Aims

The proximal tibia (PT) is the anatomical site most frequently affected by primary bone tumours after the distal femur. Reconstruction of the PT remains challenging because of the poor soft-tissue cover and the need to reconstruct the extensor mechanism. Reconstructive techniques include implantation of massive endoprosthesis (megaprosthesis), osteoarticular allografts (OAs), or allograft-prosthesis composites (APCs).

Aims. Osteofibrous dysplasia (OFD) is a rare benign lesion predominantly affecting the tibia in children. Its potential link to adamantinoma has influenced management. This international case series reviews the presentation of OFD and management approaches to improve our understanding of OFD. Methods. A retrospective review at three paediatric tertiary centres identified 101 cases of tibial OFD in 99 patients. The clinical records, radiological images, and histology were analyzed. Results. Mean age at presentation was 13.5 years (SD 12.4), and mean follow-up was 5.65 years (SD 5.51). At latest review, 62 lesions (61.4%) were in skeletally mature patients. The most common site of the tibial lesion was the anterior (76 lesions, 75.2%) cortex (63 lesions, 62.4%) of the middle third (52 lesions, 51.5%). Pain, swelling, and fracture were common presentations. Overall, 41 lesions (40.6%) presented with radiological deformity (> 10°): apex anterior in 97.6%. A total of 41 lesions (40.6%) were treated conservatively. Anterior bowing < 10° at presentation was found to be related to successful conservative management of OFD (p = 0.013, multivariable logistic regression). Intralesional excision was performed in 43 lesions (42.6%) and a wide excision of the lesion in 19 (18.8%). A high complication rate and surgical burden was found in those that underwent a wide excision regardless of technique employed. There was progression/recurrence in nine lesions (8.9%) but statistical analysis found no predictive factors. No OFD lesion transformed to adamantinoma. Conclusion. This study confirms OFD to be a benign bone condition with low rates of local progression and without malignant transformation. It is important to distinguish OFD from adamantinoma by a histological diagnosis. Focus should be on angular deformity, monitored with full-length tibial radiographs. Surgery is indicated in symptomatic patients and predicted by the severity of the initial angular deformity. Surgery should focus more on the deformity rather than the lesion. Cite this article: Bone Joint J 2022;104-B(2):302–308

Aims

The aim of this study was to report the results of three forms of reconstruction for patients with a ditsl tibial bone tumour: an intercalary resection and reconstruction, an osteoarticular reconstruction, and arthrodesis of the ankle.

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We retrospectively report our experience of managing 30 patients with a primary malignant tumour of the distal tibia; 25 were treated by limb salvage surgery and five by amputation. We compared the clinical outcomes of following the use of different methods of reconstruction.

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The primary aim of this study was to determine the morbidity of a tibial strut autograft and characterize the rate of bony union following its use.

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We present a retrospective review of patients treated with extracorporeally irradiated allografts for primary and secondary bone tumours with the mid- and long-term survivorship and the functional and radiographic outcomes.

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Intercalary allografts following resection of a primary diaphyseal tumour have high rates of complications and failures. At our institution intercalary allografts are augmented with intramedullary cement and fixed using compression plating. Our aim was to evaluate their long-term outcomes.

Aims. The aim of this study was to identify any progression between benign osteofibrous dysplasia (OFD), OFD-like adamantinoma and malignant adamantinoma, and to investigate the rates of local recurrence, metastases and survival, in order to develop treatment algorithms for each. Patients and Methods. A single institution retrospective review of all patients presenting with OFD, OFD-like adamantinoma and adamantinoma between 1973 and 2012 was undertaken. Complete data were available for 73 patients (42 with OFD; ten with an OFD-like adamantinoma and 21 with an adamantinoma). The mean follow-up was 10.3 years (3 to 25) for OFD, 9.2 years (3.0 to 26.3) for OFD-like and 11.6 years (0.25 to 33) for adamantinoma. Results. The mean age at diagnosis for OFD was 13.5 years (1 to 49), 10.5 years (6 to 28) for OFD-like and 34 years (14 to 86) for adamantinoma. A total of 24 of the 42 patients with OFD (57%) have not required any treatment and have been managed with observation. A total of 18 of the 42 patients with OFD underwent surgery, 13 with curettage and five with resection. In all, three patients developed recurrence following curettage (23%) but none following resection. All these patients were cured with further limited surgery. A total of six patients initially diagnosed with OFD were subsequently found to have OFD-like adamantinoma. Of the ten patients initially diagnosed with OFD-like adamantinoma, three (30%) were managed with observation alone and seven underwent surgery, two with curettage and five with resection. Local recurrence arose in two patients, one each after curettage and resection. No patients with either OFD or an OFD-like adamantinoma developed metastases or had progression to adamantinoma. All patients with an adamantinoma were treated by surgery, three with curettage, six with amputation and 12 with excision. In all, two of the three treated with curettage developed local recurrence, requiring further surgery. Late development of both local recurrence and metastases led to a ten year disease specific survival of 93% which had dropped to 39% by 20 years. Conclusion. We found no evidence of progression from OFD to adamantinoma. Conservative management with observation or curettage is often successful for patients with OFD and OFD-like adamantinoma. Resection with clear margins is required for patients with adamantinoma. Late tumour recurrence is not uncommon in adamantinoma and prolonged follow-up should be considered. Cite this article: Bone Joint J 2017;99-B:409–16

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.

Vascularised fibular grafts (VFGs ) are a valuable surgical technique in limb salvage after resection of a tumour. The primary objective of this multicentre study was to assess the risk factors for failure and complications for using a VFG after resection of a tumour.

The study involved 74 consecutive patients (45 men and 29 women with mean age of 23 years (1 to 64) from four tertiary centres for orthopaedic oncology who underwent reconstruction using a VFG after resection of a tumour between 1996 and 2011. There were 52 primary and 22 secondary reconstructions. The mean follow-up was 77 months (10 to 195).

In all, 69 patients (93%) had successful limb salvage; all of these united and 65 (88%) showed hypertrophy of the graft. The mean time to union differed between those involving the upper (28 weeks; 12 to 96) and lower limbs (44 weeks; 12 to 250). Fracture occurred in 11 (15%), and nonunion in 14 (19%) patients.

In 35 patients (47%) at least one complication arose, with a greater proportion in lower limb reconstructions, non-bridging osteosynthesis, and in children. These complications resulted in revision surgery in 26 patients (35%).

VFG is a successful and durable technique for reconstruction of a defect in bone after resection of a tumour, but is accompanied by a significant risk of complications, that often require revision surgery. Union was not markedly influenced by the need for chemo- or radiotherapy, but should not be expected during chemotherapy. Therefore, restricted weight-bearing within this period is advocated.

Cite this article: Bone Joint J 2015;97-B:853–61.

We report our experience of using a computer navigation system to aid resection of malignant musculoskeletal tumours of the pelvis and limbs and, where appropriate, their subsequent reconstruction. We also highlight circumstances in which navigation should be used with caution.

We resected a musculoskeletal tumour from 18 patients (15 male, three female, mean age of 30 years (13 to 75) using commercially available computer navigation software (Orthomap 3D) and assessed its impact on the accuracy of our surgery. Of nine pelvic tumours, three had a biological reconstruction with extracorporeal irradiation, four underwent endoprosthetic replacement (EPR) and two required no bony reconstruction. There were eight tumours of the bones of the limbs. Four diaphyseal tumours underwent biological reconstruction. Two patients with a sarcoma of the proximal femur and two with a sarcoma of the proximal humerus underwent extra-articular resection and, where appropriate, EPR. One soft-tissue sarcoma of the adductor compartment which involved the femur was resected and reconstructed using an EPR. Computer navigation was used to aid reconstruction in eight patients.

Histological examination of the resected specimens revealed tumour-free margins in all patients. Post-operative radiographs and CT showed that the resection and reconstruction had been carried out as planned in all patients where navigation was used. In two patients, computer navigation had to be abandoned and the operation was completed under CT and radiological control.

The use of computer navigation in musculoskeletal oncology allows accurate identification of the local anatomy and can define the extent of the tumour and proposed resection margins. Furthermore, it helps in reconstruction of limb length, rotation and overall alignment after resection of an appendicular tumour.

Cite this article: Bone Joint J 2015;97-B:258–64.

The purpose of this study is to provide a systematic review of the literature and assess outcome of our experience of Ilizarov Bone Transport in reconstruction for primary malignant tumours of bone (PMTB). A systematic review of the literature for reported cases of primary reconstruction of PMTB using distraction osteogenesis was performed. All cases of distraction osteogenesis for primary reconstruction of PMTB in our institution were reviewed. Outcome was determined from retrospective review of case notes and radiology. Patients were contacted to define final status. There are few cases of primary reconstruction of PMTB using Ilizarov method in the literature. Most reports relate to benign tumours or reconstruction of secondary deformities or non-union after tumour resection. At our institution we have treated 7 patients with bone defects resulting from excision of a PMTB. Mean age was 42.1 years (23–48). Tumours occurred in the tibia in 4 cases and the femur in 3 cases. Histologic diagnosis was chondrosarcoma in 3, malignant fibrous histiocytoma in 2, adamantinoma in 1 and malignant intraosseous nerve sheath tumour in 1. All patients were assessed through the hospital sarcoma board and shown to have isolated bone lesions without metastases. Mean bone defect after resection was 13.1 cm (10–17). Mean frame time was 13.6 months (5–23). Mean follow-up was 46 months (15–137). Complications included pin infection, docking site non-union, premature fusion of corticotomy, soft tissue infection and minor varus deformity. There was one local recurrence of tumour at five months after resection, resulting in a through hip disarticulation. The other cases remain tumour-free with united, well-aligned bones and acceptable long-term function. PMTB is rare and poses a major reconstructive dilemma. Distraction osteogenesis provides an effective method of biologic reconstruction in selected cases, and good outcomes can be achieved

A systematic literature review of distraction osteogenesis (DO) for the primary reconstruction of bone defects following resection of primary malignant tumours of long bones (PMTLB) is presented. Fewer than 50 cases were identified. Most reports relate to benign tumours or secondary reconstructive procedures. The outcomes of our own series of 7 patients is also presented (4 tibiae, 3 femora). All patients had isolated bone lesions without metastases and were assessed through the hospital sarcoma board. Mean follow-up was 59 months (17–144). Mean age was 42 years. Final histologic diagnoses were 3 chondrosarcoma, 2 malignant fibrous histiocytoma, 1 adamantinoma and 1 malignant intraosseous nerve sheath tumour. Mean bone defect after resection was 13.1cm (10–17) and bone transport was the reconstruction method in all. There was one local recurrence of tumour six months post-resection, necessitating amputation. Mean frame index for remaining cases was 30.9 days/cm (15.7–41.6). Complications included pin infection, docking site non-union, premature corticotomy union, soft-tissue infection and minor varus deformity. Six cases remain tumour-free with united, well-aligned bones and good long-term function. We conclude DO provides an effective biologic reconstruction option in select cases of PMTLB

Purpose. To investigate the incidence of deep vein thrombosis (DVT) and pulmonary embolism (PE) with external fixator use and to help establish whether current guidelines are appropriate. Methods. Case notes of individuals undergoing external fixator application by the senior author (PC) from March 2005 to June 2011 were examined. In this period 207 individuals underwent 255 primary applications of Ilizarov, Taylor Spatial Frame (TSF) or monolateral fixator. Fixators applied were 173 tibial, 63 femoral and 19 to other bones. Records were obtained for 182 individuals (88%), representing 214 operations (84%). Results. Two cases of DVT were found (1%). In both cases mechanical and chemical prophylaxis had been used, as guided by risk assessment. One of these individuals also experienced a PE. This person was of notably high risk, surgery involving excision of tibial adamantinoma and a high body mass index (45). He had also recently travelled from overseas (a travel time of over 3 hours). Other than surgical time there was one additional risk factor in 39 cases, two additional risk factors in four and three additional risk factors in four. Conclusions. Little evidence exists about the incidence of DVT and PE with the use of external fixators. The National Institute of Health and Clinical Excellence (NICE) provide guidance for thromboprophylaxis in orthopaedic surgery with specific recommendations for hip and knee arthroplasty and hip fracture. For orthopaedic surgery other than lower limb arthroplasty NICE recommends thromboprophylaxis is guided by risk factors on a case by case basis. Our findings raise the question of whether extended course chemical thromboprophylaxis, as for lower limb arthroplasty, should be employed for high risk individuals undergoing application of external fixator

Introduction. No published work exists regarding deep vein thrombosis (DVT) and pulmonary embolism (PE) incidence with the elective use of external fixators. The aim of this work was to establish the rate of DVT and PE in such cases to help inform whether thromboprophylaxis guided by risk factors is adequate or if a more aggressive approach is required. Patients and methods. Information from a prospectively maintained electronic database and case notes were examined for consecutive patients from March 2005 to June 2011. Occurrence of DVT and PE, detected by ultrasound or CT angiogram, were recorded. Risk factors for thromboembolism, age, weight, height, surgical indications, type of surgery and operative time were recorded. As recommended by the National Institute for Health and Clinical Excellence (NICE) thromboprophylaxis use is guided by risks of thromboembolism and bleeding. For adults and older adolescent patients contralateral leg compression stockings and an intraoperative calf pump were used. Mobilisation began the morning after surgery and the majority of cases permitted to bear weight fully. Results. Two hundred and seven (207) individuals underwent 255 primary applications of Ilizarov, Taylor Spatial Frame (TSF) or monolateral fixator, 173 tibial, 63 femoral and 19 to other bones. Case notes were obtained for 182 individuals (88%), representing 214 operations (84%). One DVT and one PE were recorded, an overall incidence of 2/214 (0.9%) (excluding those under 16 years old 2/143 (1.4%)). In both cases mechanical and chemical prophylaxis had been used as guided by risk assessment. The PE was sustained by a person of notably high risk, surgery involving excision of tibial adamantinoma and a high body mass index (45). He had also recently travelled from overseas (a travel time of over 3 hours). Conclusions. The rate of DVT/PE for elective application of external fixators is low with risk assessment guiding prophylaxis

Adamantinoma are rare, low grade malignant, bone tumors, making up only 0.1–0.48 percent of primary malignant bone tumors. They occur predominantly in the long bones, especially the tibia. Histogenetically it is thought that it originates from embryological displacement of basal epithelium of the skin, although other hypotheses have been proposed. Clinically most patients present with swelling and possible bending of the tibia, painful or painless. It's often noticed in an earlier stadium, but symptoms are non-specific and have a slow progressive character. Median patient age is 25 to 35 years, with a range from two to 86 years. It is slightly more common in men than woman, with a ratio of 5:4. Occurrence in children is even rarer. A study by Van Rijn et al. finds only 119 references, and presents six more cases. Treatment is the same. An MRI-scan should be performed to check for metastasis, loco regional staging and for operative planning. Operative excision and reconstruction is necessary to prevent metastasis and maintain load bearing capacity. Generally these resections and reconstructions are done without objective measurements. The surgeon uses a rule of thumb, like a sculptor, or ruler approach to recreate the excised bone, either with allo- or autograft materials. An optimal fit, i.e. a minimal space between tibia and graft, is not always achieved, possibly resulting in pathological fractures. This risk of pathological fractures lengthens recovery time. The fractures elongate hospitalization time and recovery time and are a heavy burden to patients. Computer assisted surgery (CAS) systems, used for example in prosthesis placement, offer objective measurements in 3d space of hard structures with high accuracy. These can be used to produce an accurate copy of the resected bone. If the reconstruction accurately fits the bone defect that's left after the resection, it's likely that the occurrence of pathological fractures decreases. An adamantinoma in the tibia of a 12 year old boy was treated. Surgery consisted of hemicortical resection and inlay allograft reconstruction. The software used was the Orthomap navigation software (Stryker). A donor bone was supplied with help from the bone bank. The technical approach to the reconstruction was the planning of resection planes around the tumor. As the CT scale for both the patient and allograft bone is the same, the resection planes in the patient navigation setup could be copied to the allograft creation setup. Normal CAS setup was performed after first incision, with a tracker attached to the tibia. It was planned that a navigated bone saw would be used for the cutting. The tracker was attached to the saw with a new attachment, and calibrated in the universal calibration tool. During the surgery the oscillating saw proved to be impossible to navigate. The instrument calibration module was not able to accurately registered the saw, this despite accurate registrations in pre-operative testing. The CAS system was used however for accurately determining the saw planes. The planes were traced with the pointer tool. Then a non-navigated saw was used to perform both trapezoid shaped resections. A similar CAS setup was performed on the donor bone. The reconstruction was a good fit. The skin was closed in layers. Post-operative x-ray control was performed. Operation time was just over two hours. Currently the follow-up time is five months. There have been no complications and the control x-rays show good allograft ingrowth. While the original operation plan couldn't be performed the principle of computer assisted reconstruction has its merits. This was a proof of concept. The navigation was accurate to less than 1 mm, and the trapezoid resection shape guarantees a good fit. However the method of resection of the drawn planes by non-navigated bone saw was not accurate enough, because of the saw oscillations. There was improvement in operation time. With more accurate means of resection, as for example a computer controlled laser or water-jet, this type of reconstruction could have other very interesting applications

We evaluated the oncological and functional outcome of 18 patients, whose malignant bone tumours were excised with the assistance of navigation, and who were followed up for more than three years. There were 11 men and seven women, with a mean age of 31.8 years (10 to 57). There were ten operations on the pelvic ring and eight joint-preserving limb salvage procedures. The resection margins were free of tumour in all specimens. The tumours, which were stage IIB in all patients, included osteosarcoma, high-grade chondrosarcoma, Ewing’s sarcoma, malignant fibrous histiocytoma of bone, and adamantinoma. The overall three-year survival rate of the 18 patients was 88.9% (95% confidence interval (CI) 75.4 to 100). The three-year survival rate of the patients with pelvic malignancy was 80.0% (95% CI 55.3 to 100), and of the patients with metaphyseal malignancy was 100%. The event-free survival was 66.7% (95% CI 44.9 to 88.5). Local recurrence occurred in two patients, both of whom had a pelvic malignancy. The mean Musculoskeletal Tumor Society functional score was 26.9 points at a mean follow-up of 48.2 months (22 to 79). We suggest that navigation can be helpful during surgery for musculoskeletal tumours; it can maximise the accuracy of resection and minimise the unnecessary sacrifice of normal tissue by providing precise intra-operative three-dimensional radiological information

The purpose of this study was to assess the outcome of 15 patients (mean age 13.6 years (7 to 25)) with a primary sarcoma of the tibial diaphysis who had undergone excision of the affected segment that was then irradiated (90 Gy) and reimplanted with an ipsilateral vascularised fibular graft within it.

The mean follow-up was 57 months (22 to 99). The mean time to full weight-bearing was 23 weeks (9 to 57) and to complete radiological union 42.1 weeks (33 to 55). Of the 15 patients, seven required a further operation, four to obtain skin cover. The mean Musculoskeletal Society Tumor Society functional score at final follow-up was 27 out of 30 once union was complete. The functional results were comparable with those of allograft reconstruction and had a similar rate of complication.

We believe this to be a satisfactory method of biological reconstruction of the tibial diaphysis in selected patients.

Introduction. We analyzed the results of extracorporeal radiated (ECRT) autogenous tumour bone for reconstruction of diaphyseal defects after tumour resection at our institute. Methods. Sixteen diaphyseal bone tumours operated between March 2006 to March 2008 were reconstructed with ECRT bone after appropriate oncologic resection. These included 10 cases of Ewing's sarcoma, 5 of Osteosarcoma and 1 Adamantinoma. Nine involved femur, 5 tibia and 2 humerus. Suitable internal stabilisation (14 cases plate fixation, 2 intramedullary nails) was used after re-implanting ECRT bone. Average resection length was 18.9 cm (range, 11 to 30 cm). Results. There were 32 osteotomy sites in 16 patients. 4 cases with infection resulting in graft removal were excluded. Thus 23 osteotomy sites (one patient had a bipolar prosthesis inserted into re-implanted bone) were analysed for union. The average union time was 6.8 months. 12 metaphyseal sites united at average 6 months and 11 diaphyseal ends at average 7.8 months. 2 cases developed hypertrophic nonunion (both at diaphyseal sites) requiring revision and bone grafting. Reconstruction related complication were seen in 7(43%) out of 16 patients. Infection in 4 cases (25%), required multiple lavages, debridement and ultimately removal of the graft in all. The average follow up was 33 months (range, 27 to 45 months). There were 3 local recurrences (2 bony, 1 soft tissue), none of them in re-implanted bone. Four patients died of metastatic disease and 1 due to unrelated causes. At time of final review all survivors except one were capable of independent ambulation with an average MSTS Score of 26 (range, 18 – 29). Conclusion. Extracorporeal irradiation is an oncologically sound procedure with good functional outcomes. It is a convenient and inexpensive technique for limb salvage avoiding the logistic issues involved in allograft procurement and specialized expertise for microvascular anastomosis