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.

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.

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.

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.

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.

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.

Hip arthroscopy for the management of osteoid osteoma of the acetabulum: a systematic review of the literature and case report.

BACKGROUND: Intra-articular osteoid osteoma (OO) causes irreversible joint damage. Its treatment of choice is radiofrequency ablation (RFA); however, some areas of the acetabulum are hard to access. Therefore, hip arthroscopy was used to treat this tumor. We aim to systematically review the literature with regards to arthroscopic management of acetabular OO, and to report a further case in which hip arthroscopy was used for treatment. METHODS: PubMed and EMBASE were searched for articles relevant to the arthroscopic management of acetabular OO on December 2, 2014. All articles published on and before that date were reviewed, and studies which met our pre-determined inclusion criteria were included. Articles screening and data abstraction were done by two reviewers independently. We also presented a 31-year-old man with acetabular OO who underwent hip arthroscopy for the management of his tumor after failing to respond to medications and computed tomography scan (CT)-guided RFA. RESULTS: The initial search revealed 14 studies, of which ten met our inclusion criteria. A total of ten patients underwent hip arthroscopy for the management of acetabular OO. Only two patients were females, and the patients' age ranged from 7 to 47 years. Two patients underwent arthroscopic guided-RFA of the lesion, while the rest underwent excision. The follow-up period ranged from 6 months to 2 years. Success rate was 100 %, and no recurrence was reported. Minor complications (transient impotence and perineal numbness) developed in one patient (10 %). Arthroscopic-guided RFA failed to eliminate the tumor in our additional case. A second trial of CT-guided RFA was successful in treating the patient's condition. CONCLUSIONS: Hip arthroscopy is an effective and safe option for the management of acetabular OO, with success rate exceeding 90 %. Studies of higher level of evidence are required.

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.

Iterative curettage is associated with local control in giant cell tumors involving the distal tibia.

BACKGROUND: The distal tibia is an unusual location for a giant cell tumor (GCT). Treatment choices are unclear because of their rarity, the anatomy of the ankle, and difficulties associated with reconstruction. QUESTIONS/PURPOSES: We assessed: (1) the treatment modalities used by participating Canadian bone tumor centers for distal tibia GCTs; (2) the incidence of local recurrence and their management; and (3) patients' function after treatment. METHODS: A prospective tumor database served to identify all 31 patients with primarily treated distal tibia GCTs between 1991 and 2010. We extracted patients and tumor characteristics, treatment modalities for initial and recurrent tumors, and the Musculoskeletal Tumor Society (MSTS) and Toronto Extremity Salvage (TESS) scores. The median followup was 58 months (range, 24-192 months). RESULTS: Extended curettage was the only modality of treatment for all patients including all subsequent local recurrences. Nine had local recurrence, three of which had a second local recurrence; one had a third recurrence. Ultimately all patients were in remission at last followup. The local recurrence rate was 29% and appeared higher compared with recent series of all anatomic sites. The mean final MSTS and TESS scores were 91% (range, 71%-100%) and 88% (range, 35%-100%), respectively. CONCLUSIONS: Extended curettage was the unique modality of surgical treatment for all tumors. We found the incidence of local recurrence higher than that reported for other locations but recurrences were manageable with repeated curettage. Complications and function appeared better than those reported for series of ankle fusion or reconstruction for bone tumors.

Osteosarcoma Around a Ceramic-on-Ceramic Total Hip Arthroplasty.

Sarcoma arising at the site of a total hip arthroplasty (THA) is uncommon. We present a case report of a patient diagnosed with an osteosarcoma around a ceramic-on-ceramic THA and a narrative literature review of sarcomas around THA. A search of PubMed MEDLINE was performed from inception. Our case report was included in the analysis. A total of 13 studies were included in the review. We report the first case of a sarcoma around a ceramic-on-ceramic hip implant. All cases in the literature reported poor outcomes with an average time from index THA to diagnosis of 9.3 ± 8.2 years. Sarcomas around THA are extremely rare. Despite the rarity of the diagnosis, osteosarcoma must be considered in the differential diagnosis when investigating a periprosthetic mass.

Primary Sarcomas of the Spine: A Systematic Review and Pooled Data Analysis.

STUDY DESIGN: A systematic review of the literature and pooled data analysis of treatment outcomes of primary sarcomas of the spine. OBJECTIVE: To examine the current literature and treatment options for primary sarcomas of the spine. SUMMARY OF BACKGROUND DATA: A paucity of literature exists on treatment outcomes of primary sarcomas of the spine. MATERIALS AND METHODS: Two authors searched PubMed to identify articles for review, and a pooled data analysis was performed to determine overall survival for each type of surgical resection on spine sarcomas. RESULTS: In total, 1776 articles were identified, and 11 met our inclusion criteria for review. In chondrosarcoma, overall survival was significantly higher with en bloc excision when compared with piecemeal resection (HR for piecemeal resection: 4.11; 95% CI: 2.08-8.15). Subgroup analysis showed that the addition of radiation therapy after piecemeal excision increased overall survival to 60 months from 48 months with piecemeal excision alone. In osteosarcoma, there was no significant difference in overall survival between en bloc and piecemeal resection (HR for piecemeal resection: 1.76; 95% CI: 0.776-3.99). In Ewing's sarcoma, overall survival was significantly higher when a successful en bloc resection was achieved and coupled with chemotherapy and radiation therapy for local control (HR for piecemeal resection: 7.96; 95% CI: 2.12-20.1). Interestingly, when a successful en bloc resection could not be achieved, chemotherapy and radiation therapy alone had significantly higher survival than piecemeal resection (HR for piecemeal resection: 2.63; 95% CI: 1.01-6.84). A significantly higher number of local recurrences were associated with the piecemeal resection group in all types of spine sarcomas. CONCLUSION: This review and pooled data seem to favor en bloc excision for local control as the treatment of choice in primary sarcomas of the spine.

Patient-physician agreement on function and pain is associated with long-term outcomes in sarcoma: findings from a longitudinal study.

PURPOSE: We aimed to describe the level of agreement between patients and physicians on the ratings of daily functioning and pain in a cohort of sarcoma patients and assess how (dis)agreement and its change over time predicted patient-reported outcomes in survivorship. METHOD: We performed secondary analysis of longitudinal data from a sarcoma-specialty clinic in Montreal, Canada. Demographics, clinical characteristics and patient-physician agreement were summarized descriptively. Linear mixed models were used to assess the effects of time, baseline agreement, change in agreement over time, interaction of time and change in agreement and 12-month daily functioning, quality of life, and fatigue. RESULTS: Data were available for 806 patients (57.7% male, x̄ = 53.3 years) who completed at least one questionnaire. Patient-physician disagreement was common on the level of function (43.4%) and pain (45.7%). Baseline physician-patient agreement was associated with better 12-month outcomes. Improvement in agreement on function over time was significantly associated with daily functioning (F(2, 212) = 3.18, p = 0.043) and quality of life (F(2, 212) = 3.17, p < 0.044). The pattern was similar though less pronounced for the agreement on pain. CONCLUSIONS: Our study offers novel insights into the importance of patient-physician agreement and communication's role in long-term patient-reported outcomes in sarcoma. IMPLICATIONS FOR CANCER SURVIVORS: The results emphasize the importance of mutual understanding of symptoms and patients' needs and suggest that further consultation in cases of discordance of ratings and opinions might be beneficial for optimal survivorship.

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 clinical and functional outcome for patients with radiation-induced soft tissue sarcoma.

BACKGROUND: Radiation-induced soft tissue sarcomas (RI-STS) are rare, and it is believed that they are associated with a poor prognosis.The authors of this report compared the clinical and functional outcomes of adults who had extremity RI-STS with the outcomes of adults with sporadic STS. METHODS: Forty-four patients who were diagnosed with RI-STS from 1989 to 2009 were identified from 4 prospectively collected databases. Patient demographics, surgical and adjuvant treatment parameters, and oncologic and functional outcomes were evaluated. RESULTS: The median latent period from irradiation of the primary condition to RI-STS diagnosis was 16 years. The median radiotherapy dose used for the index condition was 45 gray. The median age at RI-STS diagnosis was 56 years. The most common primary diagnoses were breast cancer (36.4%) and lymphoma (34.1%). The most common RI-STS histologies were malignant fibrous histiocytoma (36.4%) and angiosarcoma (18.2%). Forty-two patients underwent surgery, 13 patients received adjuvant radiotherapy, and 8 patients received adjuvant chemotherapy. Systemic metastases occurred in 50% of treated patients (n = 21), and 26% (n = 11) developed local recurrence, the risk of which was lower among patients who received reirradiation (P = .043). The 5-year disease-free interval (DFI) and overall survival (OS) rates for patients with RI-STS who presented without metastasis were 36% and 44%, respectively. Patients who had International Union Against Cancer TNM stage III RI-STS had a significantly worse DFI compared with patients who had stage III sporadic STS (multivariate analysis, P = .051). Eighteen patients with RI-STS underwent functional assessment after surgery, and their results were comparable to those of patients with sporadic STS. CONCLUSIONS: Despite aggressive surgical treatment, patients who have RI-STS remain at greater risk of both local and systemic recurrence compared with patients who have sporadic STS, but they can anticipate similar functional outcomes. Reirradiation can be relatively safe and effective if used properly.

Primary perivascular epithelioid cell neoplasm (PEComa) of bone: report of two cases and review of the literature.

Primary perivascular epithelioid cell neoplasms (PEComas) of bone are rare mesenchymal tumors. Histologically, they are composed predominantly of perivascular epithelioid cells and have the capacity to metastasize. PEComas have been reported within intra-abdominal and intra-pelvic organs. To the best of our knowledge, only seven primary PEComas of bone have been described in the English literature. We present two cases of PEComa of bone, one arising from the distal fibula and one from the acetabulum. Both were treated by surgical excision and one also received adjuvant chemotherapy.