Clinical impact of whole-body MRI in staging and surveillance of patients with myxoid liposarcoma: a 14-year single-centre retrospective study.

OBJECTIVE: To assess the clinical impact of regular whole-body magnetic resonance imaging (WBMRI) surveillance in myxoid liposarcoma patients. METHODS: This was a retrospective cohort study of myxoid liposarcoma patients who underwent at least one WBMRI at our institution between October 2006 and December 2020. The effect of WBMRI on clinical management, namely treatment modification or additional diagnostic investigations was studied. A standardised WBMRI surveillance protocol was instituted in 2015. We compared patient outcomes for the metastatic patients who had and had not received regular WBMRI surveillance and performed survival analysis for both subgroups. RESULTS: Of the 56 patients (60.7% male, median age: 48.1 years) who underwent 345 WBMRI, 17 (30.3%) had metastases, and 168 WBMRI were performed in this group. The median imaging follow-up for the entire cohort was 35 months; the metastatic group had a median follow-up of 42 months. WBMRI changed the clinical management in 13 (76.5%) metastatic patients, with 33 instances of treatment modification. Thirty-five lesions were labelled 'indeterminate,' 16 (45.7%) had additional investigations/interventions, and 4 (11.4%) were confirmed to be metastatic. Twenty-one metastatic lesions were missed initially on WBMRI and confirmed on subsequent WBMRI, of which 5 (23.8%) were clinically significant. The 5-year survival since the detection of metastasis was better in the regular surveillance subgroup (85.7% vs. 45%), but this was not statistically significant (p = 0.068). Five patients (8.9%) developed their first metastasis more than 5 years after diagnosing the primary lesion. CONCLUSION: Regular WBMRI surveillance of myxoid liposarcoma patients considerably impacts clinical management by frequently influencing treatment decisions. CLINICAL RELEVANCE STATEMENT: WBMRI has been recently recommended as an imaging option for the staging and surveillance of myxoid liposarcoma patients. Our study highlights the impact of regular WBMRI surveillance on the clinical management of these patients and how it affects their survival.

Taper Junction Subsidence Occurs in Modular Tumor Endoprostheses: How Concerned Should We Be?

BACKGROUND: Advancements in musculoskeletal oncologic treatment have allowed for longer survival of patients with malignant bone tumors and the associated longer use of tumor endoprostheses in those who have had such reconstructions. Several studies have reported on increased serum metal ions with the use of such implants. Modularity in these implants introduces the risk of taper junction corrosion and subsidence resulting in metal wear particle release that may cause an adverse local tissue reaction or systemic toxicity. Additionally, these implants contain a large surface area of cobalt and chromium. It is unclear whether the source of the increased serum ion levels was due to the taper junction corrosion or the implant itself. To our knowledge, no prior study has reported on taper junction subsidence. QUESTIONS/PURPOSES: In this study we sought (1) to determine survivorship free from radiographic taper junction subsidence in a femoral modular tumor endoprosthesis, and (2) to identify the implant characteristics in the endoprostheses associated with taper subsidence. METHODS: Between January 1996 and February 2020, the senior author performed 150 proximal or distal femur replacements following resections of soft tissue or bone tumors of the thigh and femur. Of those, 6% (9 of 150) of patients were lost to follow-up before 2 years, 25% (37 of 150) could not be analyzed due to absence of plain radiographs during follow-up, and 13% (20 of 150) died before 2 years follow-up, leaving 56% (84 of 150) for analysis in this retrospective study, with a median time for analysis of 14 years (range 2 to 31 years) after the index resection and endoprosthetic reconstruction for patients with distal femur replacements and 5 years (range 2 to 19 years) for patients with proximal femur replacement. Radiographs involving the entire implant were evaluated for the presence or absence of subsidence of the taper junction that was evident and clear to see if present. The association between the number of taper junctions, the length of resection, the number of collapsed junctions, and the time to collapse from the initial surgery were examined using regression analysis. RESULTS: Overall, 14% (12 of 84) patients with a distal femur replacement had radiographic collapse of at least one of the modular tapers. Survivorship free from taper subsidence was 91% (95% CI 86% to 96%) at 10 years and 84% (95% CI 78% to 90%) at 20 years. All patients were in the distal femur replacement group. The median follow-up of patients with subsidence was 15 years (range 5 to 26). Fifty-eight percent (7 of 12) of patients had two junctions involved, 25% (3 of 12) had three junctions, and 17% (2 of 12) had one junction involved. All but one patient had subsidence in a single junction. The median time to subsidence was 15 years (range 4.5 to 24.0 years). The subsidence was progressive in all patients who demonstrated it. The taper junction subsidence was complete in 75% (9 of 12) of patients and partial in 25% (3 of 12). Univariate and multivariable regression analyses did not show that the risk factors we studied were associated with subsidence. Two patients with junction subsidence were revised, one for taper fracture and one during busing exchange for distal junction subsidence. CONCLUSION: Taper damage with late and progressive subsidence of the intervening junction is not uncommon after distal femur replacement. The impact of such a complication is still unknown. Further studies should examine the long-term outcomes and correlate them with metal ion levels. LEVEL OF EVIDENCE: Level III, therapeutic study.

Uncovering the gaps: A systematic mixed studies review of quality of life measures in extremity soft tissue sarcoma.

The health-related quality of life of patients with extremity soft tissue sarcoma (STS) is not precisely captured by current patient-reported outcome measures. Although functional impairment is central to their concerns, multiple sources of distress, emotional restoration, coping strategies, and somatic symptoms are crucial in approaching patients with extremity STS.

LUMiC® endoprosthesis for pelvic reconstruction: A Canadian experience.

BACKGROUND AND OBJECTIVES: The LUMiC® prosthesis was introduced to reduce the mechanical complications encountered with periacetabular reconstruction after pelvic tumor resection. Few have evaluated the outcomes associated with its use. METHODS: A retrospective study from five Orthopedic Oncology Canadian centers was conducted. All patients with a LUMiC® endoprosthesis were included. Their charts were reviewed for surgical and functional outcomes. RESULTS: A total of 16 patients were followed for 28 months (3-60). A total of 12 patients (75%) had a LUMiC® after a resection of a primary sarcoma. Mean surgical time was 555 min. Four patients (25%) had a two-stages procedure. MSTS score was 60.3 preoperatively and 54.3 postoperatively. Patients got a dual mobility bearing and the silver coated implant was used in 7 patients (43.7%). Five patients (31.3%) underwent capsular reconstruction using a fabric. Silver-coating was not found to reduce infection risk (p = 0.61) and capsuloplasty did not prevent dislocation (p = 0.6). Five patients had peroperative complications (31.3%). Eight patients (50%) had an infection including all four with two-stages surgery. Dislocation occurred in five patients (31.3%) whereas no cases of aseptic loosening were reported. A total of 10 patients (62.5%) needed a reoperation. CONCLUSION: LUMiC® endoprosthesis provides low rates of aseptic loosening on medium-term follow-up. Infection and dislocation are common complications but we were unable to show benefits of capsuloplasty and silver-coated implants.

Aggressive Behavior Predictors in Solitary Fibrous Tumor: Demographic, Clinical, and Histopathologic Characteristics of 81 Cases.

BACKGROUND: Solitary fibrous tumor (SFT) is a rare mesenchymal tumor with an intermediate tendency to metastasize. Meningeal hemangiopericytoma (HPC), arising in the meningeal membranes, also is considered an SFT. Although SFT is assumed to show an unpredictable behavior, the authors defined some factors associated with its aggressive behavior. METHODS: This retrospective study was based on the medical records of 81 SFT patients treated surgically, with the median follow-up period of 59 months. The patients were assigned to three histopathologic groups based on the 2016 WHO classification: group 1 (SFT, 29 patients), group 2 (cellular SFT/hemangiopericytoma [HPC], 27 patients), and group 3 (malignant SFT/anaplastic HPC, 25 patients). RESULTS: The SFT histopathologic classification was associated with distant metastasis (DM) (p = 0.007). The multivariate analysis showed that cellular SFT had an independent impact on DM (odds ratio [OR] = 25.42; p = 0.006). Tumor diameter larger than 7.25 cm was correlated with DM (p = 0.010) and the patient's disease-specific death (DSD) (p = 0.007). A 1-cm increase in tumor diameter enhanced the likelihood of metastasis by 1.26 (OR = 1.26; 95% confidence interval [CI], 1.05-1.53). Tumors originating from the central nervous system (CNS) showed a greater tendency toward local recurrence (LR) (p = 0.039) and DM (p = 0.05). Radiotherapy had no association with LR, DM, or DSD. The 10-year disease-specific survival rate was 82.7%. CONCLUSIONS: Tumor size and histopathologic diagnosis are the predictors of SFT's aggressive behavior. Cellular SFTs behave as aggressively as the malignant form of the tumor. A SFT grading based on SFT cellularity would contribute to anticipation of its aggressive behavior.

Which Bone-Modifying Agent is Associated with Better Outcomes in Patients with Skeletal Metastases from Lung Cancer? A Systematic Review and Network Meta-analysis.

BACKGROUND: Lung cancer is one of the most commonly diagnosed cancers and is the leading cause of cancer-related deaths. Metastatic bone disease occurs in 20% to 40% of patients with lung cancer, and these patients often present with pain or skeletal-related events (SREs) that are associated with decreased survival. Bone-modifying agents such as denosumab or bisphosphonates are routinely used; however, to our knowledge, there has been no quantitative synthesis of randomized controlled trial data to determine the most effective pharmacologic treatment of metastatic bone disease because of lung cancer. QUESTIONS/PURPOSES: We aimed to perform a network meta-analysis of randomized trials to identify the bone-modifying agent that is associated with the (1) highest overall survival, (2) longest time to SRE, (3) lowest SRE incidence, and (4) greatest likelihood of pain resolution. METHODS: We conducted our study according to the Preferred Reporting Items for Systematic Reviews and Meta-Analyses protocol and pre-registered the analysis on PROSPERO (ID: CRD42019124364). We performed a librarian-assisted search of MEDLINE, PubMed, EMBASE, Cochrane Library, and Chinese databases including China National Knowledge Infrastructure and Wanfang Data. We included randomized controlled trials reporting outcomes specifically for patients with lung cancer treated with a bisphosphonate or denosumab. SREs included pathologic fractures, spinal cord compression, hypercalcemia of malignancy, or pain resulting in surgical intervention or radiation therapy. We excluded trials exclusively reporting surrogate outcomes such as changes in bone turnover markers. Screening, data extraction, risk of bias evaluation, and Grading of Recommendations Assessment, Development, and Evaluation evaluations were performed in duplicate. We included 131 randomized controlled trials that evaluated 11,105 patients with skeletal metastases from lung cancer. The network meta-analysis was performed using a frequentist model and the R statistical software. Results are reported as relative risks or mean differences, and the I2 value is reported for heterogeneity. The P-score, a measure of ranking certainty that accounts for standard error, is reported for each outcome. Heterogeneity in the network was considered moderate for overall survival and time to SRE, mild for the incidence of SRE, and low for pain resolution. RESULTS: For overall survival, denosumab was ranked above zoledronic acid and estimated to confer a mean of 3.3 months (95% CI 0.3-6.3) of increased overall survival compared with untreated patients (P-score = 89%). For the time to SRE, denosumab was ranked first with a mean of 9.1 additional SRE-free months (95% CI 6.7-11.5) compared with untreated patients (P-score = 99%), while zoledronic acid conferred an additional 4.8 SRE-free months (95% CI 3.6-6.1). Reduction in the incidence of SREs was not different between patients treated with denosumab (relative risk 0.54; 95% CI 0.33-0.87) and those treated with zoledronic acid (relative risk 0.56; 95% CI 0.46-0.67). Patients treated with the combination of ibandronate and systemic therapy were more likely to experience successful pain resolution than untreated patients (relative risk 2.4; 95% CI 1.8-3.2). CONCLUSION: In this comprehensive synthesis of all available randomized controlled trial evidence guiding the pharmacologic treatment of bone metastases from lung cancer, denosumab was ranked above zoledronic acid for overall survival and time to SRE and was not different for reducing the incidence of SRE. Both were superior to no treatment for each of these outcomes. Given this, we encourage physicians to consider the use of denosumab or zoledronic acid in treating this patient population. The combination of ibandronate and systemic therapy was the most effective at reducing pain because of metastases. No cost-effectiveness analysis has yet been performed for denosumab and zoledronic acid on patients with metastatic lung cancer, and this represents an avenue for future research. LEVEL OF EVIDENCE: Level I, therapeutic study.

Outcomes of Cemented Distal Femoral Replacement Using "Line to Line" Technique With All-Polyethylene Tibial Implant for Tumors.

BACKGROUND: Both cemented and cementless stemmed endoprosthetic implants have been used to reconstruct large skeletal defects after tumor resection with similar outcomes. In this study, we examined the oncologic, clinical, and functional outcomes in patients undergoing distal femur replacement using the French paradox technique. METHODS: A total of 125 patients who underwent distal femur replacement between 1990 and 2019 using the line-to-line cementation technique were reviewed. Implant failure was recorded as per Henderson's classification. Functional outcomes were analyzed using the Musculoskeletal Tumor Society and Toronto Extremity Salvage Score scoring systems. The mean follow-up was 84 (1-350) months. RESULTS: Aseptic loosening of the femoral stem was recorded in one patient at 21-years of follow-up. Twenty of 125 patients required bushing exchange for polyethylene wear, all after 10 years. Six tibial bearing component fractures were recorded in four patients while one femoral stem component Morse taper fractured. Two all-polyethylene cemented tibial implants were revised for polyethylene granuloma. Deep surgical site infection occurred in 13 patients, while six patients experienced local recurrence. Kaplan-Meier estimates for implant survival for all-cause revision were 85% at 1 year and 70% at 5 years. These estimates for femur or tibia loosening as an end point were 96% at 10 years and 90% at 15 years. The mean Musculoskeletal Tumor Society and Toronto Extremity Salvage Score scores at the last follow-up were 76% and 74%, respectively. Thirty-five patients died of disease progression. CONCLUSION: The line-to-line cementation technique, used with all-polyethylene tibial implants, demonstrates low incidence of aseptic loosening at medium and long-term follow-ups. LEVEL OF EVIDENCE: III.

Psychological functioning, coping styles and their relationship to appraisal of physical limitations following invasive surgical procedures for soft-tissue sarcoma: A qualitative study.

BACKGROUND AND OBJECTIVES: This study explored psychological functioning and coping styles in adult patients with soft-tissue sarcoma who underwent surgical procedures in a single expert sarcoma medical center in Canada. METHODS: This is a qualitative study with three formats of data collection. The interview guide was based on theoretical health-related quality of life model. We began the investigation with 2 online and 2 in-person focus groups. Four individual semistructured interviews were added to further explore emerging themes. Data were analyzed using inductive thematic networks approach. RESULTS: Twenty-eight adults (13 female, 24-75 years of age) participated. In the domain of psychological functioning we identified three main themes; changes in mood, worry, and body image concerns. In the domain of coping styles, we identified four adaptive coping styles; positive reframing and optimism, finding a purpose, being proactive, and using humor. Among the maladaptive coping styles, we found passive acceptance, and avoidance and denial. CONCLUSIONS: Psychological well-being can be contingent on physical functioning and coping styles in adults with soft-tissue sarcoma. Both psychological and physical function impact quality of life. Patients with more physical limitations, psychological distress and maladaptive coping styles should be monitored for their well-being.

Radiographic, Functional, and Oncologic Outcomes of Cemented Modular Proximal Femur Replacement Using the "French Paradox" Technique.

BACKGROUND: Endoprostheses are frequently used in the management of tumors involving the proximal femur. Aseptic loosening is a common complication that has been linked to the cementing technique. The "French paradox" is well-known cementing technique in the arthroplasty literature. No previous reports have assessed loosening in proximal femur replacements using this technique. We examined rates of femoral stem aseptic loosening in proximal femur replacements, functional outcomes, complications, and oncologic outcomes. METHODS: We conducted a retrospective review of 47 patients who underwent proximal femur replacement between 2000 and 2019. Two reviewers evaluated preoperative and postoperative radiographs using the International Society of Limb Salvage scoring system and Barrack criteria for stem loosening. The acetabulum was evaluated according to the criteria of Baker et al. Functional outcomes were assessed using Musculoskeletal Tumor Society (MSTS) score and Toronto Extremity Salvage Score. The mean follow-up was 44 months. RESULTS: The mean International Society of Limb Salvage scores for the 2 reviewers were 86% ± 6% and 84% ± 6%. The first reviewer graded femoral stem loosening as "possibly loose" in 2 patients, one of whom was graded as possibly loose by the second reviewer. The 2 reviewers found no acetabular erosion in 16 (70%) and 15 (65.4%) patients, respectively. The mean Musculoskeletal Tumor Society score and Toronto Extremity Salvage Score at last follow-up were 61% and 72%, respectively. Twenty complications occurred in 13 patients, and 5 patients experienced local recurrence. CONCLUSION: Despite complications, we showed favorable femoral component survival rates. Cementing the proximal femur prosthesis with tight canal fit and thin cement mantle is a viable option for the short and medium term. LEVEL OF EVIDENCE: III.

Arthroscopic management of intra- and juxta-articular osteoid osteoma of the upper extremity: a systematic review of the literature.

BACKGROUND: Intra- and juxta-articular osteoid osteoma (OO) is rare and can result in irreversible joint damage. Recently, arthroscopic surgery is being used more and more to avoid complications associated with other treatment modalities. METHODS: On October 13, 2019, we conducted a systematic review of the literature available in PubMed and EMBASE regarding the arthroscopic management of OO involving the joints of the upper extremity. Predetermined inclusion criteria were used to include any relevant article published on and before that date for further analysis. Treatment success rate and tumor recurrence rate were considered the primary outcomes in our analysis. RESULTS: Out of 113 studies, 19 met our inclusion criteria. Of the 32 reported cases in these 19 articles, ten involved the shoulder joint, 19 involved the elbow joint and three involved the wrist joint. Overall treatment success rate was 93.8%. Tumor recurrence rate was 0.0%. No postoperative complications (0.0%) were reported among cases involving the shoulder joint. Two out of 24 (8.3%) patients with elbow OO failed arthroscopic treatment due to incomplete excision, and two (4%) experienced minor complications. Among the three cases of wrist OO, two (66.7%) patients had residual postoperative pain and decreased hand grip strength. CONCLUSION: Arthroscopic management of OO of the upper extremity joints is highly successful and results in no tumor recurrence; however, there is a risk of incomplete resection in areas more difficult to access by arthroscopy.

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.

Venous Thromboembolism in Patients with Sarcoma: A Retrospective Study.

BACKGROUND: Little has been published about the association of venous thromboembolism (VTE) and sarcoma. In this study, we sought to identify clinical features of patients with sarcoma presenting at least one VTE episode. METHODS: Our study was a retrospective case-control study of a single-institution database with univariate and multivariate analysis using chi-square and Student's t test. A p value less than .05 was considered significant. RESULTS: The overall incidence of VTE in patients with sarcoma was 7.9%. Predictive factors identified by multivariate analysis were metastatic disease and administration of chemotherapy. It was not statistically possible to correlate the risk of VTE with specific sarcoma subtypes, but observations suggested malignant peripheral nerve sheath tumor, osteosarcoma, and liposarcoma as having the highest propension. CONCLUSION: VTE is not infrequent in patients with sarcoma. Adoption of common guidelines for cancer-associated thrombosis is recommended.

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.

The surgical outcome and recurrence rate of tenosynovial giant cell tumor in the elbow: a literature review.

BACKGROUND: Tenosynovial giant cell tumor (TSGCT) is a rare proliferative disorder of the synovium. Because of its aggressive nature and recurrence potential, treatment of TSGCT involves surgical resection with or without synovectomy. There is currently a paucity of literature describing the surgical management of TSGCT at the elbow. The aim of this study was to evaluate clinical outcomes and recurrence rates following open and arthroscopic excision of TSGCT in the elbow. METHODS: Electronic databases were searched for relevant articles relating to surgical management of TSGCT of the elbow. We included all patients who received surgical treatment for TSGCT, with no age limitations. We excluded any nonsurgical treatment studies. Seventy-seven articles were identified for screening, and a total of 27 patients from 24 studies were included for the review. RESULTS: The patients' mean (standard deviation [SD]) age was 40.3 (21.7) years, and the most common presenting symptoms included pain (18/27, 66.7%), swelling (19/27, 70.4%), and decreased range of motion of the elbow (9/27, 33.3%). The majority of patients underwent open excision with or without synovectomy (23/27, 85.1%). Of those undergoing open procedures, 16 (16/23,69.6%) had diffuse TSGCT and 14 (14/23, 60.9%) remained symptom free for a mean (SD) follow-up of 38.9 (25.4) months. Four patients (4/27, 14.8%) were treated arthroscopically, all of whom had diffuse disease. CONCLUSIONS: Our review found that open synovectomy appears to be an effective treatment for both localized and diffuse TSGCT in the elbow, and arthroscopic synovectomy is emerging as a method of surgical management for diffuse TSGCT. However, because of the limited number of patients undergoing surgery for TSGCT, further studies are needed to make a definite conclusion.

Arthroscopic Management of Osteoid Osteoma of the Ankle Joint: A Systematic Review of the Literature.

Osteoid osteomas (OOs) are rare benign bone tumors that may occur in various joints including the ankle. These tumors are generally removed via open surgical excision or radiofrequency ablation. However, when they occur intra-articularly, these treatments are more difficult to perform because of more difficult access and the increased risk of damaging articular cartilage. Therefore, some have advocated for the use of arthroscopy to treat these cases. This systematic review aims to investigate the safety and efficacy of arthroscopic treatment for intra-articular OO of the ankle. Using Medline and Embase, we systematically reviewed the literature as of May 31, 2017. All articles published on and before that date were reviewed by 2 independent reviewers. Seventeen articles containing a total of 27 cases were included in the review. Most reported cases were in the talar neck, followed by the distal tibia. Of all the cases, only 2 recurrences were reported (in the same patient), and no complications were reported. Therefore, these cases demonstrate arthroscopic excision of intra-articular OO of the ankle as a safe and effective alternative to open surgical excision and radiofrequency ablation, with a success rate of 96%. However, all articles found were case studies or small case series owing to the rarity of this disease. In the future, analyses of case series with larger case collections should be performed.

Probabilistic classification of tumour habitats in soft tissue sarcoma.

The purpose of this work is to propose a method to characterize tumour heterogeneity on MRI, using probabilistic classification based on a reference tissue. The method uses maps of the apparent diffusion coefficient (ADC), T2 relaxation, and a calculated map representing high-b-value diffusion-weighted MRI (denoted simDWI) to identify up to five habitats (i.e. sub-regions) of tumours. In this classification method, the parameter values (ADC, T2 , and simDWI) from each tumour voxel are compared against the corresponding parameter probability distributions in a reference tissue. The probability that a tumour voxel belongs to a specific habitat is the joint probability for all parameters. The classification can be visualized using a custom colour scheme. The proposed method was applied to data from seven patients with biopsy-confirmed soft tissue sarcoma, at three time-points over the course of pre-operative radiotherapy. Fast-spin-echo images with two different echo times and diffusion MRI with three b-values were obtained and used as inputs to the method. Imaging findings were compared with pathology reports from pre-radiotherapy biopsy and post-surgical resection. Regions of hypercellularity, high-T2 proteinaceous fluid, necrosis, collagenous stroma, and fibrosis were identified within soft tissue sarcoma. The classifications were qualitatively consistent with pathological observations. The percentage of necrosis on imaging correlated strongly with necrosis estimated from FDG-PET before radiotherapy (R2  = 0.97) and after radiotherapy (R2  = 0.96). The probabilistic classification method identifies realistic habitats and reflects the complex microenvironment of tumours, as demonstrated in soft tissue sarcoma.

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.

Early detection of metastases using whole-body MRI for initial staging and routine follow-up of myxoid liposarcoma.

OBJECTIVE: To define the role of whole-body MRI (WBMRI) for initial staging and routine follow-up of myxoid liposarcoma (MLS). MATERIALS AND METHODS: A retrospective review of all the patients with MLS who underwent WBMRI for initial staging and routine follow-up at our institution between October 1, 2006, and September 30, 2016 was performed. Patient demographics, clinical presentation, imaging findings, tumor histology, and occurrence and location of metastatic disease were recorded. Thirty-three patients who underwent a total of 150 WBMRI examinations were included in the study. RESULTS: Nine patients (27%) were diagnosed with metastases between 0 and 60 months (median 10; interquartile range, 7-13) from the diagnosis of the primary tumor. The initial site of metastatic disease was extrapulmonary in all patients. Only two patients developed pulmonary metastases, which were diagnosed by CT chest 9 and 29 months after the diagnosis of extrapulmonary metastases. The first metastasis was diagnosed by WBMRI in seven patients (78%), by thoracic CT in one patient, and by abdominal CT in one patient. Eight of nine patients (89%) were asymptomatic at the time of diagnosis of the metastases. In seven patients (78%), WBMRI demonstrated metastases included within the field of view of, but occult on a contemporaneous CT scan. CONCLUSION: Our 10-year institutional experience demonstrates that WBMRI facilitates early detection of extrapulmonary MLS metastases before the onset of clinical symptoms and pulmonary metastases. WBMRI also depicts extrapulmonary metastases that are occult on CT scans. The current surveillance strategies are insufficient for screening for extrapulmonary MLS metastases.

Limb-sparing surgery with vascular reconstruction for malignant lower extremity soft tissue sarcoma.

BACKGROUND: The standard of care for lower extremity soft tissue sarcoma (STS) is limb-sparing surgery. A small subset of these patients will require concomitant vascular reconstruction to ensure adequate resection and to preserve limb viability and function. The aim of this study was to evaluate outcomes in these patients with respect to wound healing and postoperative functional status. METHODS: Outcomes for a total of 154 patients treated for malignant lower extremity STS during an 8-year period between 2005 and 2013 were entered in a prospective registry. Treatment was by medical management in 3 patients (2%), limb-sparing surgery with vascular reconstruction (LSVR) in 9 patients (6%), and limb-sparing surgery without vascular reconstruction (LS) in 142 patients (92%). The registry and patient records and the intraoperative records were consulted to determine the primary outcomes of patient survival and time for complete wound healing. The functional status of patients was assessed using the Musculoskeletal Tumor Society (MSTS) functional assessment score before surgery and at 6 and 12 months after surgery. RESULTS: Mean follow-up time was 74.7 months for the LSVR group and 53.4 months for the LS group. The mean time to complete wound healing was significantly longer in LSVR vs LS patients (88 days vs 34 days, respectively; P = .002), and overall survival was lower in LSVR patients (P = .01). Seven of the 9 LSVR patients required a total of 12 additional procedures to achieve wound healing, including 9 procedures to drain seromas (incision and drainage) with vacuum-assisted closure in 4 cases. Plastic surgery intervention was required in three patients, including one skin graft, one gracilis pedicle flap, and one vertical rectus abdominis myocutaneous flap. There was no significant difference in the mean MSTS scores preoperatively, at 6 months, and at 1 year after surgery between the two groups (27, 25, and 29 for LSVR vs 28, 31, and 31 for LS, respectively; P = .63, .11, and .67, respectively). CONCLUSIONS: The need for vascular reconstruction during limb-sparing surgery for lower extremity malignant STS is rare in a high-volume sarcoma center. Overall survival was lower in these patients, and the time to complete wound healing is prolonged and requires multiple secondary interventions. However, postoperative functional status as assessed by the MSTS is acceptable and comparable to that of patients not requiring vascular reconstruction.

The epigenetic regulation of SOX9 by miR-145 in human chondrosarcoma.

Chondrosarcoma is the most common primary bone malignancy in the adult population with a high rate of pulmonary metastasis. Chondrosarcoma is managed with surgical excision as the tumors do not respond well to conventional chemotherapy or radiation therapy. Thus, there exists a dire need to develop systemic treatment options to target chondrosarcoma cells for metastatic spread. We hypothesized that the expression of miR-145 is low in chondrosarcoma, leading to decreased transcriptional control of SOX9 (the master regulator of chondrogenesis), and downstream activation of the transcription factor ETV5. We have previously shown that ETV5 activates MMP-2 expression in chondrosarcoma, which in turn increases local bone matrix resorption. In this study, we confirm high expression of SOX9 in human chondrosarcoma using real-time PCR, Western blotting, and immunofluorescence. An ETV5 promoter-reporter plasmid was transfected into chondrosarcoma cells to determine if SOX9 directly regulates the expression of ETV5. Co-transfection of the ETV5 promoter-plasmid with SOX9 lentivirus significantly increased the luciferase activity derived from the ETV5 promoter, from which the regulatory relationship between SOX9 and ETV5 is established. MiR-145 was found to be down-regulated in chondrosarcoma cell lines, patient samples, and further confirmed with a public sarcoma database. After stable miR-145 lentiviral transfection, the subsequent mRNA expression levels of SOX9, ETV5, and MMP-2 were significantly decreased in chondrosarcoma cells. The results generated by this study may have important clinical significance in the treatment of patients with chondrosarcoma in that targeted miRNA may have the potential to downregulate the upstream activators of proteases such as MMP-2.