J. D. Stevenson

The role of grade in local recurrence and the disease-specific survival in chondrosarcomas

Aims The purpose of this study was to describe the effect of histological grade on disease‐specific survival in patients with chondrosarcoma. Patients and Methods A total of 343 patients with a chondrosarcoma were included. The histological grade was assessed on the initial biopsy and on the resection specimen. Where the histology showed a mixed grade, the highest grade was taken as the definitive grade. When only small focal areas showed higher grade, the final grade was considered as both. Results The concordance between the highest preoperative biopsy grading and the highest final grading of the resection specimen in total was only 43% (146/343). In 102 specimens (30%), a small number of cells or focal areas of higher grade were observed in contrast to the main histology. The disease‐specific survival, stratified according to the predominant histological grade, showed greater variation than when stratified according to the highest grade seen in the resection specimen. Conclusion The diagnostic biopsy in chondrosarcoma is unreliable in assessing the definitive grade and the malignant potential of the tumour. When categorizing the grade of the resection specimen, the prognosis for local recurrence and disease‐specific survival should be based on the highest grade seen, even when seen in only a few cells. Cite this article: Bone Joint J 2018;100‐B:662–6.

Surgical Innovation in Sarcoma Surgery

The field of orthopaedic oncology relies on innovative techniques to resect and reconstruct a bone or soft tissue tumour. This article reviews some of the most recent and important innovations in the field, including biological and implant reconstructions, together with computer-assisted surgery. It also looks at innovations in other fields of oncology to assess the impact and change that has been required by surgeons; topics including surgical margins, preoperative radiotherapy and future advances are discussed.

Outcome of surgery for primary and recurrent desmoid-type fibromatosis. A retrospective case series of 174 patients

Background The best management of relapsing desmoid-type fibromatosis, a benign but locally infiltrative soft-tissue tumour, is largely undecided. Our aim was to investigate the incidence and the factors influencing local relapse after surgery for primary and recurrent disease of the trunk and extremities. Patients and Methods Retrospective analysis of 174 patients who had surgical treatment for desmoid-type fibromatosis. The quality of the surgical margins and use of adjuvant radiotherapy or chemotherapy were analysed regarding local recurrences in primary and recurrent disease. Results Clear margins were achieved in 41% of cases. 10-year local control rate was 58% for clear primary resections as compared to 37% with intralesional primary resections (p = 0.030). Extremity tumours had a higher risk of local recurrence compared to trunk and pelvic ones (p < 0.001). Attempted resection of recurrent disease was associated with an approximately 90% incidence of relapse after each procedure, despite the quality of the surgical margins being equivalent to primary resections. Quality of surgical margins was not important for local control of recurrent lesions. Adjuvant treatments (radiotherapy and chemotherapy) had a no significant effect on the local control rate of recurrent disease (odds ratio 0.693 and 0.969 respectively). Conclusions A complete primary excision is the best window of opportunity to achieve local control of desmoid-type fibromatosis. Once the disease relapses, surgical intervention is accompanied with a high risk of failure, irrespective of the quality of the margins and adjuvant treatment given.

Functional and quality of life outcomes in bone sarcoma following amputation, rotationplasty or limb-salvage

ABSTRACT Introduction: Bone sarcomas are rare primary mesenchymal tumours affecting children and adults. Since the advent of modern chemotherapeutic and reconstructive techniques, limb salvage has replaced amputation to become the standard of care for bone sarcomas. Surgical principles mandate achieving survival outcomes and maximising limb function. Whilst oncological and functional patient outcomes after limb-salvage surgery are frequently reported in scientific literature, the health related quality of life outcomes are frequently overlooked. Areas covered: Studies examining functional outcomes have generally reported better function after limb-salvage, but this may not necessarily translate into improved quality of life. This article examines functional and quality of life outcomes following amputation, limb-salvage and rotationplasty for bone sarcoma in the lower limbs. Expert commentary: Physical function and everyday competence to perform activities of daily living are predictive of quality of life, not the type of surgical procedure. This highlights the importance of preserving limb function to maximise quality of life following all types of surgery.

Multiple Soft Tissue Sarcomas in a Single Patient: An International Multicentre Review

Developing multiple soft tissue sarcomas (STSs) is a rare process, sparsely reported in the literature to date. Little is known about the pattern of disease development or outcomes in these patients. Patients were identified from three tertiary orthopaedic oncology centres in Canada and the UK. Patients who developed multiple extremity STSs were collated retrospectively from prospective oncology databases. A literature review using MEDLINE was also performed. Six patients were identified in the case series from these three institutions, and five studies were identified from the literature review. Overall, 17 patients were identified with a median age of 51 years (range: 19 to 77). The prevalence of this manifestation in STS patients is 1 in 1225. The median disease-free interval between diagnoses was 2.3 years (range: 0 to 19 years). Most patients developed the secondary STS in a metachronous pattern, the remaining, synchronously. The median survival after the first sarcoma was 6 years, and it was 1.6 years after the second sarcoma. The 5-year overall survival rate was 83.3% and 50% following the first and second STS diagnoses, respectively. A diagnosis of two STSs does not confer a worse prognosis than the diagnosis of a single STS. Developing a second STS is a rare event with no identifiable histological pattern of occurrence. Presentation in a metachronous pattern is more common. A high degree of vigilance is required in patients with a previous STS both to detect both local recurrence and to identify new masses remote from the previous STS site. Acquiring an early histological diagnosis should be attempted.

The use of a non-invasive extendable prosthesis at the time of revision arthroplasty

Aims The use of a noninvasive growing endoprosthesis in the management of primary bone tumours in children is well established. However, the efficacy of such a prosthesis in those requiring a revision procedure has yet to be established. The aim of this series was to present our results using extendable prostheses for the revision of previous endoprostheses. Patients and Methods All patients who had a noninvasive growing endoprosthesis inserted at the time of a revision procedure were identified from our database. A total of 21 patients (seven female patients, 14 male) with a mean age of 20.4 years (10 to 41) at the time of revision were included. The indications for revision were mechanical failure, trauma or infection with a residual leg‐length discrepancy. The mean follow‐up was 70 months (17 to 128). The mean shortening prior to revision was 44 mm (10 to 100). Lengthening was performed in all but one patient with a mean lengthening of 51 mm (5 to 140). Results The mean residual leg length discrepancy at final follow‐up of 15 mm (1 to 35). Two patients developed a deep periprosthetic infection, of whom one required amputation to eradicate the infection; the other required two‐stage revision. Implant survival according to Henderson criteria was 86% at two years and 72% at five years. When considering revision for any cause (including revision of the growing prosthesis to a non‐growing prosthesis), revision‐free implant survival was 75% at two years, but reduced to 55% at five years. Conclusion Our experience indicates that revision surgery using a noninvasive growing endoprosthesis is a successful option for improving leg length discrepancy and should be considered in patients with significant leg‐length discrepancy requiring a revision procedure.

Vascularized fibular epiphyseal transfer for proximal humeral reconstruction in children with a primary sarcoma of bone

Aims Preserving growth following limb‐salvage surgery of the upper limb in children remains a challenge. Vascularized autografts may provide rapid biological incorporation with the potential for growth and longevity. In this study, we aimed to describe the outcomes following proximal humeral reconstruction with a vascularized fibular epiphyseal transfer in children with a primary sarcoma of bone. We also aimed to quantify the hypertrophy of the graft and the annual growth, and to determine the functional outcomes of the neoglenofibular joint. Patients and Methods We retrospectively analyzed 11 patients who underwent this procedure for a primary bone tumour of the proximal humerus between 2004 and 2015. Six had Ewings sarcoma and five had osteosarcoma. Their mean age at the time of surgery was five years (two to eight). The mean follow‐up was 5.2 years (1 to 12.2). Results The overall survival at five and ten years was 91% (confidence interval (CI) 95% 75% to 100%). At the time of the final review, ten patients were alive. One with local recurrence and metastasis died one‐year post‐operatively. Complications included seven fractures, four transient nerve palsies, and two patients developed avascular necrosis of the graft. All the fractures presented within the first postoperative year and united with conservative management. One patient had two further operations for a slipped fibular epiphysis of the autograft, and a hemi‐epiphysiodesis for lateral tibial physeal arrest. Hypertrophy and axial growth were evident in nine patients who did not have avascular necrosis of the graft. The mean hypertrophy index was 65% (55% to 82%), and the mean growth was 4.6 mm per annum (2.4 to 7.6) in these nine grafts. At final follow‐up, the mean modified functional Musculoskeletal Tumour Society score was 77% (63% to 83%) and the mean Toronto Extremity Salvage Score (TESS) was 84% (65% to 94%). Conclusion Vascularized fibular epiphyseal transfer preserves function and growth in young children following excision of the proximal humerus for a malignant bone tumour. Function compares favourably to other limb‐salvage procedures in children. Longer term analysis is required to determine if this technique proves to be durable into adulthood.

The role of surgical margins in chondrosarcoma

INTRODUCTION Chondrosarcoma (CS) is the second most common primary bone sarcoma with no clear role for adjuvant therapy. The purpose of this study was to investigate (1) the relationship between surgical excision margins and local recurrence free survival (LRFS), and (2) the role of local recurrence (LR) in disease specific survival (DSS) in CS of the extremity and pelvis. MATERIAL AND METHODS 341 pelvic and extremity CS diagnosed between 2003 and 2015 were studied retrospectively. RESULTS LR developed in 23% of cases. Pelvic location, pathologic fracture, margin and grade were significant factors for LR after univariate analysis. Multivariate analysis revealed surgical margin and pelvic location as positive factors for LR, and grade-1 and 2 CS as negative factors for LR. Pathologic fracture, central versus peripheral, grade, and LR were significant factors with univariate analysis for DSS; and grade was significant after multivariate analysis for all patients for DSS. After competing risk analysis, LR was statistically significant for DSS in grade-2 and grade-3 tumors. CONCLUSION Surgical margins determine LR in all CS grades, but LR affects DSS only in grade-2 and grade-3 tumors. Although narrow margins are acceptable in grade-1 tumors, since biopsy is unreliable in predicting final grade, a minimum 4-mm margin should be the aim in all cases.

Impact of specialist management on survival from radiation‐associated angiosarcoma of the breast

Radiation‐associated angiosarcoma of the breast (RAAS) is a rare complication of adjuvant radiotherapy associated with poor survival. The British Sarcoma Group guidelines recommend that all angiosarcomas are referred to a sarcoma multidisciplinary team, although there is no recommendation that patients are managed within a sarcoma service. The aims of this study were to compare survival, complete excision rates and local recurrence rates of patients managed within a sarcoma service and those managed within local hospitals.

Minimising aseptic loosening in extreme bone resections: custom-made tumour endoprostheses with short medullary stems and extra-cortical plates

Aims Following the resection of an extensive amount of bone in the treatment of a tumour, the residual segment may be insufficient to accept a standard length intramedullary cemented stem. Short‐stemmed endoprostheses conceivably have an increased risk of aseptic loosening. Extra‐cortical plates have been added to minimise this risk by supplementing fixation. The aim of this study was to investigate the survivorship of short‐stemmed endoprostheses and extra‐cortical plates. Patients and Methods The study involved 37 patients who underwent limb salvage surgery for a primary neoplasm of bone between 1998 and 2013. Endoprosthetic replacement involved the proximal humerus in nine, the proximal femur in nine, the distal femur in 13 and the proximal tibia in six patients. There were 12 primary (32%) and 25 revision procedures (68%). Implant survivorship was compared with matched controls. The amount of bone that was resected was > 70% of its length and statistically greater than the standard control group at each anatomical site. Results The mean follow‐up was seven years (one to 17). The mean length of the stem was 33 mm (20 to 60) in the humerus and 79 mm (34 to 100) in the lower limb. Kaplan‐Meier analysis of survival of the implant according to anatomical site confirmed that there was no statistically significant difference between the short‐stemmed endoprostheses and the standard stemmed controls at the proximal humeral (p = 0.84), proximal femoral (p = 0.57), distal femoral (p = 0.21) and proximal tibial (p = 0.61) sites. In the short‐stemmed group, no implants with extra‐cortical plate osseointegration suffered loosening at a mean of 8.5 years (range 2 to 16 years). Three of ten (30%) without osseointegration suffered aseptic loosening at a mean of 7.7 years (range 2 to 11.5 years). Conclusion When extensive resections of bone are required in the surgical management of tumours, and in revision cases, the addition of extra‐cortical plates to short medullary stems has shown non‐inferiority to standard length medullary stems and minimises aseptic failure. Cite this article: Bone Joint J 2017;99‐B:1689‐95.