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,
Giant cell tumour of bone (GCTB) is a locally aggressive lesion that is difficult to treat as salvaging the joint can be associated with a high rate of local recurrence (LR). We evaluated the risk factors for tumour relapse after treatment of a GCTB of the limbs. A total of 354 consecutive patients with a GCTB underwent joint salvage by curettage and reconstruction with bone graft and/or cement or en bloc resection. Patient, tumour, and treatment factors were analyzed for their impact on LR. Patients treated with denosumab were excluded.Aims
Methods
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. 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.Aims
Methods
The aim of this study was to investigate the safety and efficacy of 3D-printed modular prostheses in patients who underwent joint-sparing limb salvage surgery (JSLSS) for malignant femoral diaphyseal bone tumours. We retrospectively reviewed 17 patients (13 males and four females) with femoral diaphyseal tumours who underwent JSLSS in our hospital.Aims
Methods
The sacroiliac joint (SIJ) is the only mechanical connection between the axial skeleton and lower limbs. Following iliosacral resection, there is debate on whether reconstruction of the joint is necessary. There is a paucity of data comparing the outcomes of patients undergoing reconstruction and those who are not formally reconstructed. A total of 60 patients (25 females, 35 males; mean age 39 years (SD 18)) undergoing iliosacral resection were reviewed. Most resections were performed for primary malignant tumours (n = 54; 90%). The mean follow-up for surviving patients was nine years (2 to 19).Aims
Methods
Treatment of high-grade limb bone sarcoma that invades a joint requires en bloc extra-articular excision. MRI can demonstrate joint invasion but is frequently inconclusive, and its predictive value is unknown. We evaluated the diagnostic accuracy of direct and indirect radiological signs of intra-articular tumour extension and the performance characteristics of MRI findings of intra-articular tumour extension. We performed a retrospective case-control study of patients who underwent extra-articular excision for sarcoma of the knee, hip, or shoulder from 1 June 2000 to 1 November 2020. Radiologists blinded to the pathology results evaluated preoperative MRI for three direct signs of joint invasion (capsular disruption, cortical breach, cartilage invasion) and indirect signs (e.g. joint effusion, synovial thickening). The discriminatory ability of MRI to detect intra-articular tumour extension was determined by receiver operating characteristic analysis.Aims
Methods
Aim. Assess the oncological and clinical outcomes associated with intralesional curettage, phenol and bone grafting of the lesions. Method. A retrospective study was performed using data from the Leiden University Medical Center. Ninety patients with low grade central chondrosarcoma of long bones and small tubular bones were treated using phenol and ethanol as adjuvant therapy after intralesional curettage. Standard clinical follow-up contained regular visits to the orthopaedic department,
Cell-free DNA (cfDNA) and circulating tumour DNA (ctDNA) are used for prognostication and monitoring in patients with carcinomas, but their utility is unclear in sarcomas. The objectives of this pilot study were to explore the prognostic significance of cfDNA and investigate whether tumour-specific alterations can be detected in the circulation of sarcoma patients. Matched tumour and blood were collected from 64 sarcoma patients (n = 70 samples) prior to resection of the primary tumour (n = 57) or disease recurrence (n = 7). DNA was isolated from plasma, quantified, and analyzed for cfDNA. A subset of cases (n = 6) underwent whole exome sequencing to identify tumour-specific alterations used to detect ctDNA using digital droplet polymerase chain reaction (ddPCR).Aims
Methods
Accurate estimations of the risk of fracture due to metastatic bone disease in the femur is essential in order to avoid both under-treatment and over-treatment of patients with an impending pathological fracture. The purpose of the current retrospective in vivo study was to use CT-based finite element analyses (CTFEA) to identify a clear quantitative differentiating factor between patients who are at imminent risk of fracturing their femur and those who are not, and to identify the exact location of maximal weakness where the fracture is most likely to occur. Data were collected on 82 patients with femoral metastatic bone disease, 41 of whom did not undergo prophylactic fixation. A total of 15 had a pathological fracture within six months following the CT scan, and 26 were fracture-free during the five months following the scan. The Mirels score and strain fold ratio (SFR) based on CTFEA was computed for all patients. A SFR value of 1.48 was used as the threshold for a pathological fracture. The sensitivity, specificity, positive, and negative predicted values for Mirels score and SFR predictions were computed for nine patients who fractured and 24 who did not, as well as a comparison of areas under the receiver operating characteristic curves (AUC of the ROC curves).Aims
Methods
The purpose of this retrospective study was to differentiate
between the MRI features of normal post-operative change and those
of residual or recurrent disease after intralesional treatment of
an atypical cartilage tumour (ACT)/grade I chondrosarcoma. We reviewed the case notes, radiology and histology of 75 patients,
who had been treated for an ACT/grade I chondrosarcoma by curettage,
phenolisation and bone allografting between 1994 and 2005. The first
post-operative Gd-enhanced MRI scan was carried out within one year
of surgery. Patients had a minimum of two scans and a mean follow-up
of 72 months (13 to 169). Further surgery was undertaken in cases
of suspected recurrence.Aims
Patients and Methods
The diagnosis of surgical site infection following endoprosthetic reconstruction for bone tumours is frequently a subjective diagnosis. Large clinical trials use blinded Central Adjudication Committees (CACs) to minimise the variability and bias associated with assessing a clinical outcome. The aim of this study was to determine the level of inter-rater and intra-rater agreement in the diagnosis of surgical site infection in the context of a clinical trial. The Prophylactic Antibiotic Regimens in Tumour Surgery (PARITY) trial CAC adjudicated 29 non-PARITY cases of lower extremity endoprosthetic reconstruction. The CAC members classified each case according to the Centers for Disease Control (CDC) criteria for surgical site infection (superficial, deep, or organ space). Combinatorial analysis was used to calculate the smallest CAC panel size required to maximise agreement. A final meeting was held to establish a consensus.Objectives
Materials and Methods
Excision of the proximal femur for tumour with
prosthetic reconstruction using a bipolar femoral head places a considerable
load on the unreplaced acetabulum. We retrospectively reviewed the changes which occur around the
affected hip joint by evaluating the post-operative radiographs
of 65 consecutive patients who underwent proximal prosthetic arthroplasty
of the femur, and in whom an acetabular component had not been used.
There were 37 men and 28 women with a mean age of 57.3 years (17
to 93). Radiological assessment included the extent of degenerative
change in the acetabulum, heterotopic ossification, and protrusio
acetabuli. The mean follow-up was 9.1 years (2 to 11.8). Degenerative changes
in the acetabulum were seen in three patients (4.6%), Brooker grade
1 or 2 heterotopic ossification in 17 (26%) and protrusion of the
prosthetic head in nine (13.8%). A total of eight patients (12.3%) needed a revision. Five were
revised to the same type of prosthesis and three (4.6%) were converted
to a total hip arthroplasty. We conclude that radiological evidence of degenerative change,
heterotopic ossification and protrusion occur in a few patients
who undergo prosthetic arthroplasty of the proximal femur for tumour.
The limited extent of these changes and the lack of associated symptoms
do not justify the routine arthroplasty of the acetabulum in these patients. Cite this article:
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.
Deep-seated lipomas are often atypical histologically and are considered by some to have a high risk of recurrence after excision. We reviewed 215 deep-seated lipomas of the extremities and trunk wall with reference to histology, cytogenetics, clinical features and local recurrence. We classified tumours with atypical features and/or ring chromosomes as atypical lipomas. These were more common in men, larger than ordinary lipomas and more often located in the upper leg. The annual incidence was estimated as ten per million inhabitants and the ratio of atypical to ordinary lipomas was 1:3. In total, six tumours (3%), recurred locally after a median of eight years (1 to 16); of these, four were classified as atypical. The low recurrence rate of deep-seated lipomas of the extremity or trunk wall, irrespective of histological subtype, implies that if surgery is indicated, the tumour may be shelled out, that atypical lipomas in these locations do not deserve the designation well-differentiated liposarcoma, and that routine review after surgery is not required.
We retrospectively reviewed 71 histopathologically-confirmed bone and soft-tissue metastases of unknown origin at presentation. In order to identify the site of the primary tumour all 71 cases were examined with conventional procedures, including CT, serum tumour markers, a plain radiograph, ultrasound examination and endoscopic examinations, and 24 of the 71 cases underwent 2-deoxy-2-[F-18] fluoro-D-glucose positron emission tomography (FDG-PET). This detected multiple bone metastases in nine patients and the primary site in 12 of the 24 cases; conventional studies revealed 16 primary tumours. There was no significant difference in sensitivity between FDG-PET and conventional studies. The mean maximal standardised uptake value of the metastatic tumours was significantly higher than that of the primary tumours, which is likely to explain why FDG-PET did not provide better results. It was not superior to conventional procedures in the search for the primary site of bone and soft-tissue metastases; however, it seemed to be useful in the staging of malignancy.
We undertook a retrospective review of 33 patients who underwent total femoral endoprosthetic replacement as limb salvage following excision of a malignant bone tumour. In 22 patients this was performed as a primary procedure following total femoral resection for malignant disease. Revision to a total femoral replacement was required in 11 patients following failed segmental endoprosthetic or allograft reconstruction. There were 33 patients with primary malignant tumours, and three had metastatic lesions. The mean age of the patients was 31 years (5 to 68). The mean follow-up was 4.2 years (9 months to 16.4 years). At five years the survival of the implants was 100%, with removal as the endpoint and 56% where the endpoint was another surgical intervention. At five years the patient survival was 32%. Complications included dislocation of the hip in six patients (18%), local recurrence in three (9%), peri-prosthetic fracture in two and infection in one. One patient subsequently developed pulmonary metastases. There were no cases of aseptic loosening or amputation. Four patients required a change of bushings. The mean Musculoskeletal Tumour Society functional outcome score was 67%, the mean Harris Hip Score was 70, and the mean Oxford Knee Score was 34. Total femoral endoprosthetic replacement can provide good functional outcome without compromising patient survival, and in selected cases provides an effective alternative to amputation.
Despite local treatment with systemic chemotherapy in Ewing’s sarcoma family tumours (ESFT), patients with detectable metastases at presentation have a markedly worse prognosis than those with apparently localised disease. We investigated the clinical, pathological and laboratory differences in 888 patients with ESFT, 702 with localised disease and 186 with overt metastases at presentation, seen at our institution between 1983 and 2006. Multivariate analyses showed that location in the pelvis, a high level of serum lactic dehydrogenase, the presence of fever and a short interval between the onset of symptoms and diagnosis were indicative of metastatic disease. The rate of overt metastases at presentation was 10% without these four risk factors, 22.7% with one, 31.4% with two, and 50% for those with three or four factors. We concluded that in ESFT the site, the serum level of lactic dehydrogenase, fever, and the interval between the onset of symptoms and diagnosis are indicators of tumours having a particularly aggressive metastatic behaviour.
We present a retrospective study of patients suffering from a variety of benign tumours in whom external fixators were used to treat deformity and limb-length discrepancy, and for the reconstruction of bone defects. A total of 43 limbs in 31 patients (12 male and 19 female) with a mean age of 14 years (2 to 54) were treated. The diagnosis was Ollier’s disease in 12 limbs, fibrous dysplasia in 11, osteochondroma in eight, giant cell tumour in five, osteofibrous dysplasia in five and non-ossifying fibroma in two. The lesions were treated in the tibia in 19 limbs, in the femur in 16, and in the forearm in eight. The Ilizarov frame was used in 25 limbs, the Taylor Spatial Frame in seven, the Orthofix fixator in six, the Monotube in four and the Heidelberg fixator in one. The mean follow-up was 72 months (22 to 221). The mean external fixation period was 168 days (71 to 352). The mean external fixation index was 42 days/cm (22.2 to 102.0) in the 22 patients who required limb lengthening. The mean correction angle for those with angular deformity was 23° (7° to 45°). At final follow-up all patients had returned to normal activities. Four patients required a second operation for recurrent deformity of further limb lengthening. Local recurrence occurred in one patient, requiring further surgery.
The outcome of tibial allograft reconstruction after resection of a tumour is inconsistent and has a high rate of failure. There are few reports on the use of tibial allografts in children with open growth plates. We performed 21 allograft reconstructions (16 osteoarticular, five intercalary) in 19 consecutive patients between seven and 17 years of age. Two had Ewing’s sarcoma, one an adamantinoma and 16 osteosarcoma, one with multifocal disease. Five patients have died; the other 14 were free from disease at the time of follow-up. Six surviving patients (eight allograft reconstructions) continue to have good or excellent function at a mean of 59 months (14 to 132). One patient has poor function at 31 months. The other seven patients have a good or excellent function after additional procedures including exchange of the allograft and resurfacing or revision to an endoprosthesis at a mean of 101 months (43 to 198). The additional operations were performed at a mean of 47 months (20 to 84) after the first reconstruction. With the use of allograft reconstruction in growing children, joints and growth plates may be preserved, at least partially. Although our results remain inconsistent, tibial allograft reconstruction in selected patients may restore complete and durable function of the limb.
