral replacements (EPFR) permit limb salvage in children with bone tumours in proximity to the physis. Older designs were extended through large incisions or minimally invasive surgery. Modern EPFR are lengthened non-invasively. Lengthening improves functional score (Futani, 2006) but has been associated with complications including infection (Jeys, 2005). This study is the first to look specifically at the relationship between EPFR lengthening and complications.

Patients with peripheral primary bone tumours are often identified and referred at an early stage to a regional tumour service according to established guidelines. In patients with primary bone tumours of the spine, however, the definitive management or outcome of such patients is being prejudiced by preliminary intervention from non-specialist services prior to their referral. Objective: To audit the standards of management of patients with primary bone tumours of the spine referred to a regional tumour service. Retrospective review of case notes and radiology. Subjects: Patients with primary bone tumour of the spine managed at the Orthopaedic Spine Unit with the Regional Bone Tumour Service in Newcastle Upon Tyne Hospitals NHS Trust. Referral to tumour service, prior intervention, operative treatment, survival, factors affecting definitive management. 31 of 39 (16 benign, 23 malignant) patients were initially referred from primary care to services other than the regional tumour service, most commonly neurosurgery (11/39) and paediatric oncology (4/39). Seven of 39 of these patients had undergone interventions prior to their referral to the tumour service, which may have negatively impacted their definitive management or curative surgery. These tumours present complex issues regarding their definitive management to optimise outcome. Closer links between departments are required to enable the multidisciplinary management of primary bone tumours of the spine. Prior surgical intervention may compromise cure. Those involved in their management should be encouraged to liaise with their regional bone tumour service to improve outcome

Bone reconstruction in pediatric oncology always has to face two major problems: the frequent unavailability of small prosthetic tools and the difficulty in finding bone allografts of adequate size. Aim of this work is to present the research lines in this field, currently active in our institution to improve the planning and the results of reconstructive tumor surgery in children. Starting from patient’s CT data sets, subject-specific 3D models of bone segments can be created and compared with the similar models obtained by the CT analysis of massive allografts stored in the Rizzoli Bone Bank. In the same time the computer modelling technologies allow the development of three-dimensional environment, where the surgeon can navigate and exploit both artificial (prostheses, metallic plates and screws) or biological tools (bone allografts or autografts). The presented method has been utilized with success in 10 children (mean age 8, range 4–13) that underwent a skeletal reconstruction of the limbs in the last year (proximal humerus 1, diaphyseal humerus 1, total humerus 1, distal radius 1, proximal femur 3, diaphyseal femur 1, proximal tibia 1, diaphyseal tibia

We attempted to investigate the incidence, the treatment modalities used and the outcome of Osteosarcoma (OS) patients treated at the two major Pediatric Oncology Hospitals in Singapore. A comprehensive list of patients with OS treated at the National University Hospital and KK Women’s and Children’s Hospital Singapore between April 1980 and May 2006 was generated. During the study interval, patients received neoadjuvant chemotherapy followed by definitive surgery consisting of either limb salvage or amputation followed by adjuvant chemotherapy. Chemotherapy was as per the European Osteosarcoma Intergroup (EOI) and as per the Memorial Sloan-Kettering Cancer Center’s (MSKCC) T12 protocols. Treatment of subsequent relapses consisted of various combinations of Methotrexate, ifosfamide, etoposide, other, and/or surgery. Of the total 49 patients with OS, 30% presented with metastatic OS. Median age of diagnosis of OS was 12.4 years. For the cohort, two and five-year overall survival were 71% and 55% respectively. The two-year overall survival was 73% for patients who were treated as per the MSKCC protocol. At last follow-up, median 4.3 years (range, 0.3 – 21.6 years), 25 were alive with no evidence of disease and 16 were dead of disease. Survival from OS in Singapore appears to be improving. Rarity and complexity of OS makes it crucial for patients to seek a centralized multi-disciplinary Musculoskeletal Oncology team involving surgeons and Pediatric Oncologists dedicated to the care of these young patients with the intent to cure

Background: Intensive chemotherapy in sarcoma treatment may lead to weight loss, and in turn reduce dose intensity. A possible correlation between weight loss under treatment and outcome has never been analysed in sarcoma treatment. Ewing sarcoma (ES) patients undergoing intensive non-corticosteroid-containing chemotherapy commonly experience weight loss. The German Paediatric Oncology/Haematology Society (GPOH) Ewing sarcoma trials (CESS, EICESS) registry was analysed with respect to weight loss and outcome. Patients and Methods: Body weight (BW) both at diagnosis and after a median of 12 courses of chemotherapy was available in 837 of 1549 ES patients, excluding amputees. Changes in BW were calculated as percentage of initial BW; outcome was determined as event-free-survival (EFS) from diagnosis according to Kaplan-Meier. Correlations of BW and outcome and potential confounders like disease stage or tumour volume were estimated uni- and multivariately. Results: Weight loss was not correlated with inferior outcome: A loss of 10%–20% of BW was associated with a slightly more favourable outcome (3-year EFS 0.64 +/−0.106, N=82) than weight gain of 10%–20% of BW (3-year EFS 0.58 +/−0.098, N=97), p=0.101. Multivariate analyses revealed no confounders interfering with these results. Conclusions: In 837 ES-patients analysed, weight loss did not correlate with inferior outcome. This should be observed in discussions about tube feeding and/or parenteral nutrition under cytostatic therapy. Future analyses of the prognostic impact of extreme under- or overweight both at diagnosis or under treatment are warranted in order to develop appropriate guidelines. Validity of this observation should be analysed for other solid tumours. Supported by EC Biomed and Deutsche Krebshilfe

Purpose of study: ALL is the commonest childhood malignancy and current survival rates reach 80%. Consequently adverse effects of therapy may have significant long-term implications. Treatment is risk stratified with the higher intensity regimens B and C receiving more Dexamethasone and Methotrexate, both of which are known to have significant bony effects. The skeletal morbidity associated with ALL and its treatment, particularly AVN, is being increasingly identified. AVN is rare in paediatric practice. Its pathophysiology is largely unknown, although it is probably related to corticosteroid use. Method: The records of a series of 7 out of 53 children treated with chemotherapy for ALL as per MRC ALL 99/01 and ALL 2003 protocols in a single tertiary paediatric oncology centre were reviewed. All 7 cases (3 male) had MRI confirmation of AVN (2 normal x-rays). All cases had been treated on higher intensity chemotherapy regimens and were at least 6 months from commencement of anti-leukaemic therapy. All presented with transient limb pain. Results: The AVN was multi-focal (median 4 sites) and was associated with significant other bony abnormalities, including joint collapse, fracture, osteomyelitis and septic arthritis. In this series the dose of steroids did not correlate with the severity of the AVN. Treatment options were limited with all cases receiving conservative treatment. Conclusions: Careful consideration must precede a recommendation to stop steroids as this may compromise leukaemic cure. Although the long-term consequences of AVN on bone health are unknown, we recommend a high index of suspicion in professionals who are treating these children to ensure prompt diagnosis

Synovial sarcoma is the most common NRSTS, that typically affects the extremities of adolescents. To improve the results of the treatment of synovial sarcoma for children and adolescents is the target of this study. 19 children and adolescents at the mean age of 10,84±3,28 years (9 males, 10 females) with synovial sarcoma were treated between 1999 and 2008 years at the Research Institution of Pediatric Oncology in the Russian Cancer Center. Histologically, 5 patients had the biphasic,12 had the monophasic, and 2 of them had the poorly differentiated pattern. The most often affected area was the area of the lower extremity – 10 cases, the area of the upper extremity was affected in 3 cases, and the trunk – 6 cases. According to the staging systems adopted, the size > 5cm (TB) was reported in 12 cases. Five patients (non-staging) had relapse of disease. Four patients had nodal involvement, and 4 had distant metastases (mostly at lungs). The general scheme of the treatment included: 8 courses of chemotherapy (used ifosfamide or cyclophosphamide, ethoposide, carboplatine); the harvesting and preservation of the stem cells after the stimulation of the haemophoesis by G-CSF, the stage of the local control of the tumor consisting of the surgical ablation of the primary lesion (in 1 case it was not available) and the radiotherapy of the initial tumor and metastasis left after the induction. The partial effect was registered by most of the patients – 80%. We observed 1 case of progression of the disease during inductive CT. The toxicity of intensive chemotherapy was reduced by support of sub transplantation doses of peripheral blood stem cells – 0,9-1,5±0,1·106 per kg. In our research we have analyzed the 5-year overall and disease free survival. Thus, 5-year disease-free survival was 66,1±11,3 %, overall 5-year survival −75,6±10,6%

Sclerosing epitheloid fibrosarcoma (SEF) is an extremely rare soft tissue sarcoma arising from connective tissue cells of mesenchymal origin. SEF mostly occurs in extraosseous sites in the soft tissue; however two cases of primary localization in the bone have been described. Despite benign cytological features the clinical course is complicated by a high local recurrence rate and late metastases. SEF represents a clinically challenging entity especially because no standardized treatment regimens are available. We report a 16-year old female patient who showed persistent load-dependent pain focused on the right proximal tibia. Radiological evaluation revealed an osteolytic lesion and the diagnosis of a benign bone cyst was consented. The tumor was surgically removed. Only after recurrence of the tumor and repeated histopathological analysis diagnosis of SEF could be established. Because of the bone localization of the tumor the patient underwent standardized neoadjuvant chemotherapy analogous to the European-American EURAMOS-1 protocol for the treatment of osteosarcoma followed by tumor resection and endoprothesis. Histopathological analysis of the resected tumor showed > 90% vital tumor cells suggesting no response to the neoadjuvant chemotherapy. Therefore, therapy was reassigned to the CWS protocol of the German Society for Pediatric Oncology and Hematology (GPOH) for treatment of soft tissue sarcoma. To date, the patient is alive and no metastases of the primary tumor can be detected. SEF represents a taunting clinical entity due to deceptive histopathological features and rare occurrence. Localization in the bone represents an additional challenge with regards to the therapeutical approach. Standardized treatment regimens are currently not available for SEF. This case report, to our knowledge, is the first outlining a therapeutic approach in detail. Our data suggest that SEF may be resistant to a chemotherapy regimen containing Cisplatin, Doxorubicin and Metho-trexate despite close association to the bone, possibly indicative of the soft tissue histogenesis of this tumor. The response to the soft tissue sarcoma targeting CWS chemotherapy remains to be determined

Introduction: Late effects of treatment in pediatric oncology patients are major issues of follow up care. Success in overall survival of patients with Ewing’s sarcoma/PNET (ES/PNET) correlates with cumulative doses of alkylating agents especially. Renal toxicity is subject of interest due to irreversible tubulopathy and need of many concomitant nephrotoxic drugs given during treatment. Objectivee: Patients suffered from ES/PNET on Euro Ewing 99 protocol were eligible for evaluation of renal functions during therapy and follow up. Methods: Design ofthis study was single institution observational. Total of 20 patients were included 9 females and 11 males. Renal toxicity was assessed as glomerular filtration rate according to Schwartz’s formula (GFR), serum creatinine (S-crea), fractionated phosphate reabsorption (Tp/Ccrea), daily phosphate waste in urine (U-P), daily protein waste in urine (U-Pr) and minimal signs of Fanconi syndrome (FS; positive urine glucose and/or protein). Results: Median age at time of diagnosis was 11.7 years. Median follow up from time of diagnosis was 1.2 months. Median dose of ifosfamide was 87 g/ m2. Median GFR decreased from 2.7ml/s to 2.2 ml/s (p=0.001). Median of S-crea was initially 48 μl/l and 58.1 μl/l at time of last follow up (p< 0.001). Median of Tp/Ccrea was 1.2 mmol/l and decreased to 1.1 mmol/ l at the end of treatment (p= 0.026). U-P was initially and finally 11.9 mmol and 25.1mmol, respectively (p = 0.008). Median of tubular reabsorption of phosphate decreased from 95% initially to 90% (p=0.001). Daily waste of protein increased from 0.11 g to 0.43 g (p= 0.051). Minimal signs of FS developed in 9 of 17 patients (53%). Conclsions: Patients with high cumulative doses of ifosfamide are at risk of renal impairement. Despite statistically significant differences of severeral measures observed in this study, clinical impact of post treatment values does not exceed grade 1 toxicity. Observation of minimal signs of FS developed in 53% of patients is of concern and further treatment of ES/PNET should be carefully focused on late effect too

Purpose of the study: We wanted to assess long-term outcome after treatment for Ewing tumor of the pelvis. Material and method: We reviewed 62 patients aged 5 to 28 years treated from 1983 through 1993. There were 35 males and 27 males. Sixteen patients had pulmonary metastases at diagnosis. Patients were given chemotherapy using three protocols (Ew 84, Ew 88, Ew 93) proposed by the French Society of Pediatric Oncology. Fourteen patients were give high-dose chemotherapy with a bone marrow graft. The local treatment was not randomized. Radiotherapy was used alone in 25 patients and 15 underwent surgery and radiotherapy. Eighteen underwent surgery without complementary radiotherapy. For patients were not given local treatment. Outcome at last follow-up was assessed retrospectively. Results: Mean follow-up was 6.6 years (3 months to 18 years); 29 patients were in remission, 6 had progressive disease, and 27 had died. Two patients who had bone marrow grafts developed a second tumor in the radiated territory. The overall chances of survival were 55±6% at five years and 53±7% at ten years. There was no significant difference by type of chemotherapy. In the group of operated patients, the five year survival was 68% versus 43% in the group of non-operated patients (p=0.007). In patients with initial metastases, chances of survival at ten years were 19.7±10% versus 65.9±7% in patients without metastasis. Only two patients who presented metastases initially were in remission at last follow-up. Five patients developed local recurrence after surgery and none had been radiated despite incomplete response to chemotherapy or presence of contaminated resections. Discussion: Rigorous comparison between operated and non-operated patients is hindered due to the different indications. Results of treatment of Ewing tumors of the pelvis without metastasis are comparable to those obtained for tumors in other localizations. The fact that a second tumor can develop in the radiated territory is a particularly important factor in patients given high-dose chemotherapy with a bone marrow graft. Conclusion: Surgical treatment appears to improve local control of Ewing tumors of the pelvis. If initial metastasis is not present, the prognosis appears to be similar to other localizations. Radiotherapy remains and indispensable adjuvant in the event of surgical resection or incomplete response to chemotherapy

Purpose of the study: Advances in chemotherapy protocols over the last 20 years have considerably improved the prognosis and functional outcome in patients with osteogenic sarcoma. We report here the results of a cooperative study conducted under the auspices of the French Society of Pediatric Oncology (SFOP). Twenty-nine oncology centers participated in this retrospective national multicentric study. Materials and methods: The study included 15 .3 patients with osteogenic sarcoma of the limb who were treated by the OS87 protocol with conservative surgery between 1987 and 1994. The OS87 protocol consisted in conservative or nonconservative surgery combined with pre- and postoperative chemotherapy. The following inclusion criteria were used: age under 20 years, tumor localization in a limb (pelvis and spine excluded), no metastasis at diagnosis, biopsy proven osteogenic sarcoma. Results: Mean age at diagnosis was 13 years. The knee localization predominated (80 p. 100). 82.5 p. 100 of the patients had grade IIB disease (Enneking classification). For the 187 patients included in the protocol surgery was nonconservative in 20 p. 100 of the cases and conservative in 80 p. 100. The choice of the surgical technique (arthroplasty, allograft, autograft, resection without reconstruction) depended on the patient’s age and school situation. Data analyzed here concerned only those patients who had conservative treatment. Mean follow-up was 64 months. The actuarial survival curve plateaued at 71 p. 100 at more than 6 years. Early and late complications were numerous and variable (mechanical, infectious, local recurrence). Secondary amputation was required in 10 p. 100 of the patients. The overall functional outcome of the preserved limbs was nevertheless good with rapid restoration of self-sufficiency despite major surgery and a high number of reoperations (about 65 p. 100 of cases). Discussion: In light of the frequency and the seriousness of the complications, these results are modest. Patients and family should be advised of the risk, particularly the risk of secondary amputation which may be required early due to contaminated excision or at mid term due to major non-cancerological complications. As survival has been improved, functional capacity must be preserved for several years. This orients surgery towards more “biological” reconstruction which can provide greater longevity than arthroplasty

Aims

The aim of this study was to identify the information topics that should be addressed according to the parents of children with developmental dysplasia of the hip (DDH) in the diagnostic and treatment phase during the first year of life. Second, we explored parental recommendations to further optimize the information provision in DDH care.

The surgical community is plagued with a reputation for both failing to engage and to deliver on clinical research. This is in part due to the absence of a strong research culture, however it is also due to a multitude of barriers encountered in clinical research; particularly those involving surgical interventions. ‘Trauma’ amplifies these barriers, owing to the unplanned nature of care, unpredictable work patterns, the emergent nature of treatment and complexities in the consent process. This review discusses the barriers to clinical research in surgery, with a particular emphasis on trauma. It considers how barriers may be overcome, with the aim to facilitate future successful clinical research.

Cite this article: Bone Joint Res 2014;3:123–9.