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

We evaluated the long-term functional outcome in 118 patients treated for osteosarcoma or Ewing’s sarcoma in the extremities a minimum of five years after treatment. We also examined if impaired function influenced their quality of life and ability to work. The function was evaluated according to the Musculoskeletal Tumor Society (MSTS) score and the Toronto Extremity Salvage Score (TESS). Quality of life was assessed by using the Short Form-36 (SF-36). The mean age at follow-up was 31 years (15 to 57) and the mean follow-up was for 13 years (6 to 22). A total of 67 patients (57%) initially had limb-sparing surgery, but four had a secondary amputation. The median MSTS score was 70% (17% to 100%) and the median TESS was 89% (43% to 100%). The amputees had a significantly lower MSTS score than those with limb-sparing surgery (p < 0.001), but there was no difference for the TESS. Tumour localisation above knee level resulted in significantly lower MSTS scores and TESS (p = 0.003 and p = 0.02, respectively). There were no significant differences in quality of life between amputees and those with limb-sparing surgery except in physical functioning. Of the patients 11% (13) did not work or study. In multivariate analysis, amputation, tumour location above the knee and having muscular pain were associated with low physical function. We conclude that most of the bone tumour survivors managed well after adjustment to their physical limitations. A total of 105 are able to work and have an overall good quality of life

Between December 1995 and March 2003, 38 adult patients with intermediate or high-grade liposarcoma in a limb were treated by limb-sparing surgery and post-operative radiotherapy. The ten-year local recurrence-free survival was 83%, the ten-year metastasis-free survival 61%, the ten-year disease-free survival 51% and the ten-year overall survival 67%. Analysis of failure and success showed no association with the age of the patients, gender, the location of the primary tumour, the type of liposarcoma and the quality of resection. Our results indicate that liposarcoma may recur even ten years after the end of definitive therapy and may spread to unexpected sites as for soft-tissue sarcoma

Our purpose was to assess the role of preoperative radio-therapy +/− neoadjuvant chemotherapy in nonmetastatic soft tissue sarcoma of extremities for limb-sparing surgery and identify the role of neoadjuvant therapies on local control and survival rate. Forty-seven patients with soft tissue sarcoma of extremities who were treated at Cerrahpasa Medical Faculty within a limb salvage protocol, including preoperative radiotherapy +/− chemotherapy were retrospectively analized. Median age was 45 years (17–72 years). The tumor size was between 5–33 cm. Seventeen patients were in stage I, 11 in stage II, 19 in stage III. The most common histology was synovial sarcoma. Nine patients were treated for locally recurrent tumour. The tumour and surrounding tissues with probable microscopic tumour involvement observed clinically and radiologically, were irradiated. Thirty-two patients, with a high grade tumour and/or tumours larger than 8 cm, also received neoadjuvant chemotherapy. Neoadjuvant chemotherapy regimen was consisted of doxorubicine and ifosphamide with mesna. Preoperative radiotherapy was applied, usually between the second and third cycles of chemotherapy. Definitive surgery was administered 2–6 weeks after radiotherapy or after the third cycle of chemotherapy. Chemotherapy was completed to 6 courses after the surgery. Postoperative external beam radio-therapy boost of 16 Gy was given who had close or positive surgical margins. Median follow-up time was 67 months (12–217 months). All of the patients had limb-sparing surgery. Patients had; 30 marginal excision, 13 wide local excision, 4 radical resection. Nine patients locally recurred. Limb-sparing surgery was performed for 8 patients. 25 patients had distant metastases. Metastasectomy were applied for 10 patients with lung metastasis. The 5-year local control, disease free survival and overall survival rates were 82.3%, 50.1% and 67.2%, respectively. Preoperative radiotherapy +/− chemotherapy seems to increase the chance of extremity-sparing surgery with good local control and the survival rates which were comparable with the literature

The August 2024 Oncology Roundup. 360. looks at: What factors are associated with osteoarthritis after cementation for benign aggressive bone tumour of the knee joint: a systematic review and meta-analysis; Recycled bone grafts treated with extracorporeal irradiation or liquid nitrogen freezing after malignant tumour resection; Intercalary resection of the tibia for primary bone tumours: are vascularized fibula autografts with or without allografts a durable reconstruction?; 3D-printed modular prostheses for the reconstruction of intercalary bone defects after joint-sparing limb salvage surgery for femoral diaphyseal tumours; Factors influencing the outcome of patients with primary Ewing’s sarcoma of the sacrum; The significance of surveillance imaging in children with Ewing’s sarcoma and osteosarcoma; Resection margin and soft-tissue sarcomas of the extremities treated with limb-sparing surgery and postoperative radiotherapy

The December 2023 Oncology Roundup. 360. looks at: A single osteotomy technique for frozen autograft; Complications, function, and survival of tumour-devitalized autografts used in patients with limb-sparing surgery; Is liquid nitrogen recycled bone and vascular fibula the biological reconstruction of choice?; Solitary pulmonary metastases at first recurrence of osteosarcoma; Is a radiological score able to predict resection-grade chondrosarcoma in primary intraosseous lesions of the long bones?; Open versus core needle biopsy in lower-limb sarcoma – current practice patterns and patient outcomes; Natural history of intraosseous low-grade chondroid lesions of the proximal humerus; Local treatment modalities and event-free survival in patients with localized Ewing’s sarcoma; Awaiting biopsy results in solitary pathological proximal femoral fractures

Background. Although soft tissue sarcoma (STS) is a rare malignancy, myxofibrosarcoma is a common form diagnosed. Myxofibrosarcoma is complicated by a high local recurrence rate (18–54%) and significant morbidity following treatment, hence management can be challenging. Patients and Methods. Patients treated between 2003–2012 were identified via a database within the histopathology department and case notes were retrospectively assessed. All histology samples were reviewed by a senior histopathologist to ensure a correct diagnosis. Results. 29 patients (12 male, 17 female) with an average age of 61 years (range 19–89 years) underwent surgery at a single centre, with 24 patients receiving adjuvant and two receiving neo-adjuvant radiotherapy. 22 patients had lower limb and 7 had upper limb tumours. 3 were treated for secondary recurrence after having primary surgery elsewhere. 21 patients had Trojani Grade 2 or 3 tumours. All underwent limb-sparing surgery initially but six patients (20.7%) suffered local recurrence after an average follow-up of 28 months and all ultimately required above knee amputation. Four patients developed wound infection, with one requiring VAC therapy. One patient required a flap repair of the forearm. 5-year survival rate was 87.5%. Conclusions. Our results compare favourably against results published so far in the literature with a low local recurrence rate and mortality. Limb-sparing surgery aims to reduce morbidity and disability following treatment but more research is required in adjuvant treatments to further reduce the risk of local recurrence of tumour

Background Osteosarcoma is the most common bone sarcoma, and the 3rd most common malignancy in children and adolescents. It accounts for 20% of primary malignant bone tumors. Methods A retrospective review of osteosarcomas from the Scottish National Bone Tumor Registry (1940–2000) involving the upperlimb bones is presented. Patient demography, type and location of lesions, treatment options, recurrence and survival rates, and metastasis have been analysed. Results 75 cases were identified from the registry. Sex incidence showed a slight male preponderance with male: female ratio 1.14: 1.Age at presentation ranged from 4–88 Yrs (mean 28.44 Yrs). 46.7% sarcomas occurred in the second decade (11–20 Yrs). The humerus was the bone most frequently involved (78.6% of lesions), and the proximal humerus the commonest site (60%). The scapula was involved in 9.3% and the forearm in 8%.A rare solitary lesion of the clavicle was encountered.17% presented with pathological fractures at diagnosis. Patients typically present with dull aching pain of weeks to months. All patients underwent radiological studies and diagnostic biopsy. Treatment modalities included amputation, limb-sparing surgery, adjuvant/neoadjuvant chemotherapy and radiotherapy. The cumulative 5 year survival for the series was 32%.Death was usually due to pulmonary and skeletal metastasis, and the mean survival in such patients was 21.5 mts. Patients presenting with metastatic pulmonary disease had poor prognosis. Limb-sparing surgery with wide margins does not compromise survival. Results with custom endoprosthesis are encouraging. Discussion Osteosarcomas require a multidisciplinary approach to diagnosis and treatment to optimise survival. During the first half of the study period amputation was the mainstay of treatment with high incidence of mortality due to metastatic disease. Recent advances in neoadjuvant/adjuvant chemotherapy have improved the ability to perform limb –sparing resections, and disease free and overall survival rates have improved. Regular, long follow-up is indicated in these patients

Ewing sarcoma (ES) and Osteosarcoma (OS) are the 2 most common malignant primary bone tumors. A patient's response to neoadjuvant chemotherapy has important implications in subsequent patient management and prognosis, as a favourable response to chemotherapy allows orthopedic oncologists to be more aggressive in pursuing limb-sparing surgery. An accurate and non-invasive pre-operative marker of response would be ideal for planning surgical margins and as a prognostic tool. ES and OS have differing biological characterisitcs and respond differently to chemotherapy. We reviewed 18F-FDG PET imaging characteristics of ES and OS patients at baseline and following treatment to determine whether this biological variation is reflected in their imaging phenotype. A retrospective review of ES and OS patients treated with neoadjuvant chemotherapy and surgery was done, correlating PET results with histologic response to chemotherapy. Change in the maximum standardized Uptake Value (SUVmax) between baseline and post-treatment scanning was not significantly associated with histologic response for either ES or OS. Metabolic tumor volume (MTV) and the percentage of injected 18F-FDG dose (%ID) in the primary tumor were found to be different for ES and OS response subgroups. A 50% reduction in MTV (MTV2:1 < 0.5) was found to be significantly associated with histologic response in OS. Using the same criteria for ES incorrectly predicted good responders. Increasing the cut-offs for ES to a 90% reduction in MTV (MTV 2:1 < 0.1) resulted in association with histologic response. Response to neoadjuvant chemotherapy as reflected by changes in PET characteristics should be interpreted differently for ES and OS

Background. Decisions about local treatment are important in osteosarcoma treatment. The purpose of this study was to review decisions about local treatment in one centre. Methods. This was a retrospective review of the records of all patients with high-grade extremity osteosarcoma presenting to our centre between 1997 and 2008. Particular attention was paid to local control decisions. Results. 54 patients were included, 37 were male. Median age was 18 (4.1 to 71.3 years). The anatomical location was distal femur in 33, tibia in 8, humerus in 7, ankle/foot in 3, fibula in 2 and clavicle in 1. 8 (14.8%) patients had metastases at presentation. 13 (24.1%) patients underwent primary amputation, predominantly in the early years of the series. The remaining 41 patients had limb-sparing surgery, 5 of whom had microscopically positive margins. 21 of 54 (38.8%) had >90% necrosis in the resected tumour. 3 patients had poor necrosis and positive margins. These were a 70 yo intolerant of chemotherapy, who refused amputation, developed LR and metastatic disease; a 15 yo with metastatic disease, who had a secondary amputation and metastatectomy and survived and a 43 yo who developed metastases and LR on chemotherapy. 4 further patients had local recurrence after LSS. All had poor necrosis after chemotherapy but adequate margins. All developed metastatic disease and 3 have died. Overall survival was 60%. 5-year survival without metastatic disease at presentation was 65%. Conclusion. Our series is similar to other centres. Challenges include older patients, poor response to chemotherapy and metastases

We considered whether a positive margin occurring after resection of a soft-tissue sarcoma of a limb would affect the incidence of local recurrence. Patients with low-grade liposarcomas were expected to be a low-risk group as were those who had positive margins planned before surgery to preserve critical structures. Two groups, however, were expected to be at a higher risk, namely, patients who had undergone unplanned excision elsewhere with a positive margin on re-excision and those with unplanned positive margins occurring during primary resection. Of 566 patients in a prospective database, 87 with positive margins after limb-sparing surgery and adjuvant radiotherapy were grouped according to the clinical scenario by an observer blinded to the outcome. The rate of local recurrence differed significantly between the two low- (4.2% and 3.6%) and the two high-risk groups (31.6% and 37.5%). This classification therefore provides useful information about the incidence of local recurrence after positive-margin resection

We reviewed the surgical treatment and oncological results of 40 patients with pathological fractures from localised osteosarcoma of the long bones to determine the outcome of limb salvage in their management. All had had adjuvant chemotherapy. There were 26 males and 14 females with a median age at diagnosis of 18 years (2 to 46) and a median follow-up of 55 months (8 to 175). We performed limb salvage in 27 patients and amputation in 13. The margins of resection were radical in five patients, wide in 26, marginal in six, wide but contaminated in two and intralesional in one. Local recurrence developed in 19% of those treated by limb salvage and in none of those who had an amputation. The cumulative five-year survival of all the patients was 57% and in those treated by limb salvage or amputation it was 64% and 47%, respectively (p > 0.05). Limb-sparing surgery with adequate margins of excision can be achieved in many patients with pathological fractures from primary osteosarcoma without compromising survival, but the risk of local recurrence is significant

The purpose of this study is to compare functional results of hemipelvectomy and internal hemipelvectomy following resection of pelvic tumours. The emotional acceptance of such surgery is also examined. From 1998 to 2003, 19 male and 13 female patients, aged from 6 to 76 years, underwent hemipelvectomy, and 12 male and seven female patients, aged from 13 to 65 years, underwent internal hemipelvectomy. In the series as a whole, follow-up ranged from 1 to 156 months. Five patients with external hemipelvectomy, six with internal hemipelvectomy and one who underwent internal hemipelvectomy followed by external hemipelvectomy were evaluated functionally, clinically and psychologically. Patients expressed emotional concern about body image and mobility. Psychosocial adjustment was difficult for all patients, but some were able to overcome their difficulties more easily than others. Patients with internal hemipelvectomy rather than external hemipelvectomy had more difficulty adjusting emotionally. The site of the tumour and morbidity rates, which remain high when limb-sparing surgery is performed for pelvic tumours, are important considerations when deciding upon the type of surgery. Patients and their families need extensive preoperative psychological preparation and postoperative psychotherapy is imperative

Limb-sparing surgery has become the preferred surgical treatment of malignant bone tumours of the knee. In patients with intra-articular extension of their tumour, extra-articular limb sparing surgery can prevent the knee from amputation. In a retrospective study between January 1985 and December 2007, we performed 34 extra-articular tumour resections of the knee-joint for a bone- or soft tissue tumour in the distal femur or proximal tibia with (suspect) intra-articular tumour extension into the knee on MRI. Contra-indications were extension of the tumour into the extensor mechanism and/or tumour involvement of the neurovascular bundle. Osteosarcoma (23/34) was the most common primary malignancy. Mean age was 36 years (17–70) and the mean follow up was 9 years (1–19). Patient survival rates at 5 years and 10 years are 78% and 58% respectively, mean patient survival was 47 months (8–211). In 12 (35%) patients, the primary implanted prosthesis failed during follow up. Prosthetic survival rates including minor revision surgery were 63% at 5 years and 36% at 10 years. Six (18%) patients had local recurrence of their malignancy, 5 of them in the popliteal fossa. Local recurrence was significantly correlated with marginal margins (P< 0.05). Fifteen patients had major complications (44%) mainly deep infection in proximal tibia resections and aseptic loosening in distal femur resections. Aseptic loosening was significantly correlated with non HA-coated stems (P< 0.05). Functional outcome scores according to MSTS (mean 81, (65–93)) and TESS (mean 85, (56–98)) of survivors are good. Our results suggest that extra-articular tumour resections of the knee-joint can provide a functional endoprosthesis and can be an alternative for primary amputation. However it is a technical demanding procedure with acceptable local recurrence and high complication rates in patients with, in general, poor survival

Biopsy is a key step in the diagnosis of bone and soft tissue tumours. An inadequately performed biopsy may fail to allow proper diagnosis. An improperly planned biopsy may jeopardise plans for limb salvage surgery. Aims: To analyse the effectiveness of core-needle biopsy for evaluation of suspected primary musculoskeletal neoplasms. Methods: Core-needle biopsy was performed at our institution in 130 consecutive patients suspected of having a mesenchymal neoplasm. Details of the biopsy and any additional procedures were recorded including size of sample, method of localisation and any complications. Clinical and histological features of the neoplasm and previous radiological or histological diagnosis were compared. Core-needle biopsy results were correlated with results from specimens subsequently obtained at definitive surgery. Results: 130 consecutive core biopsies were performed for evaluation of suspected primary musculoskeletal neoplasms. All patients tolerated the procedure well and there were no significant complications. A definitive diagnosis was obtained from a single core biopsy in 107 (82%) patients; an additional biopsy was necessary in 24 (18%) following equivocal histology. Twenty-three (96%) of these repeat biopsies were an open procedure. In 98% of patients, core-needle biopsy results were concordant with results from specimens subsequently obtained at surgery with respect to tumour histological features and grade. The accuracy and rate of performance of open biopsy for soft tissue lesions were not significantly different from those for bone lesions. Conclusions: Obtaining tissue safely, for diagnosis of bone and soft tissue tumours is the goal of all biopsies The biopsy, however, must be well planned so as to avoid creating inadvertent tumour spread, and take into consideration any subsequent approaches for limb-sparing surgery

Introduciton In our experience, amputation is rarely indicated in osteosarcoma. Amputation is more frequently required in soft tissue sarcoma for the following reasons: 1) recurrent tumour in previously radiated field; 2) composite tissue involvement of soft tissue, bone, vessels and nerves; 3) size of lesion. We have measured functional outcome in bone and soft tissue sarcoma using a combination of clinimetric measures describing impairment and patient determined measures assessing disability (. 1. ,. 2. ). Methods In a matched case-control study (. 3. ), 12 patients with amputation were matched with 24 patients treated by limb-sparing surgery on the following variables: age, gender, length of follow-up, bone versus soft-tissue tumor, anatomic site, and treatment with adjuvant chemotherapy. End points included the Toronto Extremity Salvage Score (TESS), a measure of physical disability; the Shortform-36 (SF-36), a generic health status measure; and the Reintegration to Normal Living (RNL), a measure of handicap. Results Mean TESS score for the patients with amputations was 74.5 versus 85.1 for the limb-sparing patients. (p = .15). Only the physical function subscale of the SF-36 showed statistically significant differences, with means of 45 and 71.1 for the amputation versus limb-sparing groups, respectively (p = .03). The RNL for the amputation group was 84.4 versus 97 for the limb-sparing group (p = .05). Seven of the 12 patients with amputations experienced ongoing difficulty with the soft tissues overlying their stumps. There was a trend toward increased disability for those in the amputation group versus those in the limb-sparing group, with the amputation group showing significantly higher levels of handicap. Conclusions These data suggest that the differences in disability between amputation and limb-sparing patients are smaller than anticipated. The differences may be more notable in measuring handicap

The April 2024 Oncology Roundup³⁶⁰ looks at: Midterm outcomes of total hip arthroplasty after internal hemipelvectomy and iliofemoral arthrodesis; Intraosseous conventional central chondrosarcoma does not metastasize irrespective of grade in pelvis, scapula, and in long bone locations; Oncological and functional outcomes after resection of malignant tumours of the scapula; Reconstruction following oncological iliosacral resection – a comparison of techniques; Does primary tumour resection improve survival for patients with sarcomas of pelvic bones, sacrum, and coccyx who have metastasis at diagnosis?; Older patients with Ewing’s sarcoma: an analysis of the National Cancer Database; Diagnostic challenges in low-grade central osteosarcoma; Effect of radiotherapy on local recurrence, distant metastasis, and overall survival in 1,200 limb soft-tissue sarcoma patients: a retrospective analysis using inverse probability of treatment weighting-adjusted models.

The February 2024 Oncology Roundup³⁶⁰ looks at: Does primary tumour resection improve survival for patients with sarcomas of the pelvis with metastasis at diagnosis?; Proximal femur replacements for an oncologic indication offer a durable endoprosthetic reconstruction option: a 40-year experience; The importance of awaiting biopsy results in solitary pathological proximal femoral fractures: do we need to biopsy solitary pathological fractures?; Effect of radiotherapy on local recurrence, distant metastasis, and overall survival in 1,200 extremity soft-tissue sarcoma patients; What to choose in bone tumour resections? Patient-specific instrumentation versus surgical navigation; Optimal timing of re-excision in synovial sarcoma patients: immediate intervention versus waiting for local recurrence; Survival differences of patients with resected extraskeletal osteosarcoma receiving two different (neo) adjuvant chemotherapy regimens; Solitary versus multiple bone metastases in the appendicular skeleton: should the surgical treatment be different?.

Aims

Endoprosthetic reconstruction following distal femur tumour resection has been widely advocated. In this paper, we present the design of an uncemented endoprosthesis system featuring a short, curved stem, with the goal of enhancing long-term survivorship and functional outcomes.