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Background: Low-grade fibromyxoid sarcoma (LGFMS) is a rare malignant fibroblastic tumor that often appears deceptively benign. Accurate diagnosis is challenging due to its variable morphology and low mitotic activity. This systematic review provides a comprehensive overview of LGFMS and its subtypes. Methods: A systematic search of PubMed and Embase up to September 2025 identified 273 studies, complemented by four institutional cases from Amsterdam UMC. Individual patient data were pooled to analyze clinical presentation, diagnostic approaches, treatment modalities, and outcomes. Results: In total, 773 patients were included, with a median age of 35 years and equal gender distribution. Tumors were predominantly deep-seated (80%), most commonly located in the thigh or pelvis. MUC4 positivity (96%) and FUS-CREB3L2 fusion (47%) were the most consistent diagnostic markers. Surgery was the mainstay of treatment (98%), with R0 resection achieved in 36% of cases and R1 in 15%. Adjuvant therapies, including chemotherapy and radiotherapy, were rarely used and showed limited efficacy. After a median follow-up of 3.0 years, 19% developed local recurrence and 21% developed metastases. R0 resections were associated with significantly better recurrence-free survival than R1 resection (p < 0.05). Conclusions: LGFMS exhibits indolent histology but potential for late recurrence and metastasis, warranting prolonged radiological follow-up and multicenter studies to evaluate adjuvant strategies.

Diffuse-type tenosynovial giant cell tumors’ (D-TGCTs) intra- and extra-articular expansion about the knee often necessitates an anterior and posterior surgical approach to facilitate an extensive synovectomy. There is no consensus on whether two-sided synovectomies should be performed in one or two stages. This retrospective study included 191 D-TGCT patients from nine sarcoma centers worldwide to compare the postoperative short-term outcomes between both treatments. Secondary outcomes were rates of radiological progression and subsequent treatments. Between 2000 and 2020, 117 patients underwent one-stage and 74 patients underwent two-stage synovectomies. The maximum range of motion achieved within one year postoperatively was similar (flexion 123–120°, p = 0.109; extension 0°, p = 0.093). Patients undergoing two-stage synovectomies stayed longer in the hospital (6 vs. 4 days, p < 0.0001). Complications occurred more often after two-stage synovectomies, although this was not statistically different (36% vs. 24%, p = 0.095). Patients treated with two-stage synovectomies exhibited more radiological progression and required subsequent treatments more often than patients treated with one-stage synovectomies (52% vs. 37%, p = 0.036) (54% vs. 34%, p = 0.007). In conclusion, D-TGCT of the knee requiring two-side synovectomies should be treated by one-stage synovectomies if feasible, since patients achieve a similar range of motion, do not have more complications, but stay for a shorter time in the hospital.

Background The optimal surgical treatment for patients presenting with (impending and complete) pathological proximal femoral fractures is predicated on prognosis. Guidelines recommend a preoperative biopsy to exclude sarcomas, however no evidence confirms a benefit. Objective This study aimed to describe the diagnostic accuracy, morbidity and sarcoma incidence of biopsy results in these patients. Material and Methods All patients ( n  = 153) presenting with pathological proximal femoral fractures between 2000 and 2019 were retrospectively evaluated. Patients after inadvertent surgery ( n  = 25) were excluded. Descriptive statistics were used to evaluate the accuracy and morbidity of diagnostic biopsies. Results Of 112/128 patients who underwent biopsy, nine (8%) biopsies were unreliable either due to being inconclusive ( n  = 5) or because the diagnosis changed after resection ( n  = 4). Of impending fractures, 32% fractured following needle core biopsy. Median time from diagnosis to surgery was 30 days (interquartile range 21–46). The overall biopsy positive predictive value (PPV) to differentiate between sarcoma and non-sarcoma was 1.00 (95% confidence interval [CI] 0.88–1.00). In patients with a previous malignancy ( n  = 24), biopsy ( n  = 23) identified the diagnosis in 83% (PPV 0.91, 95% CI 0.71–0.99), of whom five (24%) patients had a new diagnosis. In patients without a history of cancer ( n  = 61), final diagnosis included carcinomas ( n  = 24, 39.3%), sarcomas ( n  = 24, 39.3%), or hematological malignancies ( n  = 13, 21.3%). Biopsy ( n  = 58) correctly identified the diagnosis in 66% of patients (PPV 0.80, 95% CI 0.67–0.90). Conclusion This study confirms the importance of a preoperative biopsy in solitary pathological proximal femoral fractures due to the risk of sarcoma in patients with and without a history of cancer. However, biopsy delays the time to definite surgery, results can be inconclusive or false, and it risks completion of impending fractures.

In Tenosynovial Giant Cell Tumours (TGCT), previously named Pigmented Villonodular Synovitis (PVNS), a distinction is made between a single nodule (localized-type) and multiple nodules (diffuse-type). Diffuse-type is considered locally aggressive. Onset and extermination of this orphan disease remain unclear. Surgical resection is the most commonly performed treatment. Unfortunately, recurrences often occur (up to 92%), necessitating reoperations and adjuvant treatments. Once all treatments fail or if severe complications occur, limb amputation may become unavoidable. We describe four cases of above-knee amputation after TGCT diagnosis.

At present, the treatment strategies in patients with localized and diffuse forms of pigmented villonodular synovitis have more or less been standardized. However, these strategies are not optimal because high recurrence rates persist and studies with a sufficient level of evidence are lacking. This systematic review article describes all known treatment options for intra-articular pigmented villonodular synovitis and their clinical results. Based on this research, we provide guidelines to support physicians in making the optimal treatment decisions. Given the rarity of the disease, randomized studies are not to be expected, but an international registry through existing networks would offer the benefit of getting a better insight into the outcome of this disease. Therefore, we propose a basic set of data to be investigated and ideally to be reported on in such a registry.

BackgroundThe optimal surgical treatment for patients presenting with (impending and complete) pathological proximal femoral fractures is predicated on prognosis. Guidelines recommend a preoperative biopsy to exclude sarcomas, however no evidence confirms a benefit.ObjectiveThis study aimed to describe the diagnostic accuracy, morbidity and sarcoma incidence of biopsy results in these patients.Material and MethodsAll patients (n = 153) presenting with pathological proximal femoral fractures between 2000 and 2019 were retrospectively evaluated. Patients after inadvertent surgery (n = 25) were excluded. Descriptive statistics were used to evaluate the accuracy and morbidity of diagnostic biopsies.ResultsOf 112/128 patients who underwent biopsy, nine (8%) biopsies were unreliable either due to being inconclusive (n = 5) or because the diagnosis changed after resection (n = 4). Of impending fractures, 32% fractured following needle core biopsy. Median time from diagnosis to surgery was 30 days (interquartile range 21–46). The overall biopsy positive predictive value (PPV) to differentiate between sarcoma and non-sarcoma was 1.00 (95% confidence interval [CI] 0.88–1.00). In patients with a previous malignancy (n = 24), biopsy (n = 23) identified the diagnosis in 83% (PPV 0.91, 95% CI 0.71–0.99), of whom five (24%) patients had a new diagnosis. In patients without a history of cancer (n = 61), final diagnosis included carcinomas (n = 24, 39.3%), sarcomas (n = 24, 39.3%), or hematological malignancies (n = 13, 21.3%). Biopsy (n = 58) correctly identified the diagnosis in 66% of patients (PPV 0.80, 95% CI 0.67–0.90).ConclusionThis study confirms the importance of a preoperative biopsy in solitary pathological proximal femoral fractures due to the risk of sarcoma in patients with and without a history of cancer. However, biopsy delays the time to definite surgery, results can be inconclusive or false, and it risks completion of impending fractures.

Diffuse-type tenosynovial giant-cell tumour is a rare, locally aggressive, and difficult-to-treat soft tissue tumour. Clinical and surgical outcomes depend on multiple factors, including preoperative diagnostic assessment, the localisation and extent of disease, and possibly the choice of treatment modalities by orthopaedic surgeons. We did a retrospective cohort study to characterise global surgical treatment protocols, and assess surgical outcomes, complications, and functional results in patients with diffuse-type tenosynovial giant-cell tumours. In this international, multicentre, retrospective cohort study, we included consecutive patients treated in 31 sarcoma reference centres between Jan 1, 1990, and Dec 31, 2017. Eligible patients were of any age and had histologically proven diffuse-type tenosynovial giant-cell tumour of large joints. Patient data were retrieved from the local databases of participating centres. Patients with localised-type tenosynovial giant-cell tumour were excluded. In the analysis, we only included patients with complete core criteria data regarding admission status, date of treatment, type of treatment at participating centre, and first local recurrence after treatment. We used a non-parametric method to estimate recurrence-free survival at 3, 5, and 10 years after initial surgical resection in a tertiary centre. We used a multivariate Cox regression model to estimate the effect of risk factors. We also present subgroup analyses of disease status at presentation (primary vs recurrent disease) and recurrence-free survival by surgery type (open surgery vs arthroscopic synovectomy), and prespecified risk factors were tested in a univariate and multivariable analyses, with an endpoint of first local recurrence after treatment in a tertiary centre. Data collection for these analyses occurred between January, 2016, and May, 2018. We received the records of 1192 patients, of which 966 (81%) were surgically treated and had complete information on core criteria. 445 patients were admitted with therapy-naive disease of the knee and were primarily treated in a tertiary centre. Since patients with wait and see treatment do not have a starting date of treatment, these patients were excluded in the calculation of median follow-up time for all patients. For this calculation we used time of surgery as a starting date. 758 (64%) of 1192 patients had knee involvement and 628 (54%) of 1163 patients with complete data on type of surgery had one-staged open synovectomy. At a median follow-up of 54 months (IQR 27–97), recurrent disease developed in 425 (44%) of all 966 surgically treated cases, and recurrence-free survival was 62% (95% CI 59–65) at 3 years, 55% (51–58) at 5 years, and 40% (35–45) at 10 years. Surgical complications were reported in 105 (12%) of 906 patients who had complete data on surgical complications. Pain improved after surgical treatment in 255 (59%) of 434 patients and swelling improved in 328 (72%) of 453 patients who had complete data. This study of patients with diffuse-type tenosynovial giant-cell tumour provides a comprehensive and up-to-date disease overview, assessing the clinical profile and management of the disease in multiple specialised referral centres. Surgical treatment of diffuse-type tenosynovial giant cell tumours is not a definitive treatment for every patient because it involves a high risk for local recurrent disease and a relatively high risk for postoperative complications. After surgical treatment in treatment-naive patients, risk factors for recurrent disease in individual patients were not identified in what we believe is the largest cohort to date. Daiichi Sankyo.