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

Aims

The standard of surgical treatment for lower limb neoplasms had been characterized by highly interventional techniques, leading to severe kinetic impairment of the patients and incidences of phantom pain. Rotationplasty had arisen as a potent limb salvage treatment option for young cancer patients with lower limb bone tumours, but its impact on the gait through comparative studies still remains unclear several years after the introduction of the procedure. The aim of this study is to assess the effect of rotationplasty on gait parameters measured by gait analysis compared to healthy individuals.

Primary malignant bone and soft tissue tumours often occur in the lower extremities of active individuals including children, teenagers and young adults. Survivors routinely face long-term physical disability. Participation in sports is particularly important for active young people but the impact of sarcoma treatment is not widely recognised and clinicians may be unable to provide objective advice about returning to sports. We aimed to identify and summarise the current evidence for involvement in sports following treatment of lower limb primary malignant bone and soft tissue tumours. A comprehensive search strategy was used to identify relevant studies combining the main concepts of interest: (1) Bone/Soft Tissue Tumour, (2) Lower Limb, (3) Surgical Interventions and (4) Sports. Studies were selected according to eligibility criteria with the consensus of three authors. Customised data extraction and quality assessment tools were used. 22 studies were selected, published between 1985 – 2020, and comprising 1005 patients. Fifteen studies with data on return to sports including 705 participants of which 412 (58.4%) returned to some form of sport at a mean follow-up period of 7.6 years. Four studies directly compared limb sparing and amputation; none of these were able to identify a difference in sports participation or ability. Return to sports is important for patients treated for musculoskeletal tumours, however, there is insufficient published research to provide good information and support for patients. Future prospective studies are needed to collect better pre and post-treatment data at multiple time intervals and validated clinical and patient sports participation outcomes such as type of sports participation, level and frequency and a validated sports specific outcome score, such as UCLA assessment. In particular, more comparison between limb sparing and amputation would be welcome

Aims

The modified Glasgow Prognostic Score (mGPS) uses preoperative CRP and albumin to calculate a score from 0 to 2 (2 being associated with poor outcomes). mGPS is validated in multiple carcinomas. To date, its use in soft-tissue sarcoma (STS) is limited, with only small cohorts reporting that increased mGPS scores correlates with decreased survival in STS patients.

Aims

Pelvic reconstruction after the resection of a tumour around the acetabulum is a challenging procedure due to the complex anatomy and biomechanics. Several pelvic endoprostheses have been introduced, but the rates of complication remain high. Our aim was to review the use of a stemmed acetabular pedestal cup in the management of these patients.

Aims

Myxofibrosarcomas (MFSs) are malignant soft-tissue sarcomas characteristically presenting as painless slowly growing masses in the extremities. Locally infiltrative growth means that the risk of local recurrence is high. We reviewed our experience to make recommendations about resection strategies and the role of the multidisciplinary team in the management of these tumours.

Limb salvage in musculoskeletal tumor surgery may be complicated by infection. With the advent of modern techniques and medical management limb sparing surgeries can be considered as an alternative to ablation. Between 1992 and 2014, 17 patients were treated for infected megaprostheses after being surgically treated for musculoskeletal tumors. There were nine females and eight males. The mean time from the index procedure until infection was 30 months. Following radical debridement, the resultant skeletal defect averaged 30 cm. Patients were treated with local antibiotics in polymethyl methacrylate (PMMA) spacers and endoprostheses as well as IV antibiotics for a minimum of six weeks followed by oral antibiotics for an additional six weeks. The initial tumor procedure involved the femur in eleven patients, the tibia in two, the acetabulum in one, the humerus in two, and the ulna in one. Patients had repeat cultures before two-stage reimplantation when their WBC, ESR, and CRP returned to normal. Patients were reimplanted when final cultures were negative. Thirteen patients were treated using a two-stage protocol with customized intraoperative antibiotic impregnated PMMA spacers including intramedullary nails for a mean of 10 months and the other four patients had a one-stage procedure. These four patients included two patients with a total femur replacement and two patients with an allograft-prosthetic composite of the proximal humerus and ulna. The organisms cultured were gram positive in 14 cases, mixed gram positive and negative in one case, and two patients had no growth on cultures but histologic evidence of acute infection. Reimplantation was successful in 13 patients after the initial procedure (76%). Four patients had recurrent infections. One of these patients was successfully reimplanted after a one-stage procedure, two had a second two-stage procedure and have retained their spacers, and one had an amputation. Successful limb salvage in regards to infection control occurred in 14/17 patients (82%). One additional patient required an amputation for an oncologic complication (local recurrence), so the overall limb salvage rate was 13/17 (76%). Patients with megaprosthetic infections following limb salvage treatment for musculoskeletal tumors do not have to be uniformly subject to amputation. Radical debridement and appropriate antibiotics in conjunction with custom spacers followed by selective one- and two-stage reimplantation results in successful limb salvage in 82% of patients. This result is similar to other reports despite the large size average defects

Patients who have limb amputation for musculoskeletal tumours are a rare group of cancer survivors. This was a prospective cross-sectional survey of patients from five specialist centres for sarcoma surgery in England. Physical function, pain and quality of life (QOL) outcomes were collected after lower extremity amputation for bone or soft-tissue tumours to evaluate the survivorship experience and inform service provision.

Of 250 patients, 105 (42%) responded between September 2012 and June 2013. From these, completed questionnaires were received from 100 patients with a mean age of 53.6 years (19 to 91). In total 60 (62%) were male and 37 (38%) were female (three not specified). The diagnosis was primary bone sarcoma in 63 and soft-tissue tumour in 37. A total of 20 tumours were located in the hip or pelvis, 31 above the knee, 32 between the knee and ankle and 17 in the ankle or foot. In total 22 had hemipelvectomy, nine hip disarticulation, 35 transfemoral amputation, one knee disarticulation, 30 transtibial amputation, two toe amputations and one rotationplasty. The Toronto Extremity Salvage Score (TESS) differed by amputation level, with poorer scores at higher levels (p < 0.001). Many reported significant pain. In addition, TESS was negatively associated with increasing age, and pain interference scores. QOL for Cancer Survivors was significantly correlated with TESS (p < 0.001). This relationship appeared driven by pain interference scores.

This unprecedented national survey confirms amputation level is linked to physical function, but not QOL or pain measures. Pain and physical function significantly impact on QOL. These results are helpful in managing the expectations of patients about treatment and addressing their complex needs.

Cite this article: Bone Joint J 2015;97-B:1284–90.

The February 2014 Children’s orthopaedics Roundup³⁶⁰ looks at: flexible plasters; dual 8-plate or ablation for knee epiphysiodesis; ultrasounds for pulled elbow; leg length without the radiation; Boyd amputation in limb deficiencies; gold standard club foot treatment; quadrupled semitendinosis graft effective in paediatric ACL reconstruction; and predicting complications following cerebral palsy hip reconstruction

The February 2014 Oncology Roundup³⁶⁰ looks at: suspicious lesions; limb salvage in pelvic sarcomas; does infection affect oncological survival?; cancer patient pathways; radiological arthritis with cement augmentation in GCT; and post-chemotherapy increase in tumour volume as a predictor of poor prognosis.

Pathological fractures in children can occur as a result of a variety of conditions, ranging from metabolic diseases and infection to tumours. Fractures through benign and malignant bone tumours should be recognised and managed appropriately by the treating orthopaedic surgeon. The most common benign bone tumours that cause pathological fractures in children are unicameral bone cysts, aneurysmal bone cysts, non-ossifying fibromas and fibrous dysplasia. Although pathological fractures through a primary bone malignancy are rare, these should be recognised quickly in order to achieve better outcomes. A thorough history, physical examination and review of plain radiographs are crucial to determine the cause and guide treatment. In most benign cases the fracture will heal and the lesion can be addressed at the time of the fracture, or after the fracture is healed. A step-wise and multidisciplinary approach is necessary in caring for paediatric patients with malignancies. Pathological fractures do not have to be treated by amputation; these fractures can heal and limb salvage can be performed when indicated.

CT and MRI scans are complementary preoperative imaging investigations for planning complex musculoskeletal bone tumours resection and reconstruction. Conventionally, tumour surgeons analyse two-dimensional (2-D) imaging information, mentally integrate and formulate a three-dimensional (3-D) surgical plan. Difficulties are anticipated with increase in case complexity and distorted surgical anatomy. Incorporating computer technology to aid in this surgical planning and executing the intended resection may improve precision. Although computer-assisted surgery has been widely used in cranial biopsies and tumour resection, only small case series using CT-based navigation are recently reported in the field of musculoskeletal tumor surgery. We investigated the results of CT/MRI image fusion for Computer Assisted Tumor Surgery (CATS) with the help of a navigation system. We studied 21 patients with 22 musculoskeletal tumours who underwent CATS from March 2006 to July 2009. A commercially available CT-based spine navigation system (Stryker Navigation; CT spine) was used. Of the 22 patients, 10 were males, 11 were females, and the mean age was 32 years at the time of surgery (range, 6–80 years). Five tumours were located in the pelvis, seven sacrum, eight femurs, and two tibia. The primary diagnosis was primary bone tumours in 16 (3 benign, 13 sarcoma) and metastatic carcinoma in four. The minimum follow-up was 17 months (average, 35.5 months; range, 17–52 months). Preoperative CT and MRI scan of each patient were performed. Axial CT slices of 0.0625mm or 1.25mm thickness and various sequences of MR images in Digital Imaging and Communications in Medicine (DICOM) format were obtained. CT and MR images for 22 cases were fused using the navigation software. All the reconstructed 2-D and 3-D images were used for preoperative surgical planning. The plane of tumour resection was defined and marked using multiple virtual screws sited along the margin of the planned resection. We also integrated the computer-aided design (CAD) data of custom-made prostheses in the final navigation resection planning for eight cases. All tumour resections could be carried out as planned under navigation guidance. Navigation software enabled surgeons to examine all fused image datasets (CT/MRI scans) together in two spatial and three spatial dimensions. It allowed easier understanding of the exact anatomical tumor location and relationship with surrounding structures. Intraoperatively, image guidance with the help of fusion images, provided precise visual orientation, easy identification of tumor extent, neural structures and intended resection planes in all cases. The mean time for preoperative navigation planning was 1.85 hours (1 to 3.8). The mean time for intraoperative navigation procedures was 29.6 minutes (13 to 60). The time increased with case complexity but lessened with practice. The mean registration error was 0.47mm (0.31 to 0.8). The virtual preoperative images matched well with the patients' operative anatomy. A postoperative superficial wound infection developed in one patient with sacral chordoma that resolved with antibiotic whereas a wound infection in another with sacral osteosarcoma required surgical debridement and antibiotic. After a mean follow-up of 35.5 months (17–52 months), five patients died of distant metastases. Three out of four patients with local recurrence had tumors at sacral region. Three of them were soft tissue tumour recurrence. The mean functional MSTS score in patients with limb salvage surgery was 28.3 (23 to 30). All patients (except one) with limb sparing surgery and prosthetic reconstruction could walk without aids. Multimodal image fusion yields hybrid images that combine the key characteristics of each image technique. Back conversion of custom prosthesis in CAD to DICOM format allowed fusion with navigation resection planning and prosthesis reconstruction in musculoskeletal tumours. CATS with image fusion offers advanced preoperative 3-D surgical planning and supports surgeons with precise intraoperative visualisation and identification of intended resection for pelvic, sacral tumors. It enables surgeons to reliably perform joint sparing intercalated tumor resection and accurately fit CAD custom-made prostheses for the resulting skeletal defect

Introduction. Telangiectatic osteosarcoma (TOS) is a rare subtype of osteosarcoma. We review our experience to characterize its prevalence, treatment, relapse and survivorship at long term follow-up. Methods. Eighty-seven patients aged from 4 to 60 years (mean 20 years), were treated from 1985 to 2008. Lesions affected the femur (38), humerus (20), tibia (19), fibula (4), pelvis (3), foot (2) and radius (1). Eight patients had metastatic disease at diagnosis. Seventy-eight patients were treated with neoadjuvant chemotherapy with three or more drugs according to different protocols, nine had surgery as first treatment. Limb salvage surgery was performed in 71 cases, amputation in 14 and rotationplasty in one. One patient died before surgery. Prognostic factors were evaluated with Kaplan-Meier analysis. Results. At a mean follow-up of 8 years, overall survival was 81%, 65% and 65% at 2, 5 and 10 years respectively. Fifty-two patients were disease-free, three were alive with disease, twenty-nine died with disease and three died of other causes. Thirteen local recurrences were observed. Twenty-three patients developed lung (20) or bone (3) metastases. Pathologic fracture did not significantly influence survivorship. Prognostic influence of age of the patients was evaluated at three different cut-off (15, 20 and 25 years-old): younger patients had better survivorship, without statistical significance. Induced necrosis according to Huvos’ classification was significant at both univariate and multivariate regression Cox analysis (p=0.0001). Conclusion. TOS does not have a poor prognosis as previously reported in the literature. A high percentage of patients can be cured with neoadjuvant chemotherapy and surgery. In most patients, limb sparing surgery is possible and safe

We retrospectively reviewed 101 consecutive patients with 114 femoral tumours treated by massive bone allograft at our institution between 1986 and 2005. There were 49 females and 52 males with a mean age of 20 years (4 to 74). At a median follow-up of 9.3 years (2 to 19.8), 36 reconstructions (31.5%) had failed. The allograft itself failed in 27 reconstructions (24%).

Mechanical complications such as delayed union, fracture and failure of fixation were studied. The most adverse factor on the outcome was the use of intramedullary nails, followed by post-operative chemotherapy, resection length > 17 cm and age > 18 years at the time of intervention. The simultaneous use of a vascularised fibular graft to protect the allograft from mechanical complications improved the outcome, but the use of intramedullary cementing was not as successful.

In order to improve the strength of the reconstruction and to advance the biology of host–graft integration, we suggest avoiding the use of intramedullary nails and titanium plates, but instead using stainless steel plates, as these gave better results. The use of a supplementary vascularised fibular graft should be strongly considered in adult patients with resection > 17 cm and in those who require post-operative chemotherapy.

Internal hemipelvectomy is a standard treatment for malignant tumours of the pelvis. Reconstruction using a non-vascularised fibular graft is relatively straightforward compared to other techniques. We describe the surgical and functional outcomes for a series of ten patients who underwent an internal hemipelvectomy (type I or I/IV) with reconstruction by a non-vascularised fibular graft between 1996 and 2009. A key prerequisite for this procedure was a preserved sciatic notch, confirmed pre-operatively on MRI.

Graft-host union was achieved in all patients with a single fibular graft, and in the lower graft where two grafts had been used. The mean time to union was 7.3 months (3 to 12). The upper graft did not unite in four of six cases where two grafts had been used. Seven patients were eventually able to walk without a stick. The mean post-operative Musculoskeletal Tumour Society score was 75.4% (16.7 to 96.7). There were no cases of deep post-operative infection. The mean pelvic shortening was 0.9 cm (0.2 to 3.4). Recurrent tumour occurred in three cases, and death from tumour-related disease occured in one.

Patients who need an internal hemipelvectomy will do well if their pelvic ring is reconstructed with a non-vascularised fibular graft. The complication rate is low, and they attain a good functional outcome.

Background: The incidence of osteosarcoma varies considerably with age and preschool children are extremely rarely affected. This study was conducted to investigate presentation, treatment, and outcome in very young children with osteosarcoma. Patients and methods: The authors retrospectively analyzed the data of 2706 consecutive COSS patients with newly diagnosed high-grade osteosacroma of bone and identified 28 patients (1.0%) aged less than five years at diagnosis. Demographic, diagnostic, tumor, and treatment related variables; response and survival data of these 28 were analyzed. Results: Of the 28 (male, N=16; female, N=12) toddlers, 27 presented with high-grade central osteosarcoma of an extremity (femur, N= 12; humerus, N=10; tibia, N=5) and one with a secondary osteosarcoma of the orbit. The size of primary extremity tumors was large (≥ 1/3 of the involved bone) in 20/27 evaluable patients. Primary metastases were detected in 4 children. All patients received multiagent chemotherapy, and 13/20 analyzed tumors responded well (> 90% necrosis) to neoadjuvant chemotherapy. Limb sparing surgery was performed in 11, ablative procedures were performed in 14, and no local surgery was performed in two patients with extremity tumors. With a median follow-up of 3.8 years (6.2 years for survivors), 13 patients were alive (CR1, N=12; CR3, N=1). Four patients never achieved a complete remission and 12 developed recurrences (local, N=3; metastatic, N=8; site unknown, N=1); and 15 of these 16 patients died. Five-year overall and event free survival probabilities were 50% (SE 10%) and 46% (SE 10%). Better survival was correlated with good response to chemotherapy. Conclusions: Osteosarcoma is extremely rare in pre-school children. These young patients often have large tumors which may require mutilating resections. Prognosis may be poorer than in older patients

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

Introduction: Radical excision and amputation are the surgical options for advanced soft tissue tumours (STS) of the limbs. The sheer size of these advanced tumours in relation to the limb makes limb sparing surgery difficult. The rate of positive margins is high and further management options are quite limited. Patients & Methods: We have identified 17 patients (13 males, 4 females) with sarcomas with skin changes at presentation, including recurrences and primary referrals. The average age was 67 (37–83) years. 11 patients had lower limb tumours and 6 had upper limb tumours. All patients were managed either with radical excision or amputation. Post op radiotherapy and chemotherapy was used s adjuvants when appropriate. All patients were followed up with regular clinical and radiological assessments for recurrences and metastases. The results of adequacy of clearance, recurrences, metastases and overall survival are presented. Results: The follow-up was an average of 30 (7–120) months. Two patients had primary amputations and 15 had wide excision. Four patients had distant metastases at the time of referral. Positive margins were identified in 8 of the 17 patients after primary surgery. 5 patients had a single recurrence and 3 patients had two recurrences. Eight patients needed revision surgery (3 amputations/5 wider excisions) for a positive margin or a recurrence. A total of 8 patients had metastases by 1 year. Overall disease free survival in this cohort was 20 (3–41) months. Conclusion: We encountered a very high rate of positive margins with high morbidity which seems quite common after limb sparing surgery in fungating STS. Amputation comparatively attains better local disease control but probably does not affect the overall survival