Chronic hepatitis C virus infections in Switzerland in 2020: Lower than expected and suggesting achievement of WHO elimination targets.

In this multi-method study, we investigated the prevalence of chronic infections with the hepatitis C virus (HCV) in Switzerland in 2020, and assessed Switzerland's progress in eliminating HCV as a public health problem by 2030 with regard to the World Health Organization (WHO) criteria targeting infections acquired during the preceding year ('new transmissions') and HCV-associated mortality. Based on a systematic literature review, the reappraisal of a 2015 prevalence analysis assuming 0.5% prevalence among the Swiss population and data from many additional sources, we estimated the prevalence among subpopulations at increased risk and the general population. For new transmissions, we evaluated mandatory HCV notification data and estimated unreported new transmissions based on subpopulation characteristics. For the mortality estimate, we re-evaluated a previous mortality estimate 1995-2014 based on new data on comorbidities and age. We found a prevalence of ≤0.1% among the Swiss population. Discrepancies to the 2015 estimate were explained by previous (i) underestimation of sustained virologic response numbers, (ii) overestimation of HCV prevalence among PWID following bias towards subgroups at highest risk, (iii) overestimation of HCV prevalence among the general population from inclusion of high-risk persons and (iv) underestimation of spontaneous clearance and mortality. Our results suggest that the WHO elimination targets have been met 10 years earlier than previously foreseen. These advancements were made possible by Switzerland's outstanding role in harm-reduction programmes, the longstanding micro-elimination efforts concerning HIV-infected MSM and nosocomial transmissions, little immigration from high-prevalence countries except Italian-born persons born before 1953, and wealth of data and funding.

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.

Diagnostic utility of molecular and cytogenetic analysis in lipoblastoma: a study of two cases and review of the literature.

AIMS: Lipoblastoma is a benign neoplasm of embryonic white fat tissue that results from the proliferation of primitive adipocytes, in which histological features can be ambiguous. In order to discriminate between lipoblastoma and other lipogenic and lipomatous tumours, we studied chromosomal alterations and protein expression in two cases of lipoblastoma in infants. METHODS AND RESULTS: Standard cytogenetic analysis, fluorescence in-situ hybridization, array comparative genomic hybridization and Western blotting allowed us to demonstrate the presence of chromosome abnormalities involving the 8q11-13 region containing the pleomorphic adenoma gene 1 (PLAG1), which are classically reported in lipoblastoma, and aberrant expression of PLAG1. CONCLUSIONS: This report illustrates two different tumorigenic pathways implicating PLAG1 in lipoblastoma: amplification through multiple copies of a small marker chromosome derived from chromosome 8, and a paracentric inversion of the long arm of chromosome 8. Both these anomalies induced aberrant expression of PLAG1, emphasizing the role of PLAG1 in tumorigenesis. The aberrant expression of PLAG1 protein has been hypothesized, but this is the first report to demonstrate its occurrence in lipoblastoma.

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.

Effectiveness of pamidronate as treatment of symptomatic osteonecrosis occurring in children treated for acute lymphoblastic leukemia.

BACKGROUND: Osteonecrosis (ON) is a severe complication of acute lymphoblastic leukemia (ALL) treatments. Recent studies suggest that bisphosphonates might reduce pain and loss of motor function in patients with ON. We assessed the effects of pamidronate compared to standard care in patients with symptomatic ON (sON) and studied whether steroids might be continued after diagnosis of ON in some patients. METHODS: We evaluated 17 patients with sON as complication of primary ALL treatment between 2000 and 2008. Fourteen patients were treated with pamidronate. Mobility and pain control were monitored in all patients. Affected joints were classified by magnetic resonance imaging (MRI) at ON diagnosis and after 6-72 months. RESULTS: Out of 220 patients with ALL, 17 (7.7%) patients developed sON. The median age at ALL diagnosis was 11 years (range: 2.7-16.6 years) and sON occurred a median of 13.4 months (range: 2.5-34 months) after ALL diagnosis. Affected joints were hip, knee and ankle. MRI scans showed 7 severe, 4 moderate, and 6 mild ON lesions. Fourteen patients showed improvement in pain (77% of patients) and motor function (59% of patients), even though corticoids were reintroduced in 4 patients. MRI demonstrated improvement, stability or worsening in 6, 3, and 5 cases, respectively. CONCLUSIONS: Pamidronate seems to be effective in the management of pain and motor function recovery in sON. Further studies are needed to provide evidence as to whether bisphosphonates can be recommended for the treatment or the prevention of ON in childhood ALL patients.

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.

Myxoid\round cell liposarcoma (MRCLS) revisited: an analysis of 418 primarily managed cases.

BACKGROUND: Objectives of this study were to evaluate oncologic outcomes and to provide guidelines for the management of primary myxoid (MLS) and round cell liposarcoma (RCLS). METHODS: A multicenter, retrospective study of 418 cases of MRCLS primarily managed by Canadian multidisciplinary sarcoma teams. RESULTS: Study included 418 cases (MLS: 311 patients and RCLS: 107; >5% round cell) with a median age of 45 years and a median follow-up of 5.2 years. Median tumor size was 10 cm, and 81% were deep and 90% were in lower limb. The majority of patients underwent surgical resection and radiotherapy, with a small percentage (6%) receiving chemotherapy. The overall 10-year local control rate was 93% with no differences between MLS and RCLS. Radiotherapy was significant in preventing local relapse and reducing tumor diameter (median=18%) and improving microscopic margin status, but did not impact survival. Radiotherapy and the margin status were independent predictors of local recurrence. The 5- and 10-year metastatic-free survivals were 84 and 77% respectively for MLS and 69 and 46% for RCLS. The initial site of metastasis was found in multiple locations (34%) and bone involvement was frequent (40%) with predilection for spine (79%). Round cell percent (>5%) and tumor diameter (>10 cm) correlated with increased risk for metastasis and death. CONCLUSIONS: MLS and RCLS showed different metastatic risk but equally good local control. Radiotherapy was effective in preventing local recurrence and should be delivered as neoadjuvant. New staging strategies are to be defined to account for the unusual metastatic pattern.

Malignant perineurioma (malignant peripheral nerve sheath tumor with perineural differentiation).

The great majority of malignant peripheral nerve sheath tumors (MPNST) exhibit Schwannian differentiation. In recent years, a subset of perineurial MPNST (malignant perineurioma) has been identified based on their histologic, immunohistochemical and ultrastructural features. Immunopositivity for epithelial membrane antigen (EMA), glut-1 and claudin-1, is characteristic. Such tumors must be distinguished from benign perineurioma and a variety of atypical or malignant soft tissue tumors featuring EMA positivity. Herein, we report a perineurial MPNST involving the buttock of a 42-year-old woman. Nerve involvement was noted. The clinicopathologic features of reported examples are ummarized and key differential diagnoses are discussed.

Bilateral Pseudoarthrodesis: A Case Report of Complex Bilateral Total Knee Replacement for a Severe Hemophilia A Patient with Inhibitors.

CASE: We present a severe hemophilia A patient with high titers of inhibitors presenting stage IV knee arthropathy with functional bilateral arthrodesis. On presentation, his mode of ambulation was to project himself forward without the benefit of any significant ankle motion. Total knee arthroplasty was performed on both knees and allowed significant improvement in the range of motion of both knees from 5° to 100°. CONCLUSION: Although hemophilic patients with inhibitors can represent complex cases, successful outcomes can be achieved in a multidisciplinary team setting. However, we would recommend performing this type of surgery at an earlier stage when less extensive muscle and tendon release is required.

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.

Local recurrence after initial multidisciplinary management of soft tissue sarcoma: is there a way out?

BACKGROUND: Multimodality treatment of primary soft tissue sarcoma by expert teams reportedly affords a low incidence of local recurrence. Despite advances, treatment of local recurrence remains difficult and is not standardized. QUESTIONS/PURPOSES: We (1) determined the incidence of local recurrence from soft tissue sarcoma; (2) compared characteristics of the recurrent tumors with those of the primary ones; (3) evaluated local recurrences, metastases and death according to treatments; and (4) explored the relationship between the diagnosis of local recurrence and the occurrence of metastases. METHODS: From our prospective database, we identified 618 soft tissue sarcomas. Thirty-seven of the 618 patients (6%) had local recurrence. Leiomyosarcoma was the most frequent diagnosis (eight of 37). The mean delay from original surgery was 22 months (range, 2-75 months). Mean size was 4.8 cm (range, 0.4-28.0 cm). Median followup after local recurrence was 16 months (range, 0-98 months). RESULTS: Recurrent tumors had a tendency toward becoming deeper seated and higher graded. Nineteen of the 37 patients with recurrence underwent limb salvage (nine free flaps) and six had an amputation. Twenty-two (59%) had metastases, including 10 occurring after the local recurrence event at an average delay of 21 months (range, 1-34 months). Six patients developed additional local recurrences, with no apparent difference in risk between amputation (two of six) and limb salvage (four of 19). CONCLUSIONS: Patients with a local recurrence of a soft tissue sarcoma have a poor prognosis. Limb salvage and additional radiotherapy remain possible but with substantial complications. Amputation did not prevent additional local recurrence or death.

Case series. Soft-tissue sarcoma of the foot.

BACKGROUND: We reviewed cases of soft-tissue sarcoma of the foot to gain insight into the presentation, treatments and outcomes for this rare disease and to determine whether limb-salvage surgery yields reasonable functional and oncological outcomes. METHODS: We reviewed the cases of 16 patients treated by 2 of us (R.T. and M.I.) for soft-tissue sarcoma of the foot over a 15-year period. We extracted the following information from each patient's medical record: disease status at presentation, prior treatment, histological diagnosis, American Joint Committee on Cancer (AJCC) stage, details of treatment, oncological outcome and functional outcome. Functional outcome was assessed with the Toronto Extremity Salvage Score (TESS) and the Musculoskeletal Tumor Society (MSTS 1987). RESULTS: Follow-up averaged 6 (range 2­15) years. Eight patients presented after unplanned excision. Histological diagnosis was synovial sarcoma for 7 of 16 patients.The tumours were evenly distributed among the hindfoot, midfoot and forefoot. Most patients (n = 13) presented with AJCC stage II or III disease. Amputation was necessary for 3 patients, whereas limb salvage was possible for the other 13. Free tissue transfer (n = 9) and radiation therapy (n = 12) were used in most cases. Surgical margins were microscopically positive in 4 of the 13 patients treated with limb salvage.Local disease recurred in 2 patients. Lung metastases occurred in 4 patients. At last follow-up, 11 of 16 patients were alive without disease, 2 with disease and 3 had died of their disease. Functional assessment with MSTS 1987 and the TESS averaged 28%and 90%, respectively, after limb salvage. CONCLUSION: In this series, we found that, first, patients frequently presented after unplanned excision, and this may have led to worse oncological outcomes compared with patients who presented primarily. Second, limb salvage was usually possible, but it required accepting marginal resections, relying on free tissue transfer to obtain coverage and using radiation therapy to obtain local control. Third, this combination yielded an acceptable local control rate and very good functional outcomes.

Outcomes in patients with popliteal sarcomas.

BACKGROUND: Soft-tissue sarcoma involving the popliteal fossa remains challenging because it is difficult to achieve wide margins with limb salvage in this location. Adjuvant therapy is frequently necessary, and limb function can be adversely affected. We reviewed our experience with these tumours. METHODS: Our prospective tumour database served to identify all patients with popliteal sarcomas treated at the McGill University Health Centre and the Maisonneuve-Rosemont Hospital between 1994 and 2005. We assessed oncologic and functional outcomes as well as complications. RESULTS: Our study included 18 patients (12 women and 6 men). The mean age was 54 (range 16-84) years. The mean duration of follow-up was 55 (range 4-126) months. Frequent histologic diagnoses were liposarcoma (n = 6), synovial sarcoma (n = 4) and leiomyosarcoma (n = 3). Tumour size varied from 2 to 21 cm (median 11 cm). American Joint Committee on Cancer staging was as follows: 4 patients had stage IIa disease, 3 patients had stage IIb, 10 patients had stage III and 1 patient had stage IV disease. Treatment consisted of limb salvage in 15 patients and amputation in 3. Fourteen patients had radiotherapy, 4 had chemotherapy, and 3 needed partial sciatic nerve resection. Margins were negative in 7 of 18 patients and microscopically positive in 11 patients. Complications included wound infections in 3 patients and thrombophlebitis in 2 patients. Of the patients undergoing limb-salvaging procedures, 1 experienced local recurrences after limb salvage (7%), and 5 experienced lung metastases (20%). Local recurrence was always associated with positive margins, whereas metastases occurred only in patients without local recurrence. The mean Musculoskeletal Tumor Society 1987 score was 33 (range 24-35). The mean Toronto Extremity Salvage Score results was 82.4 (range 63.8-100). At latest follow-up, 6 patients had died of disease, 1 was alive with disease, and 11 (61%) patients remained free of disease. CONCLUSION: Despite the high rate of microscopically positive margins, the local recurrence rate was 7%. Amputation did not prevent death. We found function to be good to excellent in most patients who had limb-salvaging surgery.

Swiss multicenter study evaluating the efficacy, feasibility and safety of peginterferon-alfa-2a and ribavirin in patients with chronic hepatitis C in official opiate substitution programs.

BACKGROUND: Though patients in opiate substitution programs are commonly infected with HCV, due to safety and efficacy concerns, they are rarely treated with interferon and ribavirin. METHODS: In a multicenter study, HCV-infected patients in opiate maintenance treatment programs received 180 microg pegylated interferon-alfa-2a once weekly, plus daily ribavirin for 24 weeks (genotypes 2, 3), or 48 weeks (genotypes 1, 4). RESULTS: Of the 67 patients enrolled, 31 (46%) had HCV genotypes 1 or 4, and 36 (54%) had genotypes 2 or 3. Intent-to-treat analysis showed end-of-treatment virologic response in 75% of patients (81% of genotypes 2 or 3; 65% of genotypes 1 or 4), and a sustained virologic response in 61% of patients (72% of genotypes 2 or 3; 48% of genotypes 1 or 4). Fifteen patients (22%) did not complete the study, in 5 (8%) cases because of severe adverse events. CONCLUSIONS: Drug users with chronic HCV infection, regularly attending an opiate maintenance program in which close collaboration between hepatologists/internists and addiction specialists is assured, can be treated effectively and safely with pegylated interferon-alfa-2a and ribavirin. Treatment results are very similar to those in other patient groups, and thus therapy should also be considered for this population.

Low-dose chemotherapy for extra-abdominal desmoid tumor.

OBJECTIVE: To assess the outcome of patients with extra-abdominal desmoid tumor treated with low dose chemotherapy (methotrexate and vinblastine) both for tumor response and treatment related toxicity. METHODS: We retrospectively reviewed the outcome of 12 patients who underwent low dose chemotherapy for extra abdominal desmoid of different locations. The study took place in the McGill University Health Center, Montreal, Canada between 1996 and 2003. We evaluated the patients for their compliance, tumor response, complications of treatment, and impact of treatment on symptoms. There were 7 females and 5 males with a mean age of 46 years. RESULTS: Disease related morbidity included pain in 7 patients, functional limitation in 7 and cosmetic defects in 3. The mean tumor size was 11 cm (3-20 cm). The mean follow-up was 43 months (15-71 months). Chemotherapy was administered weekly. Complications were significant. Only 2 patients did not experience the toxicity. According to Response Evaluation Criteria in Solid Tumors, 6 tumors showed a partial response and 6 remained stable. None showed progression. Of the 7 patients who had painful tumors, 6 achieved significant symptom relief. Function was improved in 3 and restored to normal in 4. Cosmesis was improved in 2 of the 3. At latest follow-up, tumors remained stable in 8, one has markedly regressed and 3 exhibited progression at an average of 54 months. Only one patient required surgery. The only long term side effect was a sensory peripheral neuropathy. CONCLUSION: Low dose chemotherapy was found to be a valuable adjunct to prevent local progression and improve symptoms.