Primary malignant bone tumours are a scarce entity with limited population-based data from developing countries. The aim of the study is to investigate the frequency and anatomical distribution of primary malignant bone tumours in a local South African population. This will be an epidemiological retrospective study. Data will be used of patients that were diagnosed with primary malignant bone tumours over a period of nine years spanning from 1 January 2014 to 31 December 2022. This data will be received from private and government laboratories. Data to be considered are type of primary malignant bone tumours diagnosed, incidence of primary malignant bone tumours over a period of nine years and the most common anatomical sites of primary malignant bone tumours. The rationale behind our study is to assess the frequency of different primary malignant bone tumours in another geographic area of South Africa and to compare these findings to local and international literature. With a projected increase in diagnosis of primary malignant bone tumours in developing countries it is important to have more available data about primary malignant bone tumours from these areas to have a better understanding of these conditions and to understand the impact of the burden they impose on healthcare systems so that management of these conditions can also be improved. Preliminary results show that 23.83% of primary malignant bone tumours occurred in the age group 0–24 years of age, 49.22% in the 25–59 age group and 26.95% in the 60+ age group. The most common tumour that occurred was chondrosarcoma (49.21%) followed by osteosarcoma (41.80%) then Ewing's sarcoma (4,69%) and lastly chordoma (4.30%). From the 256 samples that met the inclusion criteria the five most common anatomical sites were distal femur (63), proximal tibia (41), proximal humerus (38), pelvis (34) and proximal femur (20)

En bloc resection for primary bone tumours and isolated metastasis are complex surgeries associated with a high rate of adverse events (AEs). The primary objective of this study was to explore the relationship between frailty/sarcopenia and major perioperative AEs following en bloc resection for primary bone tumours or isolated metastases of the spine. Secondary objectives were to report the prevalence and distribution of frailty and sarcopenia, and determine the relationship between these factors and length of stay (LOS), unplanned reoperation, and 1-year postoperative mortality in this population. This is a retrospective study of prospectively collected data from a single quaternary care referral center consisting of patients undergoing an elective en bloc resection for a primary bone tumour or an isolated spinal metastasis between January 1st, 2009 and February 28th, 2020. Frailty was calculated with the modified frailty index (mFI) and spine tumour frailty index (STFI). Sarcopenia, determined by the total psoas area (TPA) vertebral body (VB) ratio (TPA/VB), was measured at L3 and L4. Regression analysis produced ORs, IRRs, and HRs that quantified the association between frailty/sarcopenia and major perioperative AEs, LOS, unplanned reoperation and 1-year postoperative mortality. One hundred twelve patients met the inclusion criteria. Using the mFI, five patients (5%) were frail (mFI ³ 0.21), while the STFI identified 21 patients (19%) as frail (STFI ³ 2). The mean CT ratios were 1.45 (SD 0.05) and 1.81 (SD 0.06) at L3 and L4 respectively. Unadjusted analysis demonstrated that sarcopenia and frailty were not significant predictors of major perioperative AEs, LOS or unplanned reoperation. Sarcopenia defined by the CT L3 TPA/VB and CT L4 TPA/VB ratios significantly predicted 1-year mortality (HR of 0.32 per one unit increase, 95% CI 0.11-0.93, p=0.04 vs. HR of 0.28 per one unit increase, 95% CI 0.11-0.69, p=0.01) following unadjusted analysis. Frailty defined by an STFI score ≥ 2 predicted 1-year postoperative mortality (OR of 2.10, 95% CI 1.02-4.30, p=0.04). The mFI was not predictive of any clinical outcome in patients undergoing en bloc resection for primary bone tumours or isolated metastases of the spine. Sarcopenia defined by the CT L3 TPA/VB and L4 TPA/VB and frailty assessed with the STFI predicted 1-year postoperative mortality on univariate analysis but not major perioperative AEs, LOS or reoperation. Further investigation with a larger cohort is needed to identify the optimal measure for assessing frailty and sarcopenia in this spine population

Purpose of the study. To review the primary bone tumours of the spine treated at our unit. Description of methods. Retrospective review of folders and x-rays of all the patients with primary bone tumours of the spine treated at our unit between 2005 and 2012. All haematological tumours were excluded. Summary of results. We treated 15 cases during this period. The median age at presentation was 36 years (8–65). There was a significant delay from onset of symptoms to diagnosis in most cases (median 7 months). Histological diagnoses included:. -Benign tumours.  Active. Hemangioma. 3. Osteoid osteoma. 1. Eosinophilic granuloma. 1.  Aggressive. Osteoblastoma. 1. Giant cell tumours. 2. Aneurysmal bone cysts. 4. -Malignant tumours.  Osteosarcomas. 2.  Leiomyosarcoma of bone. 1. A variety of definitive surgical methods were utilised. Seven patients had a debulking or intralesional resection of the tumour. Eight patients had an attempted marginal excision. This was achieved through anterior surgery only in 1 case, posterior only surgery in 6 cases and combination anterior and posterior surgery in 8 cases. The anterior and posterior surgery was performed in a single sitting in 5 cases and in a staged fashion in 3 cases. Adjuvant radiotherapy and chemotherapy were used where indicated. Three cases presented with significant neurological impairment. Of these 2 made a significant recovery. There were no cases of neurological deterioration following surgery. All 3 patients with malignant tumours died in the follow up period. We had 1 case of hardware failure due to chronic sepsis. Conclusion. Primary bone tumours of the spine are associated with a significant delay in diagnosis. Surgical treatment options and adjuvant therapy should be tailor made for each case depending on the diagnosis. Acceptable results with minimal complications can be achieved with this approach

Osteosarcoma is the most common primary bone tumour worldwide. This disease presents a formidable challenge to the orthopaedic surgeon, with a mortality rate of 30 per cent, even after surgical clearance. Aberrant Wnt signalling has been implicated in the pathogenesis osteoblastic tumours. The objective of this study is 2 fold- to investigate if osteosarcoma does indeed demonstrate aberrant Wnt signaling, and if so, does osteosarcoma respond to a novel Wnt inhibitor(ETC159). This can potentially lead to the development of a new adjuvant treatment modality for osteosarcoma. A novel Wnt signaling pathway protein antibody (YJ5) was used in immunihistochemistry staining of clinical osteosarcoma samples. A Wnt high osteosarcoma cell line(SJSA-1) was then implanted subcutaneously in a mouse model. These mice were treated with a novel PORCN inhibitor, ETC 159 for a period of 4 weeks in a two-arm randomised control study. The results of treatment were evaulated by clinical outcome parameters as well as immunohstochemistry. 100 per cent of clinical osteosarcoma samples demonstrated increased WLS expression and Wnt protein expression. SJSA-1 showed no significant decrease in tumour volume after 30 days of drug treatment (3070 SD 625 mm3 vs 3480 SD 433 mm3 p= 0.605 and 2060 SD 209 vs 1677 SD 213 mm3 p=0.219 respectively). Significantly, SJSA-1 demonstrated increased tumour necrosis in the treatment arm(30–60 percent increase across all samples p < 0 .005) Treated tumours also demonstrated markedly less angiogenesis compared to the non treatment arm. Osteosarcoma demonstrates aberrant Wnt signaling in a large percentage of cases. The use of a novel PORCN inhibitor ETC 159 for the treatment of Osteosarcoma has a marked effect on tumour necrosis. Our results suggest that ETC159 may cause tumour necrosis by inhibiting angiogenesis within the tumour. Further evaluation and understanding of the mechanism of Wnt singaling in regulating tumour pathogenesis may hold the potential for developing a curative therapeutic drug for this deadly disease

The distal humerus represents 1% of all primary bone tumours. Endoprosthetic replacement can potentially improve function and provide good pain relief. We present out experience with the custom made Stanmore elbow endoprosthesis used after resection of malignant tumours of the distal humerus. Between 1970–2009 we carried out 19 endoprosthetic replacments for malignant tumours of the distal humerus. 10 were a result of metastasis and 9 were primary bone tumours. 7 patients had a pathological fracture as their first presentation and 3 had pathological fractures after the diagnosis was made. 11 patients died between 3 months to 16 year following surgery. The mean survival of the patient group was 7.1 years (range 3 months to 37 years). 4 patients underwent a revision EPR and one patient underwent two revision EPR's due to loosening. Two patients underwent maintenance procedures (rebushing) due to wear of the poly bushing. We have had no revisions since the design of hte implant was changed to a floppy hinge design. One patient underwent an above elbow amputation four years after surgery due to local recurrence. There were no early post operative infections. One patient developed a sinus requiring multiple wound explorations, one year after insertion of the endoprosthesis for a sarcoma. This patient was infection free till the time of death 3 years later. There were no nerve palsies, periprosthetic fractures or wound problems. The mean TES score was 72% (59–78%) in the surviving patients at review. As the majority of the patients were implanted for metastatic disease the initial reliablity and low complication rate of the procedure, in our series, confirms that this is a suitable reconstruction for patients in significant metastatic pain from a destructive lesion of the distal humerus, rapidly restoring function and relieving pain in a predictable manner

Introduction. Aseptic loosening is the most common mode of failure of massive endoprostheses. Introduction of Hydroxyapatite coated collars have reduced the incidence of aseptic loosening. However bone growth is not always seen on these collars. Objectives. The aims of our study were to determine the extent of osseous integration of Hydroxyapatite coated collars, attempt a grading system for bone growth and to determine the effect of diagnosis, surgical technique and adjuvant therapy on bone growth. Methods. We reviewed the records and radiographs of 58 patients who had a massive endoprosthesis implanted by two surgeons in our unit over the last five years. Revision surgeries were recorded separately. Bone growth was graded 1–4 based on appearance in antero-posterior and lateral radiographs. Results. Three groups were identified. Group 1-Resections for primary bone tumours (33 patients), Group 2-resections for metastatic bone disease (22 patients) and Group 3- Resections for non tumour indications (3 patients). Overall, 60% of patients had grade 1, 12% had grade 2, 19% had grade 3 and 9% had grade 4 osteointegration. Grade 3 or 4 Collar osteointegration was found in 37% of patients in Group 1, 9% in group 2 and 67% in group 3. 5% of patients with grade 1 integration, 100% patients with grade 2 integration and none of the patients with grade 3 or 4 integration underwent revision for aseptic loosening. Appearance or widening of a gap between the resected bone end and the collar indicated loosening and impending revision. Proximal humeral replacements had the lowest rate of osteointegration (12%). Adjuvant therapy did not affect osteointegration. Conclusion. Osteointegration of collars is seen more often after resection of primary bone tumours. The role of collars in metastatic tumour surgery is questionable. Our radiographic grading system of bone growth predicted aseptic loosening

Background. Endoprosthetic reconstruction is an established method of treatment for primary bone tumours in children. Traditionally these were implanted with cemented intramedullary fixation. Hydroxyapatite collars at the shoulder of the implant are now standard on all extremity endoprostheses, but older cases were implanted without collars. Uncemented intramedullary fixation with hydroxyapatite collars has also been used in an attempt to reduce the incidence of problems such as aseptic loosening. Currently there are various indications that dictate which method is used. Aims. To establish long term survivorship of cemented versus uncemented endoprosthesis in paediatric patients with primary bone tumours. Methods. This was a retrospective study of 441 endoprostheses implanted in 367 consecutive patients aged 18 years or less, between 1973 and 2005. This included the use of case notes, hospital databases and a radiological review. Information obtained included patient demographics, indications for surgery, anatomical distribution and type of implants, complications and survivorship. Results. Mean age was 13.9 (range 3 - 38). 210 patients were male, 157 were female. There were 364 primaries and 77 revision implants. 161 extendable and 280 definitive prostheses. 282 patients had osteosarcoma, 54 had Ewing's sarcoma and 28 had other diagnoses. Commonest sites included 197 distal femoral replacements, 85 proximal tibial implants and 57 were in the upper limb. Kaplan-Meier survival analysis was used to compare anatomical sites and method of fixation. Upper limb implants had the best long term survival. Failure rates for distal femoral replacements were compared for cemented fixation (21.7% due to aseptic loosening) with cement plus hydroxyapatite collars (3.1%) and uncemented implants with hydroxyapatite collars (6.2%). Conclusions. In the distal femur cemented fixation with hydroxyapatite collars gave the best survivorship in definitive primary prostheses. Uncemented fixation with hydroxyapatite collars gave the best survivorship in extendable prostheses. Cemented fixation without hydroxyapatite gave the worst survivorship

This study investigates the experience of one treatment centre with routine surveillance MRI following excision of sarcoma. Casenotes, MRI and histology reports for fifty-nine patients were reviewed. The primary outcome was the presence of local tumour recurrence and whether this was identified on surveillance or interval scanning. Forty-eight patients had a diagnosis of soft tissue sarcoma, the remaining 11 a primary bone tumour. Fifteen patients had local recurrence (25%). Eight were identified on surveillance scan, and the remaining 7 required interval scans. Surveillance scanning has a role in the early detection of local recurrence of bone and soft tissue sarcoma

Skeletal Cryptococcosis although rare has been reported in immunodeficient individuals and in particular those with HIV. We present a case in a HIV- negative patient who presented to the London Sarcoma service masquerading as a primary bone tumour and review the relevant literature. A 71 year old lady presented with a three month history of right submammary pain associated with a new lump. Chest radiographs showed an osteolytic lesion in the right 6. th. rib. CT scans demonstrated mediastinal lymphadenopathy and numerous lung nodules. Differential diagnosis of the lesion included TB abscess, myeloma, lymphoma or as a primary lung tumour presenting with hilar lymphadenopathy and necrotic skeletal metastasis. CT guided biopsy was performed with histology showing necrotising granulomatous inflammation with numerous yeast like organisms in keeping with Cryptococcus fungal infection. She was treated successfully with a six week course of voriconazole. Cryptococcal skeletal infections can cause significant morbidity and mortality and should be considered as a rare cause of lytic osseous lesions

Aims and methods. We present a review of our use of the Ilizarov apparatus in a non-acute NGO hospital in Cambodia specialising in limb reconstruction. Frames are applied without on table image intensification. A retrospective case-note analysis of Ilizarov apparatus use for all indications was conducted. 53 frames were applied between November 2005 and October 2011. Indications for application were chronic open fracture, osteomyelitis, fracture malunion, infective and non-infective non-union, bone lengthening, primary bone tumour resection, ankle fusion, congenital deformity or pseudarthrosis, chronic hip dislocation, or a combination of the above. Results. Median delay in presentation was 104 weeks for all indications (range 4–864). Median treatment length was 21 weeks (3–76). The most frequent complication was pin-site infection. This occurred in 18 patients (34%). Return to theatre occurred in 21 patients (40%). Indications were frame adjustment, pin addition or removal, addition of bone graft or re-osteotomy. Failure of union occurred in three patients. These rates are comparable with those published in both Asian and Western literature. Conclusions. Our data demonstrate the versatility of the Ilizarov apparatus and its importance in limb reconstruction in a developing world setting. Our centre relies on it as a cost-effective tool for traditional and novel indications. In our centre the apparatus is applied without x-ray control and is maintained without a dedicated outreach pin-site care programme. Despite this our complication rates are comparable with western literature. We therefore recommend it as a safe and cost-effective tool for use in other developing world settings

Background. Advances in diagnosis and treatment should mean that hindquarter amputation is now rarely needed. Unfortunately this is not the case. We have performed 166 of these amputations in the past 36 years. We have investigated the reasons why this procedure is still required and the outcomes following it. Method. A retrospective review of data stored on a prospective database. Results. Hindquarter amputation was used as treatment for 15% of all primary bone tumours affecting the pelvis. 146 were performed with curative intent but 20 were performed purely for palliation, usually to relieve pain. 96 of the procedures were needed as part of primary treatment, with the other 70 being needed following failure of local control after other surgical procedures. The indication for amputation in primary disease was almost always due to a significant delay in diagnosis, allowing tumours (particularly chondrosarcomas) to become massive by the time of diagnosis. The peri-operative mortality was 3% and 45% had major wound healing problems or infection. The median survival times after curative and palliative procedures were 36 months and 8 months respectively. The survival after hindquarter amputation for curative intent at 1, 3 and 5 years was 74%, 60% and 48%. Overall survival was better with chondrosarcoma – 52% of the patients surviving more than 10 years had chondrosarcoma. Phantom pain was a significant problem; fewer than 10% use their prosthesis regularly. Despite this functional scores averaged 61% – not significantly worse than patients who had undergone pelvic replacements!. Conclusion. Hindquarter amputation is still regularly required both for primary and salvage treatment in musculoskeletal oncology. Earlier diagnosis of pelvic tumours may avoid its use. Survival is not surprisingly worse than for tumours at other sites

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

Aims

The aticularis genu (AG) is the least substantial and deepest muscle of the anterior compartment of the thigh and of uncertain significance. The aim of the study was to describe the anatomy of AG in cadaveric specimens, to characterize the relevance of AG in pathological distal femur specimens, and to correlate the anatomy and pathology with preoperative magnetic resonance imaging (MRI) of AG.

Primary malignant bone tumours frequently arise in children close to the knee, hip or shoulder. Resection of the tumour will often require excision of the epiphysis and frequently one side of the involved joint. In these children an extendable endoprosthesis is usually required to allow for maintenance of limb length equality. We have used 180 extendable endoprostheses in 176 children since 1975. The indication for use of an extendable prosthesis was if there was more than 30mm of growth remaining in the resected bone. The age of the patients ranged from 2 to 15 and 99 were boys. The sites of the endoprostheses used were: distal femur in 91, proximal tibia in 42, proximal femur in 11, total femur in 6 and proximal or total humerus in 26. 131 of the operations were for osteosarcoma and 34 for Ewing's. Five types of lengthening mechanism have been used. Two designs used a worm screw gear, one type used a C collar, one type a ball bearing mechanism and the latest uses a non invasive lengthening system whereby a motor inside the prosthesis is activated by an electromagnetic field. Of the 176 patients, 59 have died and of the remainder, 89 have reached skeletal maturity. 19 patients had an amputation, 11 due to local recurrence and 8 due to infection. The risk of infection was 19% in surviving patients. Most of the skeletally mature had equal leg lengths. The average number of operations was 11 but ranged between 2 and 29. Most operations were for lengthening but younger children always needed revisions of the prosthesis. Functional scores were 77%. Extendable endoprostheses are demanding both for the patient and the surgeon. The high complication rate should be decreased by non invasive lengthening prostheses