Intermuscular extremity myxoid liposarcoma can be managed by marginal resection following neoadjuvant radiotherapy.

BACKGROUND: Compared with other soft tissue sarcomas, myxoid liposarcoma (MLS) occurs in younger patients, has a propensity for intermuscular locations and is highly radiosensitive. With pre-operative radiotherapy, intermuscular MLS demonstrates substantial volume reduction and can be easily separated from surrounding tissues during resection. However, it is unclear whether marginal excision of MLS is oncologically safe. This study aimed to assess the association between margins and survival in irradiated, intermuscular MLS. METHODS: The study identified 198 patients from seven sarcoma centres with a first presentation of localized, extremity, intermuscular MLS that received pre-operative radiotherapy and was diagnosed between 1990 and 2017. Patient and treatment characteristics, radiological and histological responses to neoadjuvant treatment and clinical surveillance were recorded. RESULTS: Margins were microscopically positive in 11% (n = 22), <1.0 mm in 15% (n = 29) and ≥1.0 mm in 72% (n = 143). There was no association between margin status and local recurrence-free, metastasis-free or overall survival. This finding held true even in patients at higher risk of worse overall survival based on multivariable analysis (% round cell≥5%, percentage ellipsoid tumour volume change ≤ -60.1%). CONCLUSION: Irradiated, extremity, intermuscular myxoid liposarcoma can safely undergo marginal resection without compromising oncologic control.

The Pediatric Arterialized Venous Flow-through Flap.

Arterialized venous flow-through flaps are solely vascularized through the venous plexus. The flaps were first described 40 years ago; however, reports of venous congestion and ischemia discouraged surgeons from adopting them into their armamentarium. Nevertheless, recent studies have demonstrated a resurgence of venous flow-through flaps for reconstruction of small to medium defects of the hand and digits. Although current data report variable levels of success in adults, no case reports have been published in the pediatric population for this type of flap. In this study, an arterialized venous flow-through flap from the medial forearm was used to reconstruct a volar hand defect in a young child. Flap markings, surgical technique, and aftercare are described. The surgery was uncomplicated, and the postoperative outcomes were aesthetically and functionally excellent. Venous flow-through flaps restore full-thickness defects, are relatively easy to perform, allow an early return to daily activities, and have almost no morbidity. These flaps offer excellent options for pediatric hand and finger defects.

Willingness of patients with sarcoma to participate in cancer surveillance research: a cross-sectional patient survey.

OBJECTIVES: To determine the proportion of patients with extremity sarcoma who would be willing to participate in a clinical trial in which they would be randomised to one of four different postoperative sarcoma surveillance regimens. Additionally, we assessed patients' perspectives on the burden of cancer care, factors that influence comfort with randomisation and the importance of cancer research. DESIGN: Prospective, cross-sectional patient survey. SETTING: Outpatient sarcoma clinics in Canada, the USA and Spain between May 2017 and April 2020. Survey data were entered into a study-specific database. PARTICIPANTS: Patients with extremity sarcoma who had completed definitive treatment from seven clinics across Canada, the USA and Spain. MAIN OUTCOME MEASURES: The proportion of patients with extremity sarcoma who would be willing to participate in a randomised controlled trial (RCT) that evaluates varying postoperative cancer surveillance regimens. RESULTS: One hundred thirty complete surveys were obtained. Respondents reported a wide range of burdens related to clinical care and surveillance. The majority of patients (85.5%) responded that they would agree to participate in a cancer surveillance RCT if eligible. The most common reason to participate was that they wanted to help future patients. Those that would decline to participate most commonly reported that participating in research would be too much of a burden for them at a time when they are already feeling overwhelmed. However, most patients agreed that cancer research will help doctors better understand and treat cancer. CONCLUSIONS: These results demonstrate that most participants would be willing to participate in an RCT that evaluates varying postoperative cancer surveillance regimens. Participants' motivation for trial participation included altruistic reasons to help future patients and deterrents to trial participation included the overwhelming burden of a cancer diagnosis. These results will help inform the development of patient-centred RCT protocols in sarcoma surveillance research. LEVEL OF EVIDENCE: V.

Extended intralesional curettage preferred over resection-arthrodesis for giant cell tumour of the distal radius.

BACKGROUND: Distal radius giant cell tumour (GCT) is known to be associated with distinct management difficulties, including high rates of local recurrence and lung metastases compared to other anatomic locations. Multiple treatment options exist, each with different outcomes and complications. QUESTIONS/PURPOSES: To compare oncological and functional outcomes and complications following treatment of patients with distal radius GCT by extended intralesional curettage (EIC) or resection-arthrodesis. METHODS: Patients operated on for distal radius GCT were identified from prospectively collected databases at four Canadian musculoskeletal oncology specialty centres. There were 57 patients with a mean age of 35.4 years (range 17-57). Thirteen tumours were Campanacci grade 2, and 40 were Grade 3 (4 unknown). Twenty patients presented with an associated pathologic fracture. There were 34 patients treated by EIC and 23 by en bloc resection and wrist arthrodesis. All resections were performed for grade 3 tumours. The mean follow-up was 86 months (range 1-280). RESULTS: There were a total of 11 (19%) local recurrences: 10 of 34 (29%) in the EIC group compared to only 1 of 23 (4%) in the resection-arthrodesis group (p = 0.028). For the 10 patients with local recurrence following initial treatment by EIC, 7 underwent repeat EIC, while 3 required resection-arthrodesis. The one local recurrence following initial resection was managed with repeat resection-arthrodesis. Six of the 11 local recurrences followed treatment of Campanacci grade 3 tumours, while 4 were in grade 2 lesions and in one case of recurrence the grade was unknown. There were no post-operative complications after EIC, whereas 7 patients (30%) had post-operative complications following resection-arthrodesis including 4 infections, one malunion, one non-union and one fracture (p = 0.001). The mean post-operative Musculoskeletal Tumor Society score was 33.5 in the curettage group compared to 27 in the resection group (p = 0.001). The mean Toronto Extremity Salvage Score was 98.3% following curettage compared to 91.5% after resection (p = 0.006). No patients experienced lung metastasis or death. CONCLUSIONS: EIC is an effective alternative to wide resection-arthrodesis following treatment of distal radius GCT, with the advantage of preserving the distal radius and wrist joint function, but with a higher risk of local recurrence. Most local recurrences following initial treatment by EIC could be managed with iterative curettage and joint preservation. Wide excision and arthrodesis were associated with a significantly lower risk of tumour recurrence but was technically challenging and associated with more frequent post-operative complications. EIC was associated with better functional scores. Resection should be reserved for the most severe grade 3 tumours and recurrent and complex cases not amenable to treatment with EIC and joint salvage. LEVEL OF EVIDENCE: III, retrospective comparative trial.

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

BACKGROUND: 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. QUESTIONS/PURPOSES: (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? METHODS: 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. RESULTS: 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). CONCLUSIONS: 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 OF EVIDENCE: Level II, outcome measurement development.

Early Improvement in Pain and Functional Outcome but Not Quality of Life After Surgery for Metastatic Long Bone Disease.

BACKGROUND: Bone metastases represent the most frequent cause of cancer-related pain, affecting health-related quality of life and creating a substantial burden on the healthcare system. Although most bony metastatic lesions can be managed nonoperatively, surgical management can help patients reduce severe pain, avoid impending fracture, and stabilize pathologic fractures. Studies have demonstrated functional improvement postoperatively as early as 6 weeks, but little data exist on the temporal progress of these improvements or on the changes in quality of life over time as a result of surgical intervention. QUESTIONS/PURPOSES: (1) Do patients' functional outcomes, pain, and quality of life improve after surgery for long bone metastases? (2) What is the temporal progress of these changes to 1 year after surgery or death? (3) What is the overall and 30-day rate of complications after surgery for long bone metastases? (4) What are the oncologic outcomes including overall survival and local disease recurrence for this patient population? METHODS: A multicenter, prospective study from three orthopaedic oncology centers in Quebec, Canada, was conducted between 2008 and 2016 to examine the improvement in function and quality of life after surgery for patients with long bone metastases. During this time, 184 patients out of a total of 210 patients evaluated during this period were enrolled; of those, 141 (77%) had complete followup at a minimum of 2 weeks (mean, 23 weeks; range, 2-52 weeks) or until death, whereas another 35 (19%) were lost to followup but were not known to have died before the minimum followup interval was achieved. Pathologic fracture was present in 34% (48 of 141) of patients. The median Mirel's score for those who underwent prophylactic surgery was 10 (interquartile range, 10-11). Surgical procedures included intramedullary nailing (55), endoprosthetic replacement (49), plate osteosynthesis (31), extended intralesional curettage (four), and allograft reconstruction (two). Seventy-seven percent (108 of 141) of patients received radiotherapy. The Musculoskeletal Tumor Society (MSTS), Toronto Extremity Salvage Score (TESS), Brief Pain Inventory (BPI) form, and Quality Of Life During Serious Illness (QOLLTI-P) form were administered pre- and postoperatively at 2 weeks, 6 weeks, 3 months, 6 months, and 1 year. Analysis of variance followed by post hoc analysis was conducted to test for significance between pre- and postoperative scores. The Kaplan-Meier estimate was used to calculate overall survivorship and local recurrence-free survival. A p value of < 0.05 was considered statistically significant. RESULTS: MSTS and BPI pain scores improved at 2 weeks when compared with preoperative scores (MSTS: 39% ± 24% pre- versus 62% ± 19% postoperative, mean difference [MD] 23, 95% confidence interval [CI], 16-32, p < 0.001; BPI: 52% ± 21% pre- versus 30% ± 21% postoperative, MD 22, 95% CI, 16-32, p < 0.001). Continuous and incremental improvement in TESS, MSTS, and BPI scores was observed temporally at 6 weeks, 3 months, 6 months, and 1 year; for example, the TESS score improved from 44% ± 24% to 73% ± 21% (MD 29, p < 0.001, 95% CI, 19-38) at 6 months. We did not detect a difference in quality of life as measured by the QOLLTI-P score (6 ± 1 pre- versus 7 ± 4 postoperative, MD 1, 95% CI, -0.4 to 3, p = 0.2). The overall and 30-day rates of systemic complications were 35% (49 of 141) and 14% (20 of 141), respectively. The Kaplan-Meier estimates for overall survival were 70% (95% CI, 62.4-78) at 6 months and 41% (95% CI, 33-49) at 1 year. Local recurrence-free survival was 17 weeks (95% CI, 11-24). CONCLUSIONS: Surgical management of metastatic long bone disease substantially improves patients' functional outcome and pain as early as 2 weeks postoperatively and should be considered for impending or pathologic fracture in patients whose survival is expected to be longer than 2 weeks provided that there are no immediate contraindications. Quality of life in this patient population did not improve, which may be a function of patient selection, concomitant chemoradiotherapy regimens, disease progression, or terminal illness, and this merits further investigation. LEVEL OF EVIDENCE: Level II, therapeutic study.

Uterine lipoleiomyosarcoma: Complete medullary compression as presentation of a solitary metastasis.

•A rare case of lipoleiomyosarcoma.•Patient presented at our center with paraplegia caused by a solitary vertebral metastasis of a uterine cancer.•Lobectomy performed for a solitary lung metastasis.

Forearm soft tissue sarcoma: tumors characteristics and oncologic outcomes following limb salvage surgery.

BACKGROUND: Complex anatomy of the forearm may impact on local control and survivals of soft tissue sarcoma. Little is known about characteristics and oncologic outcomes following surgical treatment. METHODS: Demographic and tumor data of 117 patients with forearm soft tissue sarcoma were collected and analyzed. Following limb salvage, survivals, and prognostic factors were studied. RESULTS: Seventy-three patients were males (62%) and 53 (45%) were referred after unplanned excision. Pleomorphic undifferentiated sarcoma was most frequent (45%). The average tumor size was 5.1 cm and grade III histology was mostly identified (53%). With radiotherapy, local recurrence occurs in 8 patients (7%) and 30 patients (24%) developed metastasis. Overall survival, disease free survival, local recurrence free survival, and metastasis free survival were 83%, 74%, 93%, and 74%, respectively. Better survival was found for grade I (80% vs. 60%) and small size (<5 cm) (72% vs. 47%). Large size tumor, extra-compartmental site, extramuscular, and virgin tumor were positive predictors of metastasis. CONCLUSION: Soft tissue sarcomas of the forearm are often referred after unplanned excision. Limb salvage was achieved for most and local recurrence remained low in context of radiotherapy. Metastatic progression remained frequent. Low grade and small size were predictors of survival.

Prophylactic antibiotic regimens in tumour surgery (PARITY): protocol for a multicentre randomised controlled study.

INTRODUCTION: Limb salvage with endoprosthetic reconstruction is the standard of care for the management of lower-extremity bone tumours in skeletally mature patients. The risk of deep postoperative infection in these procedures is high and the outcomes can be devastating. The most effective prophylactic antibiotic regimen remains unknown, and current clinical practice is highly varied. This trial will evaluate the effect of varying postoperative prophylactic antibiotic regimens on the incidence of deep infection following surgical excision and endoprosthetic reconstruction of lower-extremity bone tumours. METHODS AND ANALYSIS: This is a multicentre, blinded, randomised controlled trial, using a parallel two-arm design. 920 patients 15 years of age or older from 12 tertiary care centres across Canada and the USA who are undergoing surgical excision and endoprosthetic reconstruction of a primary bone tumour will receive either short (24 h) or long (5 days) duration postoperative antibiotics. Exclusion criteria include prior surgery or infection within the planned operative field, known colonisation with methicillin-resistant Staphylococcus aureus or vancomycin-resistant Enterococcus at enrolment, or allergy to the study antibiotics. The primary outcome will be rates of deep postoperative infections in each arm. Secondary outcomes will include type and frequency of antibiotic-related adverse events, patient functional outcomes and quality-of-life scores, reoperation and mortality. Randomisation will be blocked, with block sizes known only to the methods centre responsible for randomisation, and stratified by location of tumour and study centre. Patients, care givers and a Central Adjudication Committee will be blinded to treatment allocation. The analysis to compare groups will be performed using Cox regression and log-rank tests to compare survival functions at α=0.05. ETHICS AND DISSEMINATION: This study has ethics approval from the McMaster University/Hamilton Health Sciences Research Ethics Board (REB# 12-009). Successful completion will significantly impact on clinical practice and enhance patients' lives. More broadly, this trial will develop a network of collaboration from which further high-quality trials in Orthopaedic Oncology will follow.

Range of motion of large head total hip arthroplasty is greater than 28 mm total hip arthroplasty or hip resurfacing.

BACKGROUND: Reduced range of motion of the hip has a detrimental influence on lower limb function. Large diameter head total hip arthroplasty may theoretically have a greater potential for restoring normal hip range of motion due to greater head-neck diameter ratio, and hence provide better function compared to conventional or hip resurfacing arthroplasty. METHOD: At minimum one year follow-up, range of motion of the operated and contra lateral hips was clinically assessed using digital photographs and bony landmarks in a clinical comparative study. We assessed if 1) large diameter head total hip arthroplasty (55 patients) restores better hip range of motion compared to 28 mm total hip arthroplasty (50 patients) or hip resurfacing (60 patients) 2) large diameter head total hip arthroplasty achieves same hip range of motion as contra lateral normal hips and 3) hip range of motion correlates with the WOMAC score. FINDINGS: The large diameter head total hip arthroplasty group had significantly greater total arcs of motion (approximately 20°), mostly due to an increase of hip flexion and external rotation, but did not reach normal hip motion. The hip range of motion showed significant correlation with the WOMAC score, especially the flexion arc. INTERPRETATION: The better hip range of motion of large diameter head total hip arthroplasty is likely due to the greater head to neck diameter ratio and hence seems to be the best option to optimize range of hip motion and improve function after hip arthroplasty.