Aim. Synovial sarcoma (SS) is a malignant soft tissue sarcoma with a poor prognosis because of late local recurrence and distant metastases. To our knowledge, no studies have minimum follow-up of 10 years that evaluate long-term outcomes for survivors. Method. Data on 62 patients who had been treated for SS from 1968 to 1999 were studied retrospectively in a multicenter study. The following parameters were examined for their potential prognostic value: age at diagnosis, sex, tumour site and size, histology, histological grade, fusion type (SYT-SSX1 vs. SYT-SSX2), and surgical margin status. Mean follow-up of living patients was 17.2 years, and of dead patients 7.7 years. Results. Mean age at diagnosis was 35.4 years (range, 6-82 years). Overall survival was 38.7%. The 5-year survival was 74.2%; 10-year survival was 61.2%; and 15-year survival was 46.5%. Fifteen patients (24%) died of disease after 10 years of follow-up. Local recurrence occurred after a mean of 3.6 years (range, 0.5-14.9 years), and metastases, at a mean of 5.7 years (range, 0.5-16.3 years). Only 4 patients (7%) were treated technically correctly. Factors associated with significantly worse prognosis included larger tumour size, metastases at the time of diagnosis, high-grade histology, trunk-related disease, and lack of wide resection as primary surgical treatment. Conclusion. In SS, metastases develop late with high mortality. Patients with SS should be followed for more than 10 years. There is a very high rate of incorrectly treated patients. Patients with suspicious lesions therefore should be sent to a referral center

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.

Aims. The purpose of this study was to report the long-term results of extendable endoprostheses of the humerus in children after the resection of a bone sarcoma. Methods. A total of 35 consecutive patients treated with extendable endoprosthetic replacement of the humerus in children were included. There were 17 boys and 18 girls in the series with a median age at the time of initial surgery of nine years (interquartile range (IQR) 7 to 11). Results. The median follow-up time was 10.6 years (IQR 3.9 to 20.4). The overall implant survival at ten years was 75%. Complications occurred in 13 patients (37%). Subluxation at the proximal humerus occurred in 19 patients (54%) but only six (17%) were symptomatic. Subluxation was seen more commonly in children under the age of nine years (86%) than in those aged nine years or more (33%) (p = 0.002). Implant failure occurred in nine patients (26%): the most common cause was aseptic loosening (four patients, 11%). Lengthening of the implant was carried out in 23 patients (66%). At final follow up, three patients had a limb that was shortened by 5 cm or more. The mean Musculoskeletal Tumor Society (MSTS) functional score was 79% (73% to 90%). Conclusion. Extendable endoprosthetic replacement is a reliable method of reconstructing humerus after excision of a bone sarcoma. Children who are less than nine years old have a high risk of subluxation. Cite this article: Bone Joint J 2020;102-B(1):64–71

Aims. The aim of this study was to evaluate the prosthesis characteristics and associated conditions that may modify the survival of total femoral endoprosthetic replacements (TFEPR). Patients and Methods. In all, 81 patients treated with TFEPR from 1976 to 2017 were retrospectively evaluated and failures were categorized according to the Henderson classification. There were 38 female patients (47%) and 43 male patients (53%) with a mean age at diagnosis of 43 years (12 to 86). The mean follow-up time was 10.3 years (0 to 31.7). A survival analysis was performed followed by univariate and multivariate Cox regression to identify independent implant survival factors. Results. The revision-free survival of the implant was 71% at five years and 63.3% at ten years. Three prostheses reached 15 years without revision. The mean Musculoskeletal Tumor Society score in the group was 26 (23 to 28). The mechanisms of failure were infection in 18%, structural failures in 6%, tumour progression in 5%, aseptic loosening in 2%, and soft-tissue failures in 1%. Prostheses used for primary reconstruction after oncological resections had lower infection rates than revision implants (8% vs 25%; p = 0.001). The rates of infection in silver-coated and non-silver-coated prosthesis were similar (17.4% vs 19.%; p = 0.869). The incidence of hip dislocation was 10%. Rotating hinge prosthesis had a lower failure rate than fixed hinge prosthesis (5.3% vs 11%). After Cox regression, the independent factors associated with failures were the history of previous operations (hazard ratio (HR) 3.7; p = 0.041), and the associated arthroplasty of the proximal tibia (HR 3.8; p = 0.034). At last follow-up, 11 patients (13%) required amputation. Conclusion. TFEPR offers a reliable reconstruction option for massive bone loss of the femur, with a good survival when the prosthesis is used as a primary implant. The use of a rotating hinge at the knee and dual mobility bearing at the hip may be adequate to reduce the risk of mechanical and soft-tissue failures. Infection remains the main concern and there is insufficient evidence to support the routine use of silver-coated endoprosthesis. Cite this article: Bone Joint J 2019;101-B:522–528

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

Aim. To identify factors leading to the success or failure of extracorporeal irradiation and re-implantation of bone (ECIR). Method. Review of experience of this technique since 1996 documenting successes and failures. Results. 35 patients with a variety of malignant bone tumours underwent ECIR from 1996 up to 2007. The most common diagnosis was Ewings' sarcoma (15) followed by osteosarcoma (9) and chondrosarcoma (5) with the most common sites being the pelvis (15) followed by the tibia (10) and humerus (3). The age range was from 7 to 66 and 8 were skeletally immature at the time of surgery. The bones were sterilised with a dose of 90Gy before reimplantation. The overall patient survival was 69% at 5 years and 62% at 10 years. Local recurrence arose in 4 cases, 3 of whom already had metastatic disease. None of the LR arose in the irradiated bone. The rate of non union was 9% at the 58 osteotomies, the greatest risk being in the tibia. There were four graft fractures of which 3 needed fixation and all united. There is one case of convincing graft resorbtion after 12 years. Two patients developed infections but there have been none since the bone was routinely immersed in antibiotics whilst being irradiated. Conclusion. ECIR is a useful technique with very limited indications. In the majority of cases it works well. It appears particularly useful in pelvic resections and diaphyseal resections. Distal tibial reconstructions do worst. The irradiated bone needs reinforcing with either cement or a bone graft for best results

Chondrosarcoma of bone is a surgical disease and excision with wide margins is the optimum treatment. Sometimes the size or location of the tumour at the time of diagnosis mean that only a marginal excision can be achieved. The effect of the margin of excision on outcome is investigated. Method. All patients with newly diagnosed primary chondrosarcoma of bone and without metastases at the time of diagnosis were identified from a prospective database. Their outcome was investigated to assess whether the margin of excision affected outcome. Results. 492 patients were included in the analysis with a mean age of 48. The mean tumour size was 11cm and 59% were male. The 10 year tumour specific survival was 85% for clear cell (N=7) and grade 1 (N=210), 60% for grade 2 (N=180), 47% for grade 3 (N=59) and 16% for dedifferentiated (N=36). The 10 year local recurrence free survival was 86% (clear cell), 73% (grade 1), 67% (grade 2), 36% (grade 3) and 56% dedifferentiated. Local recurrence was strongly related to older age (p=0.0065), grade (p⋋0.0001) and margins (p⋋0.0001). Patients who developed local recurrence had a 43% survival at 10 years compared with 76% for those who did not (p⋋0.0001). They also had a 49% risk of developing metastases compared to a 17% risk for those without local recurrence (p⋋0.0001). However most of these patients had metastases before developing LR(57%). 16 of 65 patients with local recurrence but no metastases, died due to local progression (usually pelvic or spinal tumours). There was however no relation of the margin of excision to either the risk of developing metastases or survival for any grade of tumour. Conclusion. Whilst local control is strongly related to margins of excision and grade, overall survival is related principally to grade but not to margins

Neoadjuvant chemotherapy for osteosarcoma improves outcomes for the majority, but if the chemotherapy does not work then the dilemma often arises as to whether to do limb salvage with a marginal (or worse) margin of excision or to do an amputation. If limb salvage is carried out with a close margin, does post operative radiotherapy make any difference? This study aims to address these questions. Method. All patients with limb osteosarcoma, no metastases, a poor response to chemotherapy and either a marginal excision or primary amputation were identified from a prospective database. This group were investigated in terms of overall survival and local control. Results. There were 182 patients in this category of whom 60 had an amputation, 105 limb salvage with marginal margins and 17 with an intralesional margin. Local recurrence (LR) arose in 41% of those with an intralesional margin, 22% of those with a marginal margin and 13% of those with an amputation. Radiotherapy was used in 21 of the 122 patients and the risk of LR was the same as in those who did not have radiotherapy. Neither age nor sex of the patient, size or site of the tumour affected the risk of LR. The overall survival for this group was 42% at 10 years. The survival was best in those with marginal margins (38%) than those with an amputation (28%) and worst for those with an intralesional margin (20%). Survival was worst in those who did develop LR, but no worse than in those having amputation. Conclusion. A marginal resection of osteosarcoma with a poor response to chemotherapy leads to a high risk of local recurrence but also carries a poor prognosis. Carrying out an amputation to avoid the risk of LR probably has little survival benefit and the use of radiotherapy remains unclear

Background. Cryosurgery is a well established modality in the treatment of benign aggressive and low grade malignant tumours. In this setting it allows for intra-lesional resection and preservation of function without compromising oncological outcome. Here we present the outcome of 87 patients treated with cryosurgery for low-grade chondrosarcoma of bone. Materials and methods. 87 patients were treated between 1988 and 2005. The mean age was 51 years (range, 8-77 years), and included 47 females and 40 males. Minimal follow-up was two years. Patients were treated for lesions of the distal femur (n = 30), proximal humerus (n = 33), proximal femur (n = 5), proximal tibia (n = 10), and the remaining sites included the iliac bone, distal tibia, forearm, carpal and tarsal bones (n=10). Patients were treated with intralesional curettage through a cortical window, adjuvant burr drilling, cryotherpay and reconstruction with cement or bone graft and hardware fixation when that was clinically indicated. Results. Post operative pathology reports showed high grade sarcomas in two patients, which underwent subsequent wide resection and prosthetic replacement and are excluded from this report. No local recurrence was noted after minimal follow up of two years in the remaining 85 patients. Post-operative complications included; fractures (n=4) of which two needed surgical fixation, superficial wound infection (n=5), delayed wound healing due to “cold burns” (n=6), degenerative arthritis which necessitated joint replacement (n=3), all more than 10 years after initial tumour resection. Functional outcome was rated as good or excellent in 76 patients (89%). Five patients had moderate function (6%), all of them with tumours of the proximal humerus and no outcome was available for 4 patients (5%). Conclusions. Low complication rates, good functional outcome and very low local recurrence rates can be obtained when cryoablative surgery is performed for low grade chondrosarcomas of bone

Retroperitoneal sarcomas (RS) are rare tumours that may reach considerable size at diagnosis and should optimally be treated by a specialized multidisciplinary team. In Sweden, we have since 10 years enforced referral of RS to sarcoma centers, but have experienced a considerable delay, which provides the basis for this study on delays in RS diagnosis and treatment. We identified 33 patients treated for RS at the Southern Sweden Sarcoma Center (covering a population of 1.5 million), Lund University Hospital between 2003-2009. Data, including onset of symptoms, time to diagnosis and time to treatment were recorded from clinical files. Patient's delay was defined as the time from onset of symptoms to the first visit to a doctor, which could be a general practitioner or a specialist. Doctor's delay was defined as the time from the first visit to a doctor to the start of treatment, which was in most cases surgery. In total, 30 patients were referred to the sarcoma centre for treatment. Complete data are available from 25 patients (13 men) with a median age of 62 (20-86) years. Median patient's delay was 15 days (0-9 months) and median doctor's delay was 97 days (0-40 months). Median doctor's delay was indeed somewhat longer (52 days) at the sarcoma centre than at the local hospitals (38 days). Some of the longest delays were caused by primary erroneous diagnosis (16 and 40 months) and comorbidity (4, 8 and 19 months) that required other medical procedures before surgery. Though almost all patients with RS in Southern Sweden are referred to a sarcoma centre for treatment, delays are considerable for many patients with doctor's delays outnumbering patient's delays. Our findings demonstrate that centralisation per se is not sufficient to treat RS, but that optimized diagnostics and clinical management is needed also at sarcoma centers

Aim. Assess symptoms and diagnostic problems of chest wall chondrosarcoma and factors related to long doctor's delay. Methods. The material included all 106 consecutive patients with chondrosarcoma of the chest wall diagnosed in Sweden 1980-2002. Pathological specimens were re-evaluated and graded by the Scandinavian Sarcoma Group pathology board. Files from the very first medical visit for symptoms related to the chondrosarcoma were traced and used to characterise the initial symptoms, calculate patient's and doctor's delay and to identify factors contributing to the delays. Results. The most prominent initial symptom was a palpable mass found in (73/106) 69 % of the patients at the first visit. A tumour was suspected at the first visit in 85 % of the patients. Patients delay was in median 3 (0-118) months and doctor's delay was 4 (0.1-190) months. Doctor's delay was > 6 months for 40 % of the patients. Patients with an initial plain chest radiograph interpreted as normal (34 patients), and/or normal or inconclusive results of a fine-needle aspiration biopsy had longer doctor's delay. Fine-needle aspiration biopsy done at non-specialty units resulted in only 26 % correct malignant diagnoses; at sarcoma centres 94 % were correctly diagnosed. Only 57 % of the patients were operated at a sarcoma centre and 43 % at non-specialty hospitals. The local recurrence rate was 16 % and 57 %, respectively. The 10 years survival for patients treated at sarcoma centres was 75 % but only 59 % for those not referred. Conclusion. Chest wall chondrosarcoma presents as a lump, usually painless. Plain chest radiographs and fine-needle aspiration, when done at non-specialty hospitals are often normal or inconclusive. The diagnostic difficulties led to long doctor's delay and to inadequate treatment leading to poor outcome. Patients should be referred to sarcoma centres for diagnosis and treatment

We report the results of the treatment of nine children with an aneurysmal bone cyst of the distal fibula (seven cysts were juxtaphyseal, and two metaphyseal). The mean age of the children was 10 years and 3 months (7 years and 4 months to 12 years and 9 months). All had open physes. All cysts were active and in seven cases substituted and expanded the entire width of the bone (type-2 lesions). The mean longitudinal extension was 5.7 cm (3 to 10). The presenting symptoms were pain, swelling and pathological fracture. Moderate fibular shortening was evident in one patient. In six patients curettage was performed, using phenol as adjuvant in three. Three with juxtaphyseal lesions underwent resection. A graft from the contralateral fibula (one case) and allografts (two cases) were positioned at the edge of the physis for reconstruction. The mean follow-up was 11.6 years (3.1 to 27.5). There was no recurrence. At the final follow-up there was no significant difference in the American Orthopaedic Foot and Ankle Society scores (excellent/good in all cases) and in growth disturbance, alignment, stability and bone reconstitution, but in the resection group the number of operations, including removal of hardware, complications (two minor) and time of immobilisation/orthosis, were increased. Movement of the ankle was restricted in one patient. The potential risks in the management of these lesions include recurrence, physeal injury, instability of the ankle and hardware and graft complications. Although resection is effective it should be reserved for aggressive or recurrent juxtaphyseal lesions

Aim. Ten years ago at the EMSOS 2000 meeting we have presented our experience concerning the non-surgical treatment of stage IIB osteosarcoma of extremities. The purpose of study was to evaluate long-term results and complications related to this non-standard and controversial treatment modality. Methods. Since 1988 definitive radiation gamma therapy (RT) in the median dose of 60 Gy was administrated to 36 patients who refused surgery after preoperative chemotherapy. Until 1999 induction consisted in 3-5 cycles of intra-arterial doxorubicin (DOX) 75 mg/m2 or cis-platin (CDDP) 120 mg/m2. Since 2000 induction comprised 4 cycles of DOX 90 mg/m2+CDDP 120 mg/m2 intra-arterially. Tumour response before local treatment was assessed non-invasively by means of X-ray, CT, MRI, angiography, 3-phase bone scans and alkaline phosphatase measurement. The end-points were overall (OS), metastases free (MFS) and local progression free (LPFS) survival at 10 years. Results. OS, MFS and LPFS by Kaplan-Meier method were 48±9%, 51±9%, and 45±11% respectively. The outcome was significantly related to response assessed before RT. In 12 good responders OS, MFS and LPFS were 74±13%, 91±9%, and 69±19% respectively compared with 29±11%, 27±11%, and 34±13% in 24 non-responders, p- value 0.03, 0.003, 0.009, respectively. In 14 patients 5 years survival after local progression and delayed surgery was 38±10%. Median survival time after local relapse was 36 months. Two types of major complications were seen: 7 pathological fractures and one osteomyelitis. 21 patients (58%) remained with a functional limb until the last examination or death. Conclusions. Long-term results were higher than expected in this radioresistant tumour. In good responders to induction chemotherapy RT could be an option for local treatment. Majority of patients had a functional limb during and long time after treatment. Isolated late local relapses did not influence considerably systemic control

Introduction. The aim of this study was to investigate the results of a series of cases from a single institution with respect to local disease control and patient survival to determine prognostic factors. Methods. Electronic patient records were reviewed on all patients with STS between February 1963 and January 2007. 2445 patients had over 30 types of STS. 1639 (67%) had not received any treatment prior to presentation, however, 770 patients (32%) had undergone a previous attempted excision. Survival analyses were done using Kaplan Meier and Cox regression analyses, however, for prognostic factor analysis, only patients presenting without prior treatment were included. Results. Common diagnoses were liposarcoma (292 patients, 12%), synovial sarcoma (242 patients, 10%) and leiomyosarcoma (239 patients, 10%). Most presented in the thigh (950 patients, 39%), arm (325 patients, 13%) or lower leg (275 patients, 11%) and most were deep to fascia (1581 patients, 74%). The mean size was 10.2cm. Overall cumulative patient survival was 58% at 5 years and 44% at 10 years. Locally recurrent disease occurred in 350 patients (14%), 204 patients (8%) presented with and 720 patients (30%) subsequently developed metastatic disease. Prognostic factors for locally recurrent disease were arm tumours (p=0.003, HR=0.3), hip tumours (p=0.01, HR=0.31), thigh tumours (p=0.002, HR=0.52), intralesional margins (p<0.0001, HR=3.7), high grade tumours (p=0.03, HR=1.8), tumour size 3-6cm (p=0.04, HR=0.54) and tumour size 6-10cm (p=0.03, HR=0.63). Prognostic factors for patient survival were deep location (p=0.02, HR=1.6), high grade tumours (p<0.0001, HR=4.7), intermediate grade tumours (p<0.0001, HR=3.4), surgical margins (p=0.04), age at diagnosis (p<0.0001, HR=1.02), size of tumour <3cms (p=0.04, HR=0.29), 3-6cms (p<0.0001, HR=0.41), 6-10cms (p=0.007, HR=0.63), no locally recurrent disease (p=0.0001, HR=0.59). Conclusions. Significant prognostic factors have been proven for STS, and marginal margins have not been proven to alter the risk of locally recurrent disease or patient survival

We reviewed the results of 51 patients with benign bone tumours treated by curettage and implantation of calcium hydroxyapatite ceramic (CHA). The mean follow-up was 11.4 years (10 to 15.5). Post-operative fractures occurred in two patients and three had local recurrences; three had slightly limited movement of the adjacent joint and one had mild osteoarthritis. There were no allergic or neoplastic complications. In all cases, radiographs showed that the CHA was well incorporated into the host bone. Statistical analysis showed that absorption of the implanted CHA was greater in males (odds ratio, 6.2; 95% CI, 1.6 to 23.7) and younger patients (odds ratio, 0.6 for increase in age of 10 years; 95% CI, 0.91 to 0.99). However, the implanted CHA was not completely absorbed in any patient. We conclude that CHA is a useful and safe bone substitute for the treatment of benign bone tumours

We retrospectively evaluated 18 patients with a mean age of 37.3 years (14 to 72) who had undergone pelvic reconstruction stabilised with a non-vascularised fibular graft after resection of a primary bone tumour. The mean follow-up was 10.14 years (2.4 to 15.7). The mean Musculoskeletal Tumor Society Score was 76.5% (50% to 100%). Primary union was achieved in the majority of reconstructions within a mean of 22.9 weeks (7 to 60.6). The three patients with delayed or nonunion all received additional therapy (chemotherapy/radiation) (p = 0.0162). The complication rate was comparable to that of other techniques described in the literature. Non-vascularised fibular transfer to the pelvis is a simpler, cheaper and quicker procedure than other currently described techniques. It is a biological reconstruction with good results and a relatively low donor site complication rate. However, adjuvant therapy can negatively affect the outcome of such grafts

We have studied 560 patients with osteosarcoma of a limb, who had been treated by neoadjuvant chemotherapy, in order to analyse the incidence of local and systemic recurrence according to the type of surgery undertaken. Of these, 465 patients had a limb-salvage procedure and 95 amputation or rotationplasty. At a median follow-up of 10.5 years there had been 225 recurrences. The five-year disease-free survival and overall survival rates were 60.7% and 68.5%, respectively, with no significant difference between patients undergoing amputation and those undergoing resection. The incidence of local recurrence was the same for patients treated by either amputation or limb salvage and correlated significantly with the margins of surgical excision and the histological response to chemotherapy. The outcome for patients with a local recurrence was significantly worse than for those who had recurrent disease with metastases only. We conclude that limb-salvage procedures are relatively safe in osteosarcoma treated by neoadjuvant chemotherapy. They should, however, only be performed in institutions where the margins of surgical excision and the histological response to chemotherapy can be accurately assessed. If the margins are inadequate and the histological response to chemotherapy is poor an immediate amputation should be considered

In this case study, we describe the clinical presentation and treatment of 36 patients with periosteal chondrosarcoma collected over a 59-year period by the archive of the Netherlands Committee on Bone Tumours. The demographics, clinical presentation, radiological features, treatment and follow-up are presented with the size, location, the histological grading of the tumour and the survival.

We found a slight predominance of men (61%), and a predilection for the distal femur (33%) and proximal humerus (33%). The metaphysis was the most common site (47%) and the most common presentation was with pain (44%). Half the tumours were classified histologically as grade 1. Pulmonary metastases were reported in one patient after an intra-lesional resection. A second patient died from local recurrence and possible pulmonary and skin metastases after an incomplete resection.

It is clearly important to make the diagnosis appropriately because an incomplete resection may result in local recurrence and metastatic spread. Staging for metastatic disease is recommended in grade II or III lesions.

These patients should be managed with a contrast-enhanced MRI of the tumour and histological confirmation by biopsy, followed by en-bloc excision.

Cite this article: Bone Joint J 2014;96-B:823–8.

Monostotic fibrous dysplasia of the proximal femur has a variable clinical course, despite its reported limited tendency to progress.

We investigated the natural history and predisposing factors for progression of dysplasia in a group of 76 patients with a mean follow-up of 8.5 years (2.0 to 15.2). Of these, 31 (41%) presented with an asymptomatic incidental lesion while 45 (59%) presented with pain or a pathological fracture. A group of 23 patients (30%) underwent early operative treatment for pain (19: 25%) or pathological fracture (4: 5%).

Of the 53 patients who were initially treated non-operatively, 45 (85%) remained asymptomatic but eight (15%) needed surgery because of pain or fracture. The progression-free survival of the observation group was 81% (sd 6.4%) at five-years follow-up. An initial presentation of pain (p < 0.001), a limp (p < 0.001), radiological evidence of microfracture (p = 0.001) and younger age (< 17 years) (p = 0.016) were significant predisposing factors for disease progression.

The risk of experiencing pain or pathological fracture is considerable in monostotic fibrous dysplasia of the proximal femur. Patients presenting with pain, a limp or radiological evidence of microfracture have a high chance of needing surgical treatment.

Cite this article: Bone Joint J 2014;96-B:673–6.

The best method of reconstruction after resection of malignant tumours of the tibial diaphysis is unknown. In the absence of any long-term studies analysing the results of intercalary endoprosthetic replacement, we present a retrospective review of 18 patients who underwent limb salvage using a tibial diaphyseal endoprosthetic replacement following excision of a malignant bone tumour. There were ten men and eight women with a mean age of 42.5 years (16 to 76). Mean follow-up was 58.5 months (20 to 141) for all patients and 69.3 months (20 to 141) for the 12 patients still alive. Cumulative patient survival was 59% (95% confidence interval (CI) 32 to 84) at five years. Implant survival was 63% (95% CI 35 to 90) at ten years. Four patients required revision to a proximal tibial replacement at a mean follow-up of 29 months (10 to 54). Complications included metastases in five patients, aseptic loosening in four, peri-prosthetic fracture in two, infection in one and local recurrence in one. The mean Musculoskeletal Tumor Society score and the mean Toronto Extremity Salvage Score were 23 (17 to 28) and 74% (53 to 91), respectively.

Although rates of complication and revision were high, custom-made tibial diaphyseal replacement following resection of malignant bone tumours enables early return to function and provides an attractive alternative to other surgical options, without apparent compromise of patient survival.