Extremity Tumors Research Articles

Are Accelerometer-based Functional Outcome Assessments Feasible and Valid After Treatment for Lower Extremity Sarcomas?

Aspects of physical functioning, including balance and gait, are affected after surgery for lower limb musculoskeletal tumors. These are not routinely measured but likely are related to how well patients function after resection or amputation for a bone or soft tissue sarcoma. Small, inexpensive portable accelerometers are available that might be clinically useful to assess balance and gait in these patients, but they have not been well studied. In patients treated for lower extremity musculoskeletal tumors, we asked: (1) Are accelerometer-based body-worn monitor assessments of balance, gait, and timed up-and-go tests (TUG) feasible and acceptable? (2) Do these accelerometer-based body-worn monitor assessments produce clinically useful data (face validity), distinguish between patients and controls (discriminant validity), reflect findings obtained using existing clinical measures (convergent validity) and standard manual techniques in clinic (concurrent validity)? This was a prospective cross-sectional study. Out of 97 patients approached, 34 adult patients treated for tumors in the femur/thigh (19), pelvis/hip (3), tibia/leg (9), or ankle/foot (3) were included in this study. Twenty-seven had limb-sparing surgery and seven underwent amputation. Patients performed standard activities while wearing a body-worn monitor on the lower back, including standing, walking, and TUG tests. Summary measures of balance (area [ellipsis], magnitude [root mean square {RMS}], jerkiness [jerk], frequency of postural sway below which 95% of power of acceleration power spectrum is observed [f95 of postural sway]), gait [temporal outcomes, step length and velocity], and TUG time were derived. Body-worn monitor assessments were evaluated for feasibility by investigating data loss and patient-reported acceptability and comfort. In addition, outcomes in patients were compared with datasets of healthy participants collected in parallel studies using identical methods as in this study to assess discriminant validity. Body-worn monitor assessments were also investigated for their relationships with routine clinical scales (the Musculoskeletal Tumour Society Scoring system [MSTS], the Toronto Extremity Salvage Score [TESS], and the Quality of life-Cancer survivors [QoL-CS)] to assess convergent validity and their agreement with standard manual techniques (video and stopwatch) to assess concurrent validity. Although this was a small patient group, there were initial indications that body-worn monitor assessments were well-tolerated, feasible to perform, acceptable to patients who responded (95% [19 of 20] of patients found the body-worn monitor acceptable and comfortable and 85% [17 of 20] found it user-friendly), and produced clinically useful data comparable with the evidence. Balance and gait measures distinguished patients and controls (discriminant validity), for instance balance outcome (ellipsis) in patients (0.0475 m/s [95% confidence interval 0.0251 to 0.0810]) was affected compared with controls (0.0007 m/s [95% CI 0.0003 to 0.0502]; p = 0.001). Similarly gait outcome (step time) was affected in patients (0.483 seconds [95% CI 0.451 to 0.512]) compared with controls (0.541 seconds [95% CI 0.496 to 0.573]; p < 0.001). Moreover, body-worn monitor assessments showed relationships with existing clinical scales (convergent validity), for instance ellipsis with MSTS (r = -0.393; p = 0.024). Similarly, manual techniques showed excellent agreement with body-worn monitor assessments (concurrent validity), for instance stopwatch time 22.28 +/- 6.93 seconds with iTUG time 21.18 +/- 6.23 seconds (intraclass correlation coefficient agreement = 0.933; p < 0.001). P < 0.05 was considered statistically significant. Although we had a small, heterogeneous patient population, this pilot study suggests that body-worn monitors might be useful clinically to quantify physical functioning in patients treated for lower extremity tumors. Balance and gait relate to disability and quality of life. These measurements could provide clinicians with useful novel information on balance and gait, which in turn could guide rehabilitation strategies. Level III, diagnostic study.

Predicting the prognosis of undifferentiated pleomorphic soft tissue sarcoma: a 20‐year experience of 266 cases

Undifferentiated pleomorphic sarcoma (UPS) is a rare malignant tumour of mesenchymal origin, which was conceived following re-classification of malignant fibrous histiocytoma (MFH). The objective of this study is to determine prognostic factors for the outcome of UPS, following multi-modal treatment. Data of UPS tumours from 1996 to 2016 were collected, totalling 266 unique UPS patients. Median follow-up was 7.8 years. All tumours were retrospectively analysed for prognostic factors of the disease, including local recurrence (LR) and metastatic disease (MD) at diagnosis, tumour size, grade, location and depth, patient age, adjuvant therapy and surgical margin. Overall survival (OS), post-treatment LR and metastatic-free survival were assessed as outcomes. The 5- and 10-year OS rates for all ages were 60% and 48%, respectively, with a median survival time of 10.1 years. Multivariate analysis revealed that the adverse prognostic factors associated with decreased OS were older age (P < 0.001; hazard ratio 1.03) and MD at diagnosis (P = 0.001; 2.89), with upper extremity tumours being favourable (P = 0.043; 2.30). Poor prognosis for post-operative LR was associated with older age (P = 0.046; 1.03) and positive surgical margins (P = 0.028; 2.68). Increased post-treatment MD was seen in patients with large tumours (5-9 cm (P < 0.001; 4.42), ≥10 cm (P < 0.001; 6.80)) and MD at diagnosis (P < 0.001; 3.99), adjuvant therapy was favourable, shown to reduce MD (P < 0.001; 0.34). UPS is a high-grade soft tissue sarcoma, for which surgery striving for negative margins, with radiotherapy, is the treatment of choice. Older age, lower extremity location, MD at presentation, large size and positive surgical margins, were unfavourable.

Assessing the Role of Neoadjuvant Chemotherapy in Primary High-Risk Truncal/Extremity Soft Tissue Sarcomas: An Analysis of the Multi-institutional U.S. Sarcoma Collaborative.

The role of neoadjuvant chemotherapy (NCT) for high-risk soft tissue sarcoma (STS) is questioned. This study aimed to define which patients may experience a survival advantage with NCT. All the patients from the U.S. Sarcoma Collaborative database (2000-2016) who underwent curative-intent resection of high-grade, primary truncal/extremity STS size 5cm or larger were included in this study. The primary end points were recurrence-free survival (RFS) and overall survival (OS). Of the 4153 patients, 770 were included in the study. The median tumor size was 10cm, and 669 of the patients (87%) had extremity tumors. The most common histology was undifferentiated pleomorphic sarcoma (UPS), found in 42% of the patients. Of the 770 patients, 216 (28%) received NCT. The patients who received NCT had deeper, larger tumors (p < 0.001). Of the patients with tumors 5cm or larger and 8cm or larger, NCT was not associated with improved RFS or OS. However for the patients with tumors 10cm or larger, NCT was associated with improved 5-year RFS (51% vs 40%; p = 0.053) and 5-year OS (58% vs 47%; p = 0.043). By location, the patients with extremity tumors 10cm or larger but not truncal tumors had improved 5-yearr RFS (54% vs 42%; p = 0.042) and 5-year OS (61% vs 47%; p = 0.015) with NCT. According to histology, no subtype had improved RFS or OS with NCT, although the patients with UPS had a trend toward improved 5-year RFS (56% vs 42%; p = 0.092) and 5-year OS (66% vs 52%; p = 0.103) with NCT. For the patients with high-grade STS, NCT was associated with improved RFS and OS when tumors were 10cm or larger and located in the extremity. However, no histiotype-specific advantage was identified. Future studies assessing the efficacy of NCT may consider focusing on these patients, with added focus on histology-specific strategies.

Functional And Survival Outcome Of Egyptian Children And Adolescents With Malignant Bone Tumors: An Experience In A Setting Of Limited Health Resource

Abstract Objective Evaluate outcome of paediatric malignant bone tumours at Ain Shams University, Egypt, from January 2003 to July 2016. Methods Retrospective data analysis regarding clinico-epidemiological aspects, treatment outcomes, survival analysis and musculoskeletal tumour society score (MSTS score). Results The study included 37 patients; 22 had Ewing sarcoma (ES) and 15 had osteosarcoma, male: female ratio 0.85:1, median ages of 11. The overall frequency was 2.3% among all cancers. There is wide range of time lag until diagnosis. Patients with ES were significantly younger than those with osteosarcoma were. Swelling was the most common presenting symptom and femur was the most common affected site. Fifteen patients fulfilled MSTS criteria; most of them had excellent MSTS score, which significantly affected by type of surgery. ES patients were treated with POG#9354/CCG#7942 protocols and osteosarcoma with CCG#7921 protocol. Limb salvage was the most common type for surgical local control. Most common cause of death was relapse, whereas infection was the most common complication of treatment. 1-year, 2-year, 3-year overall-survival of osteosarcoma were 93.3%, 40%, and 13.3% respectively and 77.3%, 40.9%, and 18.2% respectively for ES. 1-year, 2-year, 3-year event-free-survival were 80%, 40%, and 13.3% respectively and 72.7%, 22.7%, and 18.2% respectively for ES patients. Conclusion Although survival rates for malignant bone tumours are still unsatisfactory, the functional outcome of extremity tumours after limb salvage procedures is promising

Psychosocial Distress Scores and Needs among Newly Diagnosed Sarcoma Patients: A Provincial Experience.

Introduction Information on the psychosocial distress and needs of sarcoma patients at diagnosis is sparse. The Canadian Problem Checklist (CPC) and Psychosocial Screen for Cancer-Revised (PSSCAN-R) are validated tools to identify cancer patients' distress and are administered to all new patients referred to BC Cancer prior to their consultation. We used the CPC and PSSCAN-R to understand sarcoma patients' needs at the initial oncology consultation in British Columbia, Canada. Materials and Methods All sarcoma patients who completed the CPC and PSSCAN-R within 6 months of diagnosis between 2011 and 2016 were included. The retrospective chart review identified baseline demographics: age, performance status, disease location, resectability, and histology. Analysis was conducted using descriptive statistics, chi-squared test, Fisher's exact test, and Kaplan–Meier method. Results 413 sarcoma patients were identified. The majority of patients were over the age of 40 (83.3%) with ECOG performance status 0-1 (82.6%) and lower extremity tumors (55.4%). The most common diagnoses were liposarcoma 21.3%, undifferentiated pleomorphic sarcoma 12.1%, and myxofibrosarcoma 11.1%. At the initial consultation, 42.6% of patients were deemed resectable, 8.5% unresectable/metastatic, and 48.9% required further staging investigations. The top three patient-reported distress symptoms were feeling tense and unable to relax (50%), feeling nervous and shaky (48%), and experiencing repetitive and scary thoughts (42%). 38% of patients had subclinical/clinical anxiety symptoms, and 21% of patients had subclinical/clinical depression symptoms. 5% of patients expressed suicidal ideation. The top three concerns/needs were understanding of illness/treatment (45.5%), fear/worries (45.3%), and worry about family (23%). No differences in overall survival were identified for patients displaying symptoms of depression or anxiety versus no symptoms. Discussion Up to 45% of sarcoma patients experience some form of psychological distress at disease presentation. Patients desire information about their diagnosis and treatment. Tailored interventions to individual psychological comorbidity and improved patient education resources would be beneficial.

The Pediatric Toronto Extremity Salvage Score (pTESS): Validation of a Self-reported Functional Outcomes Tool for Children with Extremity Tumors.

The physical function of children with sarcoma after surgery has not been studied explicitly. This paucity of research is partly because of the lack of a sufficiently sensitive pediatric functional measure. The goal of this study was to establish and validate a standardized measure of physical function in pediatric patients with extremity tumors. (1) What is the best format and content for new upper- and lower-extremity measures of physical function in the pediatric population? (2) Do the new measures exhibit floor and/or ceiling effects, internal consistency, and test-retest reliability? (3) Are the new measures valid? In Phase 1, interviews with 17 consecutive children and adolescents with bone tumors were conducted to modify the format and content of draft versions of the pediatric Toronto Extremity Salvage Score (pTESS). In Phase 2, the pTESS was formally translated into French. In Phase 3, 122 participants between 7 and 17.9 years old with malignant or benign-aggressive bone tumors completed the limb-specific measure on two occasions. Older adolescents also completed the adult TESS. Floor and ceiling effects, internal consistency, test-retest reliability, and validity were evaluated. Feedback from interviews resulted in the removal, addition, and modification of draft items, and the pTESS-Leg and pTESS-Arm questionnaires were finalized. Both versions exhibited no floor or ceiling effects and high internal consistency (α > 0.92). The test-retest reliability was excellent for the pTESS-Leg (intraclass correlation coefficient [ICC] = 0.94; 95% CI, 0.90-0.97) and good for the pTESS-Arm (ICC = 0.86; 95% CI, 0.61-0.96). Known-group validity (ability to discriminate between groups) was demonstrated by lower mean pTESS-Leg scores for participants using gait aids or braces (mean = 68; SD = 21) than for those who did not (mean = 87; SD = 11; p < 0.001). There was no significant difference between pTESS arm scores among respondents using a brace (n = 5; mean = 73; SD = 11) and those without (n = 22; mean = 83; SD = 19; p = 0.13). To evaluate construct validity, we tested a priori hypotheses. The duration since chemotherapy correlated moderately with higher pTESS-Leg scores (r = 0.4; p < 0.001) but not with pTESS-Arm scores (r = 0.1; p = 0.80), and the duration since tumor resection correlated moderately with higher pTESS-Leg scores (r = 0.4; p < 0.001) but not pTESS-Arm scores (r = 0.2; p = 0.4). Higher VAS scores (that is, it was harder to do things) antecorrelated with both pTESS versions (pTESS-Leg: r = -0.7; p < 0.001; pTESS-Arm: r = -0.8; p < 0.001). To assess criterion validity, we compared the pTESS with the current "gold standard" (adult TESS). Among adolescents, strong correlations were observed between the TESS and pTESS-Leg (r = 0.97, p < 0.001) and pTESS-Arm (r = 0.9, p = 0.007). Both pTESS versions exhibited no floor or ceiling effects and had high internal consistency. The pTESS-Leg demonstrated excellent reliability and validity, and the pTESS-Arm demonstrated good reliability and reasonable validity. The pTESS is recommended for cross-sectional evaluation of self-reported physical function in pediatric patients with bone tumors. Level II, outcome measurement development.

Individual Risk Evaluation for Local Recurrence and Distant Metastasis in Ewing Sarcoma: A Multistate Model

Background: This study aims to investigate the effect of surgical margins, histological response and radiotherapy, on local recurrence (LR), distant metastasis (DM) and survival in Ewing sarcoma patients. Methods: We performed a retrospective analysis of 982 Ewing sarcoma patients who underwent surgery after induction chemotherapy. To study disease evolution a multistate model with initial state surgery, intermediate states LR, pulmonary metastasis (DMpulm), other DM±LR (DMother) and final state death was estimated. The effect of risk factors on different disease states was estimated using Cox proportional hazard models. Hazard ratios (HRs) along with 95% confidence interval (95%CI) are provided. Overall survival (OS) from surgery was calculated using Kaplan-Meier's estimates. Findings: The median follow-up was 7·6 years (95%CI 7·2-8·0). 5-year OS was 74% (71-77) for localized disease, 56% (47-55) for pulmonary metastasis, 43% (33-53%) for extrapulmonary metastasis. Risk factors for LR are pelvic location, HR 2·04(95%CI 1·10-3·80), and marginal/intralesional resection, HR 2·28(1·25-4·16), administration of radiotherapy is protective, HR 0·52(0·28-0·95), for all tumor sites combined; risk factors for DMpulm are <90% necrosis, HR 2·13(1·13-4·00), and previous pulmonary metastasis, HR 4·90(2·28-8·52). Risk factors for DMother are 90-99% necrosis, HR 1·56(1·09-2·23), <90% necrosis, HR 2·66 (1·87-3·79), and previous bone/other metastasis, HR 3·08(2·03-4·70). Risk factors for death without LR/DM are pulmonary metastasis, HR 8·08(4·01-16·29), bone/other metastasis, HR 10·23(4·90-21·36) and <90% necrosis. Early LR (0-24 months) negatively influences survival, HR 3·79(1·34-10·76). Once DMpulm/DMother arise only previous bone/other metastasis remain prognostic for death, HR 1·74(1·10-2·75). Interpretation: Disease extent at diagnosis and histological response are the main risk factors for progression to distant metastasis or death. Tumor site and surgical margins are important risk factors for LR. In case disease progression occurs, previous risk factors lose their relevance, and the balance between toxicity of intensive salvage treatments and quality of life in the remaining life span of these patients should be carefully considered. In case of isolated LR, time to recurrence is important for decision-making, since early LR (0-24 months) negatively influences survival. Radiotherapy seems to be protective for LR especially in pelvic/axial locations. The low percentage of LR in extremity tumors and associated toxicity questions the need for radiotherapy in extremity Ewing sarcoma. Funding Statement: This study was supported by a donation from the Team Westland Foundation (to PDSD), a German Cancer Aid grant 108128 to UD; ERA-Net-TRANSCAN consortium, project number 01KT1310, and Euro Ewing Consortium EEC, project number EU-FP7 602856, both funded under the European Commission Seventh Framework Program FP7-HEALTH (to UD); and by David Dressler+ (to UD). Declaration of Interests: The authors have no conflict of interest. Ethics Approval Statement: This retrospective study was reviewed and approved by the institutional review board of Leiden University Medical Center (Leiden, The Netherlands) and a waiver for informed consent was granted.

Short Term Complications and Functional Results of Sarcoma Limb Salvage Surgeries.

Limb salvaging surgeries are current surgical treatment of extremity bone sarcomas. Resected bone replacement consists of two main methods; tumor prosthesis versus structural allograft. Biological reconstruction with an allograft is an economic cheap method in young sarcoma patients, however, the surgeons are more convinced with tumor prosthesis replacement. We evaluated the short-term complications and functional results of 40 patients with aggressive extremity tumors in a retrospective cohort study. The mean age of cases was 25 and we followed them for 24 months. 17 patients underwent tumor prosthesis replacement after wide resection of limb sarcomas. 16 cases had structural allograft reconstruction and 7 patients treated with amputation. We matched confounders including age, sex, blood cell count and chemotherapy treatment in the study groups. We found 15 major complications (45.5%) in limb salvage surgeries composing infection, allograft nonunion, allograft fracture, prosthesis fracture, prosthesis loosening and device failure that needed another surgery to be resolved. We had 10 major complications in allograft group (62%) and 5 in tumor prosthesis group (29.4%). Although the rate of complications was higher in allograft group, it didn't statistically indicate strong correlation (Fisher's exact: 0.084). Mean Musculo-Skeletal tumor rating Scale (MSTS) score was 25.8(73.7%) and 22.3(63.7%) in allograft group and prosthesis cases respectively. MSTS score had a normal distribution in the different groups with no significant difference between them. Although complications were higher in the allograft group, allograft could be offered to bone sarcoma patients, whom are predicted to have short life expectancy.

Causes and Frequencies of Reoperations After Endoprosthetic Reconstructions for Extremity Tumor Surgery: A Systematic Review.

Excision of bone tumors and endoprosthetic reconstruction allow patients early weightbearing and a potential functional advantage compared with amputation. These reconstructions do not restore the limb to normal status, however, and patients are subject to complications that may result in revision or loss of the limb. Because better understanding of these complications based on current information might help the patient and surgeon in decision-making, we undertook a systematic review of studies published on this topic. (1) What are the primary modes and proportion of failure of tumor endoprostheses in patients undergoing reconstruction after excision of primary extremity bone sarcomas? We systematically searched MEDLINE, Embase, and the Cochrane Library for all studies published from April 15, 1998, to April 15, 2018. Three reviewers independently reviewed studies reporting endoprosthetic reconstruction survival and events requiring revision for primary extremity bone tumors treated with endoprosthetic reconstruction for inclusion and performed independent data extraction. We excluded all studies with fewer than five patients, any systematic review/meta-analyses, and any study not reporting on primary extremity bone tumors. All discrepancies were resolved by the study's senior author. Data extracted from included studies were any reoperation event for wound dehiscence, any operative fixation for a pathologic fracture, and any revision of the primary endoprosthesis for implant wear or breakage, deep infection not amenable to prosthesis retention, or for local recurrence. We assessed the overall quality of the evidence with the Methodological Index for Non-Randomized Studies (MINORS) approach with a higher MINORS score representative of a more methodologically rigorous study with a total possible score of 16 points for noncomparative and 24 points for comparative studies. Forty-nine studies met criteria for inclusion from an initial search return of 904 studies, of which no studies were randomized controlled trials. From a total patient population of 2721, there was a mean followup of 93 months (range, 1-516 months) with loss to followup or death occurring in 447 of 2118 (21%) patients with six studies not providing loss to followup data. The mean MINORS score was 14 for prospective studies and 11 for retrospective studies. Overall, there were 1283 reoperations among the 2721 (47%) patients. Reoperation for mechanical endoprosthetic events (soft tissue dehiscence or periarticular soft tissue instability, aseptic loosening, or implant wear/fracture) occurred in 907 of 2721 (33%) patients. Aseptic loosening occurred at a mean of 75 months (range, 1-376 months) in 212 of 315 patients (67%). Deep infection requiring removal of the initial prosthesis occurred in 247 of 2721 (9%) patients with deep infection occurring at a mean of 24 months (range, 1-372 months) in the 190 infections (77%) with time to infection data available. Local recurrence rates requiring revision or amputation occurred in 129 (5%) of all patients. There was an overall primary endoprosthesis survival rate without any surgical reintervention of 63% among reporting studies at a mean of 79 months followup. Failures of endoprosthetic reconstructions after extremity tumor surgery are common, most often resulting from implant wear or fracture, aseptic loosening, and infection. Importantly, the aggregated data are the first to attempt to quantify the time to specific complication types within this patient population. Deep infection not amenable to endoprosthesis retention appears to occur approximately 2 years postoperatively in most patients, with aseptic loosening occurring most commonly at 75 months. Although endoprosthetic reconstruction is one of the most common forms of reconstruction after bone tumor resection, the quality of published evidence regarding this procedure is of low quality with high loss to followup and data quality limiting interstudy analysis. The quality of the evidence is low with high loss to followup and inconsistent reporting of times to reintervention events. Although the most common modes of endoprosthetic failure in this population are well known, creation of quality prospective, collaborative databases would assist in clarifying and informing important elements of the followup process for these patients. Level IV, therapeutic study.

Novel 3D-printed prosthetic composite for reconstruction of massive bone defects in lower extremities after malignant tumor resection

ObjectiveTo introduce a novel 3D-printed prosthetic composite for reconstruction of massive bone defects after resection for bone malignancy of lower extremities. The design concept, surgical technique, and the preliminary outcomes were elaborated. MethodsPatients with primary malignant tumors of lower extremities requiring tumor resection and reconstruction were recruited between Jun 2015 and Nov 2018. Patient-specific 3D-printed prostheses were designed according to preoperative imaging data. After tumor resection, reconstruction was performed with composites consisting of 3D- printed prosthesis, beta-tricalcium phosphate (β-TCP) bioceramics and/or vascularized fibula. All patients underwent regular follow-up postoperatively. The functional outcomes were assessed by the Musculoskeletal Tumor Society score (MSTS). Oncological outcomes, imaging results, and complications were recorded and analyzed. ResultsTen cases averaging 12.90 years of age participated in this study. There were five femur and five tibia reconstructions. The mean follow-up period was 16.90 months. At last follow-up, all patients were alive without tumor recurrence. Average MSTS functional score was 80.33 ± 11.05%. All prostheses were intact and stable without failure or systemic breakage. No serious complications occurred after the operation. Postoperative X-ray, computed tomography (CT) and single-photon emission computed tomography (SPECT) showed an ideal integration between the bone and the prosthetic composite. Moreover, vascularized fibula and implanted β-TCP bioceramics indicated relatively high metabolic activity in vivo. ConclusionsPatient-specific 3D-printed prostheses combined with β-TCP bioceramics and/or vascularized fibula provide an excellent option for reconstruction of massive bone defects after lower extremity malignant tumor extirpation. Short-term follow up showed promising clinical results in recovering lower limb function, promoting osseointegration and reducing complications.

Breath-hold MR-HIFU hyperthermia: phantom and in vivo feasibility

Background: The use of magnetic resonance imaging-guided high-intensity focused ultrasound (MR-HIFU) to deliver mild hyperthermia requires stable temperature mapping for long durations. This study evaluates the effects of respiratory motion on MR thermometry precision in pediatric subjects and determines the in vivo feasibility of circumventing breathing-related motion artifacts by delivering MR thermometry-controlled HIFU mild hyperthermia during repeated forced breath holds.Materials and methods: Clinical and preclinical studies were conducted. Clinical studies were conducted without breath-holds. In phantoms, breathing motion was simulated by moving an aluminum block towards the phantom along a sinusoidal trajectory using an MR-compatible motion platform. In vivo experiments were performed in ventilated pigs. MR thermometry accuracy and stability were evaluated.Results: Clinical data confirmed acceptable MR thermometry accuracy (0.12–0.44 °C) in extremity tumors, but not in the tumors in the chest/spine and pelvis. In phantom studies, MR thermometry accuracy and stability improved to 0.37 ± 0.08 and 0.55 ± 0.18 °C during simulated breath-holds. In vivo MR thermometry accuracy and stability in porcine back muscle improved to 0.64 ± 0.22 and 0.71 ± 0.25 °C during breath-holds. MR-HIFU hyperthermia delivered during intermittent forced breath holds over 10 min duration heated an 18-mm diameter target region above 41 °C for 10.0 ± 1.0 min, without significant overheating. For a 10-min mild hyperthermia treatment, an optimal treatment effect (TIR > 9 min) could be achieved when combining 36–60 s periods of forced apnea with 60–155.5 s free-breathing.Conclusion: MR-HIFU delivery during forced breath holds enables stable control of mild hyperthermia in targets adjacent to moving anatomical structures.

Surgical excision and not chemotherapy is the most powerful modality in treating synovial sarcoma: the UK's North East experience.

We reviewed our experience of synovial sarcoma to identify factors predictive of local recurrence and overall survival, the impact of chemotherapy and outcomes after surgical excision alone. 81 patients were treated between 1997 and 2014 of mean age 39years (8-78). Tumours were in the extremity in 55 (67%). 9 patients presented with metastases and 10 with unresectable disease. Mean follow-up was 3.7years (SD 3.8). Treatment groups were palliative, surgery only, surgery and radiotherapy, or surgery with chemotherapy (with or without radiotherapy). Local recurrence-free survival (LRFS) was 73% at 5years, and 68% at 10 and 15years. In multivariate analysis, positive surgical margins were an independent predictor of LRFS. Overall survival (OS) was 50% at 5years for all patients, and 62% at 5years for those treated with curative intent. Larger tumour size and non-extremity locations were predictors of poorer OS. Patients who had chemotherapy did not have significantly better OS or LRS than others. These results show that where feasible, curative resection should not be delayed for chemotherapy. Treatment with surgery only can be associated with good outcomes in selected patients with smaller extremity tumours; although our series is small.

Risk factors for detectable metastatic disease at presentation in Ewing sarcoma – An analysis of the SEER registry

BackgroundEwing family of tumors (EFT) represents the second-most common primary bone malignancy in children and adolescents. Approximately 25% of patients have radiographically detectable metastatic disease at presentation and experience poorer five-year survival, yet risk factors for metastatic disease at presentation are poorly characterized. We sought to study patient characteristics associated with metastatic disease upon presentation for patients with EFT. MethodsWe identified EFT cases in the Surveillance, Epidemiology, and End Results Program (SEER) registry from 2004 to 2012. Using univariate analyses and multivariable logistic regression, we explored the relationship between demographic and clinical factors and the presence of detectable metastatic disease at presentation. ResultsAmong 870 EFT cases, 35% (n = 304) presented with detectable metastatic disease. These patients were commonly older (>24 years: 28% vs 19%, p = 0.002) and had a primary tumor site in the axial skeleton (56% vs 44%, p < 0.001). After adjusting for all covariates, compared to patients <11 years, those >24 years old faced a two-fold increase in the odds of metastatic disease (OR = 1.99, 95% CI: 1.17–3.38). Axial (OR = 2.31, 95% CI: 1.58–3.37) and “other” (OR = 2.35, 95% CI: 1.15–4.81) tumor locations had more than twice the likelihood of presenting with metastatic disease, compared to extremity tumor sites. Increasing tumor size conferred up to a three-fold increase in odds of metastatic disease (pTrend <0.001). ConclusionsAdvanced age, axial tumor location, and increasing tumor size are associated with increased odds of detectable metastatic disease upon presentation with EFT. Although these characteristics are not modifiable, they provide objective factors that may inform patient counseling of metastatic risk.

Assessing the Impact of Biopsychosocial Factors on Adolescent Survivors of Extremity Tumors

Abstract Date Presented 4/20/2018 This study investigated the survivorship needs of adult and young adult survivors of extremity tumors. By understanding the most important needs of this population, occupational therapy practitioners can create more targeted interventions that lead to higher participation and quality of life. Primary Author and Speaker: Lena Menkes Contributing Authors: Hannah Lee, Alex Wong

ADVANCED LYPOSARCOMA MYXOIDES OF THE EXTREMITY

Sarcomas are soft tissue tumors arising from primitive mesenchyme. Small incidence (4-5/100 000 in Europe) is the reason their pathogenesis is relatively unknown. Patient (38) complained of a growth on the upper part of right thigh, the size of a child’s head. A magnetic resonance imaging (MRI) scan was done and tumefaction was described: oval shape, 13.5 x 7.5 x 11cm in diameter, without infiltration of surrounding tissue. The tumor was surgically removed and was pathohistologically verified as low grade liposarcoma myxoides. After initial presentation the patient presented a series of recurrences and metastases in the abdominal wall, extremities and liver. Soft tissue metastasis from the lower extremities to the liver occur in 0.5% of cases and they are usually small and multiple, as in the presented patient. The European Sarcoma Medical Oncologist’s guide recommends that every extremity tumor larger than 5cm and suspicious of malignancy should be evaluated using biopsy and imaging methods. A biopsy should be done before excision under ultrasound or computerized tomography (CT) guidance with the goal of planning the best therapy protocol and prevention of a generalized disease with metastases. The recommended imaging method is an MRI scan, although sarcomas can have a benign presentation. Standard therapy includes surgical resection with local radiotherapy. Liposarcoma myxoides, a rare soft tissue tumor, demands biopsy and complete surgical removal with detailed and continuous postoperative imaging follow-up and oncological therapy. The therapeutic goal is to increase survival and preserve extremity function.

Feasibility of targeting canine soft tissue sarcoma with MR-guided high-intensity focused ultrasound

Purpose: Magnetic resonance imaging-guided high-intensity-focused ultrasound (MR-HIFU) is a non-invasive treatment modality that precisely focuses ultrasound energy within a tumour and can be customised to result in a wide range of local bioeffects. The purpose of this study was to determine the feasibility of using MR-HIFU to treat soft tissue sarcoma (STS) in dogs.Materials and methods: Medical records of dogs admitted to the Virginia-Maryland College of Veterinary Medicine from 1 January 2012 to 31 December 2016 were searched for a diagnosis of sarcoma with available cross-sectional imaging of the tumour (MRI or CT). Fifty-three (53) dogs were eligible for inclusion. Tumor tissue (in bone as well as in soft tissue) was considered targetable unless: (1) the ultrasound path was completely obstructed by bone or gas and (2) the MR-HIFU target was within the spinal cord or less than 1 cm from the margin of the spinal cord. Tumors were categorised as <50% targetable, ≥50% targetable or non-targetable.Results: Eighty-one percent of STS (81.1%, 43/53) were targetable. The head/spine tumour sites had the highest proportion of non-targetable tumours (36%, 9/25). The majority of truncal and axillary tumours were ≥50% targetable (88.9%, 16/18) ,and all extremity tumours were considered ≥50% targetable (100%, 5/5).Conclusions: The majority of STS were targetable. This is the first study to evaluate MR-HIFU targetability of canine STS. HIFU has potential as a therapeutic modality for treating STS in dogs, and this veterinary application is a possible model for treatment of naturally occurring STS in humans.

Time dependent dynamics of wound complications after preoperative radiotherapy in Extremity Soft Tissue Sarcomas

AimsThe purpose of the study was to investigate the time dependent dynamics of wound complications and local control after preoperative radiotherapy (RT) in Extremity Soft Tissue Sarcomas (ESTS). Patients & methodsIn this retrospective cohort study, all patients treated for an extremity sarcoma with pre-operative radiotherapy followed by surgery were identified from a prospectively maintained database. A wound complication (WC) was defined as any local complication of the surgical area requiring intervention, hospital readmission or significant extension of the initial admission period. ResultsA total of 191 preoperatively irradiated ESTS patients were included in this study. WC was seen in 31% of the patients (n = 60). WC started after a median time of 25 days from surgery, with a median duration of 76 days. Adiposity, smoking and a lower extremity or superficial tumor localization were significantly correlated with an increased WC rate. Risk factors for a duration of WC ≥ 120 days are early development of WC (≤21 days after surgery) and smoking. Local control rates after 1, 3 and 5 years were 99%, 93% and 93%, respectively. ConclusionApproximately one-third of patients selected for preoperative RT develops a WC, typically in smoking, adipose patients with superficial tumor localizations in the lower extremity. Based upon the well-established superior long-term functional outcome, maintained excellent local control rates and the temporary nature of the WC issue, preoperative RT remains our preferred treatment. Although, in patients at high risk of WC, post-operative RT might be considered.

Factors Associated With Acute and Chronic Wound Complications in Patients With Soft Tissue Sarcoma With Long-term Follow-up.

To identify the rates of acute and chronic wound complications and factors associated in a cohort of patients treated for soft tissue sarcoma (STS) with modern radiotherapy (RT) and surgical techniques. An Institutional Review Board-approved database was used to identify all adult nonmetastatic patients treated for STS at a single institution between 2006 and 2015 with a minimum follow-up of 1 year. Factors associated with acute and chronic wound complications were analyzed using binomial logistic regression including interaction terms. In all, 271 patients were identified with a median follow-up of 3.2 years. The rate of acute wound complications was 22.1%. On univariate analysis, trunk versus extremity location (P<0.001), radiation therapy (P=0.04), and preoperative therapy (P=0.03) were associated with acute wound complications and a trend was noted for reconstruction (P=0.07). On multivariate analysis, extremity tumors were associated with a higher rate of acute wound complications compared with trunk tumors without RT (P=0.02). Utilization of RT was associated with increased risk for extremity tumors (P=0.07). The rate of chronic wound complications was 3.3%. Radiation was associated with increased chronic wound complications (P=0.03) and trends were noted for trunk versus extremity location (P=0.08) and a history of acute wound complications (P=0.12). Several factors associated with acute and chronic wound complications were identified in STS patients including timing of RT, tumor site, and reconstruction use. The development of acute wound complications may also be associated with an increased risk of chronic wound complications.

Musculoskeletal Tumors of the Extremities: Challenges and Outcome of Management in a Nigeria Tertiary Hospital

Background: Patients with musculoskeletal tumors in developing countries often present late to the hospital and this poses serious challenges to the management, especially for malignant tumors. This study aims to highlight the various types of musculoskeletal extremity tumors seen in a Nigerian tertiary health center during the study period, the challenges encountered in managing them, and the outcome of the management. Materials and Methods: A prospective study in which all consenting patients with musculoskeletal extremity tumors who presented to the center from April 2015 to March 2017 were recruited. Results: Seventy‑two patients were managed during the study period. The mean age was 22.1 ± 4.5 years and the age group most affected was the 11–20 years group, n = 22 (30.6%). Male‑to‑female ratio was 1.6:1. The femur was the most commonly involved bone. Forty tumors were benign while 32 were malignant tumors. Osteochondroma was the most common benign tumor while osteosarcoma was the most common malignant tumor. The challenges encountered during the management were a late presentation, poverty, and traditional bonesetter intervention before the presentation. Patients with benign tumor had excision with good outcome in all. Twenty (62.5%) of the patients with a malignant tumor had the ablative procedure, two of these 20 patients died within 6 months of treatment, while 12 (37.5%) of the patients with malignant tumor refused the treatment. Conclusion: Management of musculoskeletal extremity tumors is highly challenging in this part of the country, especially the malignant types, due to the challenges mentioned. There is a need for more awareness about the disease, the Government should subsidize the cost of management of this disease, and more specialty training of personnel is necessary for appropriate management of the diseases. Keywords: Extremity, musculoskeletal, tumor.

Reconstructing diaphyseal tumors using radiated (50 Gy) autogenous tumor bone graft.

Establish oncologic safety using 50 Gy to sterilize tumor bearing bone before reimplantation in primary diaphyseal high grade extremity tumors, determine extracorporeal radiotherapy (ECRT) graft survival, and analyze factors that affect union at osteotomy sites. Seventy non metastatic patients underwent reconstruction with intercalary ECRT grafts sterilized with 50 Gy. Diagnosis included osteosarcoma (38) and Ewing's sarcoma (32). At last follow-up-49 patients were alive, 19 had died and 2 were lost to follow up. Survivors had minimum follow up of 3 years (range 39-127 months). Ninty one percent metaphyseal osteotomies united without additional intervention compared to 71% diaphyseal osteotomies. Additional small plate at diaphyseal osteotomy apparently reduced incidence of non-union (17% vs 31%) (P = 0.49). Addition of morsellised allograft did not help union. There were seven (10%) local recurrences, all in soft tissue. Seventeen of 69 patients (25%) needed removal of ECRT graft. Five year survival for ECRT graft (removal for all causes) was 79% and 84% (excluding removal for local recurrence). Reimplanting sterilized tumor bone using 50 Gy for tumor ablation is an easily applicable, oncologically safe, biological reconstruction option for primary diaphyseal extremity tumors.