Breast Angiosarcoma Surveillance Study: UK national audit of management and outcomes of angiosarcoma of the breast and chest wall.

BACKGROUND: Breast angiosarcomas are rare tumours of vascular origin. Secondary angiosarcoma occurs following radiotherapy for breast cancer. Angiosarcomas have high recurrence and poor survival rates. This is concerning owing to the increasing use of adjuvant radiotherapy for the treatment of invasive breast cancer and ductal cancer in situ (DCIS), which could explain the rising incidence of angiosarcoma. Outcome data are limited and provide a poor evidence base for treatment. This paper presents a national, trainee-led, retrospective, multicentre study of a large angiosarcoma cohort. METHODS: Data for patients with a diagnosis of breast/chest wall angiosarcoma between 2000 and 2015 were collected retrospectively from 15 centres. RESULTS: The cohort included 183 patients with 34 primary and 149 secondary angiosarcomas. Median latency from breast cancer to secondary angiosarcoma was 6 years. Only 78.9 per cent of patients were discussed at a sarcoma multidisciplinary team meeting. Rates of recurrence were high with 14 of 28 (50 per cent ) recurrences in patients with primary and 80 of 124 (64.5 per cent ) in those with secondary angiosarcoma at 5 years. Many patients had multiple recurrences: total of 94 recurrences in 162 patients (58.0 per cent). Median survival was 5 (range 0-16) years for patients with primary and 5 (0-15) years for those with secondary angiosarcoma. Development of secondary angiosarcoma had a negative impact on predicted breast cancer survival, with a median 10-year PREDICT prognostic rate of 69.6 per cent, compared with 54.0 per cent in the observed cohort. CONCLUSION: A detrimental impact of secondary angiosarcoma on breast cancer survival has been demonstrated. Although not statistically significant, almost all excess deaths were attributable to angiosarcoma. The increased use of adjuvant radiotherapy to treat low-risk breast cancer and DCIS is a cause for concern and warrants further study.

Blood-borne biomarkers of osteosarcoma: A systematic review.

Osteosarcoma is the most common type of primary malignant bone tumor in children and young adults. Development of clinically useful biomarkers has the potential to improve treatments. The aim of this review was to investigate the recent literature assessing the utility of biomarkers for osteosarcoma. A detailed literature search was performed, with hand searches for related research publications. The search was limited to publications in English between January 2007 and February 2017. Of 286 studies identified, 24 met the inclusion criteria. There is a wide range of osteosarcoma biomarkers identified which act as clinical prognostic factors in patient outcome.

Indeterminate nodules in osteosarcoma: what's the follow-up?

BACKGROUND: Indeterminate pulmonary nodules in patients diagnosed with osteosarcoma present a challenge for accurate staging and prognosis. The aim of this study was to explore the significance of this finding. METHODS: A retrospective cohort study of 120 patients with osteosarcoma was performed in the North East of England. Chest computed tomographies (CTs) at presentation were reviewed and the incidence of 'indeterminate' nodules recorded. Follow-up scans were reviewed and survival as well as prognostic features were analysed. RESULTS: 25% of our cohort presented with indeterminate nodules. Of these, 33% were subsequently confirmed as metastases, the majority within a year. Kaplan-Meier survival analysis showed that patients with indeterminate nodules fared better than those with frank metastatic disease, and similar to those who presented with a normal chest CT. We found no radiographic features that predicted survival. CONCLUSIONS: Indeterminate nodules remain a clinical and diagnostic dilemma. Close monitoring of patients is advised during the first year from presentation, and there is potential for indeterminate nodules to develop into frank metastases later than five years from presentation.

Patient age of less than 55 years is not an independent predictor of functional improvement or satisfaction after total knee arthroplasty.

INTRODUCTION: Management of the young patient with end-stage osteoarthritis of the knee is difficult, with surgical options of osteotomy, partial or total knee arthroplasty (TKA). The primary aim of this study was to assess whether age of less than 55 years was an independent predictor of functional outcome and satisfaction after total knee arthroplasty (TKA). The secondary aims were to identify pre-operative differences in patient demographics, comorbidity and function between patients less than 55 years old compared to those 55 years old and over. MATERIALS AND METHODS: A retrospective cohort consisting of 2589 patients undergoing a primary TKA was identified from an established arthroplasty database. Patient demographics, comorbidity, Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) and Short Form (SF) 12 scores were collected pre-operatively and 1 year post-operatively. In addition, patient satisfaction was assessed at 1 year. Regression analysis was used to identify independent pre-operative predictors of change in the WOMAC and SF-12 scores, and patient satisfaction. RESULTS: Patients less than 55 years old were significantly less likely to be satisfied with the overall outcome of their TKA (OR 0.4, p = 0.001). After adjusting for confounding variables age group was not an independent predictor of overall satisfaction with overall outcome (OR 0.71, p = 0.16). Independent predictors of an increased risk of dissatisfaction with the overall outcome at 1 year were depression (OR 0.58, p = 0.008) and worse pre-operative SF-12 MCS (p = 0.04). CONCLUSION: Age of less than 55 years is not an independent predictor of functional outcome or rate of patient satisfaction after TKA. However, depression and poor mental health are significantly more prevalent in patients less than 55 years old and were independently associated with a lower satisfaction rate.

Advances in image enhancement for sarcoma surgery.

The recurrence rate of soft tissue and bone sarcomas strongly correlates to the status of the surgical margin after excision, yet excessive removal of tissue may lead to distinct, otherwise avoidable morbidity. Therefore, adequate margination of sarcomas both pre- and intra-operatively is a clinical necessity that has not yet fully been met. Current guidance for soft-tissue sarcomas recommends an ultrasound scan followed by magnetic resonance imaging (MRI). For bone sarcomas, two plane radiographs are required, followed similarly by an MRI scan. The introduction of more precise imaging modalities may reduce the morbidity associated with sarcoma surgery; the PET-CT and PET-MRI approaches in particular demonstrating high clinical efficacy. Despite advancements in the accuracy in pre-operative imaging, translation of an image to surgical margins is difficult, regularly resulting in wider resection margins than required. For soft tissue sarcomas there is currently no standard technique for image guided resections, while for bone sarcomas fluoroscopy may be used, however margins are not easily discernible during the surgical procedure. Near infra-red (NIR) fluorescence guided surgery offers an intra-operative modality through which complete tumour resection with adequate tumour-free margins may be achieved, while simultaneously minimising surgical morbidity. NIR imaging presents a potentially valuable adjunct to sarcoma surgery. Early reports indicate that it may be able to provide the surgeon with helpful information on anatomy, perfusion, lymphatic drainage, tumour margins and metastases. The use of NIR fluorochromes have also been demonstrated to be well tolerated by patients. However, prior to widespread implementation, studies related to cost-effectiveness and the development of protocols are essential. Nevertheless, NIR imaging may become ubiquitous in the future, carrying the potential to transform the surgical management of sarcoma.

Role of routine blood tests for predicting clinical outcomes in osteosarcoma patients.

BACKGROUND: This retrospective cohort study aimed to investigate whether simple routine blood tests at presentation (C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), alkaline phosphatase and albumin) predict survival in patients with osteosarcoma. METHODS: Between January 1998 and February 2015, 134 patients with a histological diagnosis of osteosarcoma were treated in our unit. Of these, 79 patients with high-grade osteosarcomas were included in the study. Demographic and clinical data, and laboratory parameters obtained prior to biopsy (CRP, ESR, alkaline phosphatase and albumin levels), were obtained from patients' records. RESULTS: There were 44 males and 35 females. Univariate analysis showed that high pre-biopsy CRP ( p = 0.004), raised pre-biopsy ESR ( p = 0.010), older age ( p < 0.001), poor tumour necrosis rates (≤90%, p = 0.023) and metastasis at presentation ( p < 0.001) were poor prognostic factors. Multivariate analysis showed pre-biopsy CRP and ESR levels to be independent predictors of overall survival ( p = 0.020 and p = 0.025, respectively). Kaplan-Meier survival was significantly lower in patients with elevated CRP ( p = 0.002) and ESR ( p = 0.003). Hypoalbuminaemia and elevated alkaline phosphatase levels did not correlate with overall survival. CONCLUSION: Preoperative CRP and ESR levels may have value in building a prognostic model for patients presenting with osteosarcoma.

Meaningful changes in the Short Form 12 physical and mental summary scores after total knee arthroplasty.

BACKGROUND: The aim of this study was to identify minimal clinically important difference (MCID), minimal important change (MIC) and minimal detectable change (MDC) for the Short Form (SF-) 12 physical and mental component summary (PCS, MCS) scores after total knee arthroplasty (TKA) using an anchor based methodology. METHODS: During a 10-year period, 2589 TKA were performed. SF-12 PCS and MCS scores were recorded preoperatively and at one year postoperatively. At one year, patients were asked "How much did the knee replacement surgery improve the quality of your life?" Their response was recorded as: a great improvement, moderate improvement, little improvement, no improvement at all, or the quality of my life is worse. Patients recording a little (n = 211) and no (n = 115) were used to calculate the MCID and the MIC. The MDC90 was calculated using distribution based methods for the whole cohort. RESULTS: The MCID was 1.8 (p = 0.04) for the PCS and 1.5 (p = 0.33) for the MCS score. The MIC was 2.7 (p = 0.04) for the PCS and -1.4 (p = 0.17) for the MCS score. The MDC90 was 8.9 for the PCS and 13.8 for the MCS score. CONCLUSION: The MCID for the PCS can be used to compare the outcomes between groups, and the MIC can be used to ensure that a clinical difference has been observed for a cohort of patients. The values for the MDC90 can be used to assess whether or not an individual patient has experienced a change.

Three groups of dissatisfied patients exist after total knee arthroplasty: early, persistent, and late.

AIMS: The primary aim of this study was to assess whether patient satisfaction one year after total knee arthroplasty (TKA) changed with longer follow-up. The secondary aims were to identify predictors of satisfaction at one year, persistence of patient dissatisfaction, and late onset dissatisfaction in patients that were originally satisfied at one year. PATIENTS AND METHODS: A retrospective cohort consisting of 1369 patients undergoing a primary TKA for osteoarthritis that had not undergone revision were identified from an established arthroplasty database. Patient demographics, comorbidities, Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) scores, and Short Form 12 (SF-12) questionnaire scores were collected preoperatively, and one and five years postoperatively. In addition, patient satisfaction was assessed at one and five years postoperatively. Logistic regression analysis was used to identify independent predictors of satisfaction at one and five years. RESULTS: The overall rate of satisfaction did not change from one (91.7%, n = 1255) to five (90.1%, n = 1234) years (p = 0.16). Approximately half (n = 53/114) of the patients who were dissatisfied at one year became satisfied with their TKA at five years, whereas 6% (n = 74/1255) of those who were satisfied at one year became dissatisfied at five years. At one year, patients with lung disease (p = 0.04), with depression (p = 0.001), with back pain (p < 0.001), undergoing unilateral TKA (p = 0.001), or with a worse preoperative WOMAC pain score (p = 0.04) were more likely to be dissatisfied. Patients with gastric ulceration (p = 0.04) and a worse WOMAC stiffness score (p = 0.047) were at increased risk of persistent dissatisfaction at five years. In contrast, a worse WOMAC pain score (p = 0.01) at one year was a predictor of dissatisfaction in previously satisfied patients at five years. CONCLUSION: Three groups of dissatisfied patients exist after TKA: 'early' dissatisfaction at one year, 'persistent' dissatisfaction with longer follow-up, and 'late' dissatisfaction developing in previously satisfied patients at one year. All three groups have different independent predictors of satisfaction, and potentially addressing risk factors specific to these groups may improve patient outcome and their satisfaction. Cite this article: Bone Joint J 2018;100-B:161-9.

The rate and predictors of patient satisfaction after total knee arthroplasty are influenced by the focus of the question: a standard satisfaction question is required.

Aims: The primary aim of this study was to assess the rate of patient satisfaction one year after total knee arthroplasty (TKA) according to the focus of the question asked. The secondary aims were to identify independent predictors of patient satisfaction according to the focus of the question. Patients and Methods: A retrospective cohort of 2521 patients undergoing a primary unilateral TKA were identified from an established regional arthroplasty database. Patient demographics, comorbidities, Western Ontario and McMaster Universities Osteoarthritis Index (WOMAC) and 12-Item Short-Form Health Survey (SF-12) scores were collected preoperatively and one year postoperatively. Patient satisfaction was assessed using four questions, which focused on overall outcome, activity, work, and pain. Logistic regression analysis was used to identify independent preoperative predictors of increased stiffness when adjusting for confounding variables. Results: Using patient satisfaction with the overall outcome (n = 2265, 89.8%) as the standard, there was no difference in the rate of satisfaction for pain relief (n = 2277, odds ratio (OR) 0.95, 95% confident intervals (CI) 0.79 to 1.14, p = 0.60), but patients were more likely to be dissatisfied with activities (79.3%, n = 2000/2521, OR 2.22, 95% CI 1.96 to 2.70, p < 0.001) and work (85.8%, n = 2163/2521, OR 1.47, 95% CI 1.23 to 1.75, p < 0.001). Logistic regression analysis identified different predictors of satisfaction for each of the focused satisfaction questions. Overall satisfaction was influenced by diabetes (p = 0.03), depression (p = 0.004), back pain (p < 0.001), and SF-12 physical (p = 0.008) and mental (p = 0.01) components. Satisfaction with activities was influenced by depression (p = 0.001), back pain (p < 0.001), WOMAC stiffness score (p = 0.03), and SF-12 physical (p < 0.001) and mental (p < 0.001) components. Satisfaction with work was influenced by depression (p = 0.007), back pain (p < 0.001), WOMAC function (p = 0.04) and stiffness (p = 0.05) scores, and SF-12 physical (p < 0.001) and mental (p < 0.001) components. Satisfaction with pain relief was influenced by diabetes (p < 0.001), back pain (p < 0.001), and SF-12 mental component (p = 0.04). Conclusion: The focus of the satisfaction question significantly influences the rate and the predictors of patient satisfaction after TKA. Cite this article: Bone Joint J 2018;100-B:740-8.

Issues of Survivorship and Rehabilitation in Soft Tissue Sarcoma.

As the number of survivors of extremity soft tissue sarcoma increases, so does the need to understand the experience of survivors and develop measures, systems and services that support rehabilitation into normal life roles. Survivorship includes considerations of the physical, psychological and social domains, of which the physical sequelae of treatment are the best characterised in the literature. The survivorship experience may include disability, pain, lymphoedema, psychological problems, as well as difficulty with employment, relationships and lower quality of life. Rehabilitation strategies for extremity sarcoma patients must be personalised, holistic and begin early in the pathway, ideally before the first treatment intervention. The International Classification of Functioning, Disability and Health model is a useful framework for combining assessments, including objective outcome measures, which can be combined into a rehabilitation prescription. Research is needed to develop an evidence base for rehabilitation interventions to support patients with extremity soft tissue sarcoma.

Objective clinical measurement of physical functioning after treatment for lower extremity sarcoma - A systematic review.

BACKGROUND: Physical impairments and activity restrictions cause significant morbidity after surgery for sarcoma. Yet objective assessment of key components of balance, gait and physical activity (PA), using valid and reliable outcome measures, is lacking in routine clinical practice. PURPOSE OF REVIEW: We therefore performed a systematic review to identify studies quantifying balance, gait and PA in clinically useful ways, after treatment for lower extremity sarcoma. PATIENTS AND METHODS: Relevant articles quantifying balance, gait and PA in patients who underwent surgery for lower extremity bone or soft tissue sarcoma were identified from Medline, Embase, Scopus, and Web of Science up to February 2016. Results were compiled by principal research findings, objective measures used, their ability to detect differences between important clinical groups, change over time and reliability. RESULTS: Eighteen articles were included. Surgery had a significant impact on outcomes (p < 0.05). A wide range of measures and concerns about accuracy of measurement were noted, as gait and PA measures did not discriminate between distinct clinical groups such as limb sparing surgery and amputation, and did not detect changes over time. Few studies investigated reliability (n = 1) and sensitivity to change (n = 4). CONCLUSION: There is a deficit of studies quantifying balance, gait and PA in patients with lower extremity sarcoma. Studies did not use consistent, valid and reliable instruments. There is an urgent need to develop novel objective measures of physical functioning in this patient group to encourage evidence-based clinical care.

Extraskeletal osteosarcoma: A European Musculoskeletal Oncology Society study on 266 patients.

PURPOSE: Prognosis of extraskeletal osteosarcoma (ESOS) is reported to be poorer than that of skeletal osteosarcoma. This multicenter retrospective study aimed to evaluate factors influencing ESOS prognosis. PATIENTS AND METHODS: Members of the European Musculoskeletal Oncology Society (EMSOS) submitted institutional data on patients with ESOS. RESULTS: Data from 274 patients treated from 1981 to 2014 were collected from 16 EMSOS centres; 266 patients were eligible. Fifty (18.7%) had metastases at diagnosis. Of 216 patients with localised disease, 211 (98%) underwent surgery (R0 = 70.6%, R1 = 27%). Five-year overall survival (OS) for all 266 patients was 47% (95% CI 40-54%). Five-year OS for metastatic patients was 27% (95% CI 13-41%). In the analysis restricted to the 211 localised patients who achieved complete remission after surgery 5-year OS was 51.4% (95% CI 44-59%) and 5-year disease-free survival (DFS) was 43% (95% CI 35-51%). One hundred twenty-one patients (57.3%) received adjuvant or neoadjuvant chemotherapy and 80 patients (37.9%) received radiotherapy. A favourable trend was seen for osteosarcoma-type chemotherapy versus soft tissue sarcoma-type (doxorubicin ± ifosfamide) regimens. For the 211 patients in complete remission after surgery, patient age, tumour size, margins and chemotherapy were positive prognostic factors for DFS and OS by univariate analysis. At multivariate analysis, patient age (≤40 years versus >40 years) (P = 0.05), tumour size (P = 0.0001) and receipt of chemotherapy (P = 0.006) were statistically significant prognostic factors for survival. CONCLUSION: Patient age and tumour size are factors influencing ESOS prognosis. Higher survival was observed in patients who received perioperative chemotherapy with a trend in favour of multiagent osteosarcoma-type regimen which included doxorubicin, ifosfamide and cisplatin.

Myxofibrosarcoma of the extremity and trunk: a multidisciplinary approach leads to good local rates of LOCAL control.

AIMS: Myxofibrosarcomas (MFSs) are malignant soft-tissue sarcomas characteristically presenting as painless slowly growing masses in the extremities. Locally infiltrative growth means that the risk of local recurrence is high. We reviewed our experience to make recommendations about resection strategies and the role of the multidisciplinary team in the management of these tumours. PATIENTS AND METHODS: Patients with a primary or recurrent MFS who were treated surgically in our unit between 1997 and 2012 were included in the study. Clinical records and imaging were reviewed. A total of 50 patients with a median age of 68.4 years (interquartile range 61.6 to 81.8) were included. There were 35 men; 49 underwent surgery in our unit. RESULTS: The lower limb was the most common site (32/50, 64%). The mean size of the tumours was 8.95 cm (1.5 to 27.0); 26 (52%) were French Fédération Nationale des Centres de Lutte Contre le Cancer grade III. A total of 21 (43%) had positive margins after the initial excision; 11 underwent further excision. Histology showed microscopic spread of up to 29 mm beyond macroscopic tumour. Local recurrence occurred in seven patients (14%) at a mean of 21 months (3 to 33) and 15 (30%) developed metastases at a mean of 17 months (3 to 30) post-operatively. CONCLUSION: High rates of positive margins and the need for further excision makes this tumour particularly suited to management by multidisciplinary surgical teams. Microscopic tumour can be present up to 29 mm from the macroscopic tumour in fascially-based tumours. Cite this article: Bone Joint J 2016;98-B:1682-8.

Routes to diagnosis for sarcoma - Describing the sarcoma patient journey.

BACKGROUND: Understanding the route to diagnosis for patients with cancer is important to improve the diagnostic pathway and therefore outcomes. We aimed to investigate routes to diagnosis for patients with sarcoma in England. METHODS: National patient level datasets relating to 7716 soft tissue and 1240 bone sarcoma patients diagnosed between 2006 and 2008 were analysed. Routes to diagnosis were defined as: "Two Week Wait Referral", "GP Referral", "Other Outpatient", "Inpatient Elective", "Emergency Presentation", "Death Certificate Only" and "Unknown". RESULTS: Patients with sarcoma are most likely to be diagnosed after "GP Referral" or "Emergency Presentation" and are less likely to be referred under a two week wait compared with other malignancies. Patients with bone sarcoma under 10 or over 80 years of age were more likely to present by emergency routes, as were patients with vertebral column, pelvis or unspecified site tumours and those with Ewing's sarcoma or sarcoma NOS. Patients with soft tissue sarcoma under 19 or over 80 years of age and patients with GI tract tumours were more likely to present by emergency routes. Patients presenting by emergency routes more often had metastases and had lower 1 year survival. Patients from least deprived quintiles more often presented by unknown routes: those from more deprived quintiles more often presented by emergency routes. CONCLUSION: Routes to diagnosis for sarcoma patients differ from other cancers. Interventions to improve the diagnostic experience should consider the very young and elderly, tumours in abdominal, pelvic or spinal locations and on reducing emergency presentations.

Physical functioning, pain and quality of life after amputation for musculoskeletal tumours: a national survey.

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.

Classification of positive margins after resection of soft-tissue sarcoma of the limb predicts the risk of local recurrence.

We considered whether a positive margin occurring after resection of a soft-tissue sarcoma of a limb would affect the incidence of local recurrence. Patients with low-grade liposarcomas were expected to be a low-risk group as were those who had positive margins planned before surgery to preserve critical structures. Two groups, however, were expected to be at a higher risk, namely, patients who had undergone unplanned excision elsewhere with a positive margin on re-excision and those with unplanned positive margins occurring during primary resection. Of 566 patients in a prospective database, 87 with positive margins after limb-sparing surgery and adjuvant radiotherapy were grouped according to the clinical scenario by an observer blinded to the outcome. The rate of local recurrence differed significantly between the two low- (4.2% and 3.6%) and the two high-risk groups (31.6% and 37.5%). This classification therefore provides useful information about the incidence of local recurrence after positive-margin resection.

Instability after major tumor resection: prevention and treatment.

Instability is a significant problem after resection of musculoskeletal tumors. In this article, the authors discuss the issue of instability after resections of the pelvis, the femur, and the proximal tibia. The options for reconstruction in each area are discussed along with a review of the literature and a description of the authors' preferred approach.

False aneurysm and brachial plexus palsy complicating a proximal humeral exostosis.

An unusual case is described in which a false aneurysm of the brachial artery secondary to an exostosis of the proximal humerus caused a compressive lesion of the brachial plexus. Surgical treatment of the exostosis and the false aneurysm relieved the symptoms.

What do we know about survivorship after treatment for extremity sarcoma? A systematic review.

OBJECTIVE: The varied presentations and treatments of extremity bone and soft tissue sarcoma mean that the issues faced by survivors are diverse and complex. The aim of this systematic review was to investigate what is known about this topic with a view to identifying areas for further research or service development. METHODS: This was a review of the English language literature identified from Medline and Ovid and hand searches published between January 2000 and September 2012. Results were compiled according to physical, psychological and social domains of survivorship. RESULTS: Of 182 studies identified, 22 met the inclusion criteria. There is a wide range of outcome measures used and a need for more objective measures. Unsurprisingly, survivors of extremity sarcoma typically demonstrate lower levels of physical functioning than healthy controls. In addition, survivors demonstrate a substantial psychological morbidity. CONCLUSIONS: Services for survivors of extremity sarcoma should include rehabilitation and psychological support, sexual health services, expert pain management, and support to return to work.

Experience of angiosarcoma in the North of England Bone and Soft Tissue Tumour Service.

INTRODUCTION: Angiosarcomas are rare aggressive sarcomas of vascular endothelial origin. These tumours have the potential to be multicentric and are associated with high rates of local recurrence, which makes treatment challenging. The gold-standard is that these patients are managed in specialist centres by a multidisciplinary team. We present our experience of managing patients with angiosarcoma in the North of England Bone and Soft Tissue Tumour Service and a review of the literature. METHODS: A prospectively collated electronic database was used to identify patients with angiosarcoma treated between 2000 and 2008, and an analysis performed of demographics, anatomical site, surgical excision and reconstruction, local disease recurrence and metastatic disease. RESULTS: Fifteen patients (ten female, five male, mean age 71 years) were identified. Eight patients developed tumours in a previously irradiated area, after a mean of 11 years. Six patients had metastatic disease at presentation. Fourteen patients underwent wide surgical excision of the tumour, of which nine required defect reconstruction (five free latissimus dorsi flaps, two free anterolateral thigh flaps, two pedicled latissimus dorsi flaps). One patient was treated with chemotherapy only. Five of 14 patients received adjuvant radiotherapy, and one received adjuvant chemotherapy. Two out of 14 patients developed local recurrence. Eight patients developed metastases, the majority of which were pulmonary. Estimated five-year survival was calculated as 33% in our patient cohort. CONCLUSIONS: Angiosarcomas are aggressive, difficult to treat tumours, which can occur secondary to a multitude of causes. Clinical suspicion, biopsy and early diagnosis are essential to allow optimum treatment, which currently consists of radical surgery, together with adjuvant radiotherapy and chemotherapy.