Myoepithelial Carcinoma of Soft Tissue: Report of Two Cases.

CASE: A 40-year-old man was evaluated for a painful mass on his right calf, and a 36-year-old woman presented with a painless mass on her right foot. Final pathology revealed marked nuclear atypia and positivity for S100/SOX10 and AE1/AE3 confirming diagnoses of myoepithelial carcinoma. Both patients underwent surgical resection and are without evidence of local recurrence or metastatic disease at 1-year follow-up. CONCLUSION: Soft-tissue tumors presenting in the extremities warrant careful evaluation and timely histopathologic diagnosis. Myoepithelial carcinomas are rare, aggressive tumors with a propensity for local recurrence and metastasis. Treatment of these tumors should be discussed by a multidisciplinary tumor team.

Complications after limb salvage surgeries for musculoskeletal malignancies: 10-year experience of the main sarcoma center in Bulgaria.

INTRODUCTION: Limb salvage surgery is currently the most frequently used treatment option in Bulgaria for individuals with musculoskeletal malignancies. Clinical data about complications from these procedures is limited in the country, with only a few studies currently available.

Carpal Tunnel Syndrome Caused by a Juxta-articular Myxoma: A Case Report.

Juxta-articular myxoma (JAM) is a rare soft tissue tumour predominantly composed of mucinous tissue and usually found around large joints. We report a 73-year-old woman with a 5-year history of a soft tissue mass in the thenar eminence of the right wrist who presented to our department. An initial diagnosis of a ganglion cyst was made using magnetic resonance imaging (MRI) and the jellied content of the mass by aspiration. Two years after presentation, surgical treatment with tumour resection and carpal tunnel release via the radial approach was performed because the symptoms of carpal tunnel syndrome worsened, and the tumour invaded the carpal tunnel. Histopathological examination revealed a JAM. At the 1-year follow-up, the symptoms of carpal tunnel syndrome had resolved, and no recurrence was confirmed by MRI. Level of Evidence: Level V (Therapeutic).

Surgical Margins in Musculoskeletal Sarcoma.

¼ Negative margin resection of musculoskeletal sarcomas is associated with reduced risk of local recurrence.¼ There is limited evidence to support an absolute margin width of soft tissue or bone that correlates with reduced risk of local recurrence.¼ Factors intrinsic to the tumor, including histologic subtype, grade, growth pattern and neurovascular involvement impact margin status and local recurrence, and should be considered when evaluating a patient's individual risk after positive margins.¼ Appropriate use of adjuvant therapy, critical analysis of preoperative advanced cross-sectional imaging, and the involvement of a multidisciplinary team are essential to obtain negative margins when resecting sarcomas.

Functional outcomes of reconstructive flap surgery for soft tissue sarcoma: Long-term outcomes of functional restoration using innervated muscle transplantation.

Functional outcomes associated with prognostic factors and innervated muscle transplantation after wide soft tissue sarcoma resection remain unclear. We retrospectively examined the functional outcomes of reconstructive flap surgery for soft tissue sarcoma. Twenty patients underwent innervated muscle transplantation with pedicled or free flaps for functional reconstruction of resected muscles. Thirteen latissimus dorsi muscles and one vastus lateralis muscle combined with an anterolateral thigh flap were transferred as free flaps using the epi-perineural suture technique. Six latissimus dorsi muscles were transferred as pedicled flaps with neural continuity. Postoperative functional outcomes were assessed using the Musculoskeletal Tumor Society (MSTS) scores for the upper and lower extremities of 22 and 24 patients, respectively. The mean MSTS score for all patients was 82.3 at 12 months postoperatively. The mean scores for patients who underwent reconstruction with pedicled and free flaps were 89.2 and 77.1, respectively. The MSTS scores for the lower extremity, tumor size ≥5 cm, and free flap reconstruction were significantly lower than those for the upper extremity, tumor size <5 cm, and pedicled flap reconstruction (P = 0.02, 0.37, and 0.008, respectively). The postoperative MSTS score for innervated muscle transplantation was 76.7 at 12 months and was significantly higher (83.7) at 24 months (P = 0.003). Functional outcomes were significantly associated with tumor location, tumor size, and reconstructive flap type based on the MSTS scores. Innervated muscle transplantation improved functional outcomes at 24 months postoperatively via sufficient recovery of the innervated muscle, not the compensatory recovery of the remaining muscle.

Generation of post-surgical minimal residual disease models to investigate metastasis in soft tissue sarcoma patient-derived orthotopic xenografts.

Despite optimal multimodal treatment including surgical resection, 50%-80% of high-grade soft tissue sarcoma (STS) patients metastasize. Here, we present a protocol for the generation and use of post-surgical minimal residual disease models to investigate metastatic relapse in STS patient-derived xenografts. We describe steps for orthotopic engraftment of high-grade STS patient-derived tumor tissue. We then detail procedures for primary tumor resection with broad, negative resection margins and follow-up until metastases using MRI. For complete details on the use and execution of this protocol, please refer to Fischer et al. (2023).1.

Explainable Model Using Shapley Additive Explanations Approach on Wound Infection after Wide Soft Tissue Sarcoma Resection: "Big Data" Analysis Based on Health Insurance Review and Assessment Service Hub.

Background and Objectives: Soft tissue sarcomas represent a heterogeneous group of malignant mesenchymal tissues. Despite their low prevalence, soft tissue sarcomas present clinical challenges for orthopedic surgeons owing to their aggressive nature, and perioperative wound infections. However, the low prevalence of soft tissue sarcomas has hindered the availability of large-scale studies. This study aimed to analyze wound infections after wide resection in patients with soft tissue sarcomas by employing big data analytics from the Hub of the Health Insurance Review and Assessment Service (HIRA). Materials and Methods: Patients who underwent wide excision of soft tissue sarcomas between 2010 and 2021 were included. Data were collected from the HIRA database of approximately 50 million individuals' information in the Republic of Korea. The data collected included demographic information, diagnoses, prescribed medications, and surgical procedures. Random forest has been used to analyze the major associated determinants. A total of 10,906 observations with complete data were divided into training and validation sets in an 80:20 ratio (8773 vs. 2193 cases). Random forest permutation importance was employed to identify the major predictors of infection and Shapley Additive Explanations (SHAP) values were derived to analyze the directions of associations with predictors. Results: A total of 10,969 patients who underwent wide excision of soft tissue sarcomas were included. Among the study population, 886 (8.08%) patients had post-operative infections requiring surgery. The overall transfusion rate for wide excision was 20.67% (2267 patients). Risk factors among the comorbidities of each patient with wound infection were analyzed and dependence plots of individual features were visualized. The transfusion dependence plot reveals a distinctive pattern, with SHAP values displaying a negative trend for individuals without blood transfusions and a positive trend for those who received blood transfusions, emphasizing the substantial impact of blood transfusions on the likelihood of wound infection. Conclusions: Using the machine learning random forest model and the SHAP values, the perioperative transfusion, male sex, old age, and low SES were important features of wound infection in soft-tissue sarcoma patients.

Radiotherapy versus limb-sparing surgery alone in low-grade soft-tissue sarcoma of the extremity and trunk wall: a systematic review and meta-analysis.

Current guidelines recommend the use of radiotherapy in the management of intermediate and high-grade soft-tissue sarcoma of the extremity and trunk wall. Its use in low-grade sarcoma is less clear. To date there have been no pooled data analyses regarding its role in this context. Its use is not without complications and therefore must be justified. We aim to assess the oncological impact of radiotherapy versus limb-sparing surgery alone in this subset of sarcoma. Medline, EMBASE and Cochrane's databases were searched from 1982 to present. Studies on or having a subgroup analysis of low-grade soft tissue sarcoma, with a radiotherapy and a surgery only arm were included. Outcomes included local recurrence and overall survival. Patients were at least 16 years of age with primary de-novo sarcoma who had not undergone prior resection or treatment. Those undergoing concomitant therapy were excluded. Data extraction was performed independently by two reviewers. Results were pooled using a random-effects model and presented as a forest plot. Primary outcome measures included local recurrence and overall survival. Eleven unique studies were included, consisting of two RCTs and nine non-randomized studies. Overall, there were 12 799 patients. Four studies were included in meta-analysis and the overall pooled effect showed a limited role of radiotherapy in overall survival outcomes when compared to limb-sparing surgery alone HR 1.00 [0.83-1.20] P = 0.41. Descriptive analysis suggests there is limited role of radiotherapy in improving local recurrence outcomes. This study suggests there is limited role for radiotherapy versus limb-sparing surgery alone in low-grade soft-tissue sarcoma. These findings strongly suggest there is lack of high-quality data and that further research must be undertaken prior to forming any strong conclusions regarding the management of low-grade soft-tissue sarcoma. Demonstrating a role for radiotherapy may help improve the quality of excisional margins and thus potentiate limb-sparing surgery.

Management of oligometastatic/metastatic sarcomas and place of local treatments with focus on modern radiotherapy approaches.

Soft tissue sarcomas are a rare and heterogeneous disease. For localized disease, treatment is based on surgery and radiotherapy with or without chemotherapy depending on risk factors. Upfront metastases are present in 7 to 20% of cases, and are localized to the lungs in most of cases. Disseminated disease is generally considered incurable but in selected cases, aggressive local treatment of metastases allowed long survival. Treatment of primary tumour is often debated. Our purpose is to evaluate the literature concerning the role of radiotherapy in the management of primary metastatic soft tissue sarcomas.

Thoracoscopic Wedge Resection for Low-Grade Fibromyxoid Sarcoma (Evans Tumor) with Massive Calcification and Originating from the Lung: A Rare Case in an Unexpected Location.

We encountered a rare case of low-grade fibromyxoid sarcoma, which is generally known as Evans tumor, with massive calcification originating from the lung. The patient was a 22-year-old man with Duchenne muscular dystrophy who was referred for a detailed investigation of an intrathoracic tumor with massive calcification. Although our preoperative diagnosis was a solitary fibrous tumor originating from the mediastinum or diaphragm, intraoperative thoracoscopy revealed the tumor arising from the left lower lobe without adhesion to the other organs. Considering his medical history, we aimed to preserve lung function and chose wedge resection, which completely removed the tumor. Based on the pathological findings, the tumor was diagnosed as low-grade fibromyxoid sarcoma with massive calcification originating from the lung. Although extremely rare, this tumor should be considered as a differential diagnosis for a solitary lung mass with massive calcification in young adults.

What is the diagnostic accuracy of fluorescence-guided surgery for margin assessment in appendicular bone and soft tissue tumors? - A systematic review of clinical studies.

BACKGROUND: Fluorescence-guided surgery (FGS) is a novel technique to successfully assess surgical margins intraoperatively. Investigation and adoption of this technique in orthopaedic oncology remains limited. METHODS: The PRISMA guidelines were followed for this manuscript. Our study was registered on PROSPERO (380520). Studies describing the use of FGS for resection of bone and soft tissue sarcomas (STS) on humans were included. Diagnostic performance metrics (sensitivity, specificity, positive predictive value [PPV], negative predictive value [NPV] and accuracy) and margin positivity rate were the outcomes assessed. RESULTS: Critical appraisal using the Joanna Brigs Institute checklists showed significant concerns for study quality. Sensitivity of FGS ranged from 22.2 % to 100 % in three of the four studies assessing his metrics; one study in appendicular tumors in the pediatric population reported 0 % sensitivity in the three cases included. Specificity ranged from 9.38 % to 100 %. PPV ranged from 14.6 % to 70 % while NPV was between 53.3 % and 100 %. The diagnostic accuracy ranged from 21.62 % to 92.31 %. Margin positivity rate ranged from 2 % to 50 %, with six of the seven studies reporting values between 20 % and 50 %. CONCLUSIONS: FSG is a feasible technique to assess tumor margins in bone and STS. Reported performance metrics and margin positivity rates vary widely between studies due to low study quality and high heterogeneity in dying protocols. LEVEL OF EVIDENCE: Level III, diagnostic study.

Identifying risk factors and analyzing reconstructive outcomes in patients with lower-extremity soft-tissue sarcoma.

PURPOSE: Reconstructing defects after resecting soft-tissue sarcoma (STS) can be challenging. The aim of this retrospective study was to analyze the reconstructive outcomes and identify the potential risk factors in patients undergoing reconstruction after excision of lower-extremity STS. METHODS: Patients with lower-extremity STS were included. This database was compiled of patients from a single, large National Cancer Institute-accredited academic hospital. In total, 302 patients were included between January 2016 to January 2022. Univariate and multivariate analyses were performed to calculate odds ratios (ORs) for developing complications for each patient and surgical characteristic. RESULTS: The following factors were independent predictors of any complication: benign pulmonary disease (OR = 4.2; p = 0.02), preoperative radiotherapy (RT; OR = 2.5; p = 0.047), a tumor in the medial thigh (OR = 1.9; p = 0.03), body mass index (BMI) > 30 kg/m2 (OR = 1.05; p = 0.037), and full-thickness skin graft (OR = 5.4; p = 0.01). In the preoperative RT subgroup, reconstructing a defect via undermining and layered closure alone was an independent predictor of dehiscence (OR = 2.1; p = 0.02) and seroma (OR = 3.1; p = 0.02), whereas pedicled flaps (OR = 0.08; p = 0.001) and free flaps (OR = 0.05; p = 0.001) were independent protectors against any complication. CONCLUSION: Information derived from this analysis will assist with accurate preoperative patient counseling, which is crucial for informed decision-making and expectation management in lower-extremity STS. BMI and pulmonary function should be optimized to the extent possible to reduce postoperative complications. Patients treated preoperatively with RT should be reconstructed with a pedicled or free flap to optimize recovery.

Active surveillance of diffuse-type tenosynovial giant cell tumors: A retrospective, multicenter cohort study.

BACKGROUND: Diffuse-type tenosynovial giant cell tumor (D-TGCT) is a mono-articular, soft-tissue tumor. Although it can behave locally aggressively, D-TGCT is a non-malignant disease. This is the first study describing the natural course of D-TGCT and evaluating active surveillance as possible treatment strategy. METHODS: This retrospective, multicenter study included therapy naïve patients with D-TGCT from eight sarcoma centers worldwide between 2000 and 2019. Patients initially managed by active surveillance following their first consultation were eligible. Data regarding the radiological and clinical course and subsequent treatments were collected. RESULTS: Sixty-one patients with primary D-TGCT were initially managed by active surveillance. Fifty-nine patients had an MRI performed around first consultation: D-TGCT was located intra-articular in most patients (n = 56; 95 %) and extra-articular in 14 cases (24 %). At baseline, osteoarthritis was observed in 13 patients (22 %) on MRI. Most of the patients' reported symptoms: pain (n = 43; 70 %), swelling (n = 33; 54 %). Eight patients (13 %) were asymptomatic. Follow-up data were available for 58 patients; the median follow-up was 28 months. Twenty-one patients (36 %) had radiological progression after 21 months (median). Eight of 45 patients (18 %) without osteoarthritis at baseline developed osteoarthritis during follow-up. Thirty-seven patients (64 %) did not clinically deteriorate during follow-up. Finally, eighteen patients (31 %) required a subsequent treatment. CONCLUSION: Active surveillance can be considered adequate for selected therapy naïve D-TGCT patients. Although follow-up data was limited, almost two-thirds of the patients remained progression-free, and 69 % did not need treatment during the follow-up period. However, one-fifth of patients developed secondary osteoarthritis. Prospective studies on active surveillance are warranted.

Low local recurrence rates following marginal surgical resection of non-coelomic Atypical Lipomatous Tumours/Well-differentiated Liposarcomas.

INTRODUCTION: High rates of local recurrence (LR) have been reported following resection of extremity Atypical lipomatous tumours/Well-differentiated liposarcomas (ALTs). This retrospective study of patients who underwent resection of primary deep extremity and trunk ALTs at a specialist sarcoma centre aims to assess morbidity and factors associated with low local recurrence rates (LRR). METHODS: To review a homogeneous cohort of patients with low-grade disease, tumours with known high-risk histological features were excluded. Prognostic variables potentially influencing local recurrence (LR) (age, size, site, margin status, and histological findings) were analysed. Endpoints were LR, distant recurrence (DR) and local disease-free survival (LDFS). RESULTS: 127 patients were identified, with median follow-up of 54 months (0-235). Median tumour size was 17.5 cm (5-36). 85 % occurred in the lower limb. 93.7 % underwent marginal resection. No patients received radiotherapy. Median hospital stay was 3 days (0-16). 7.9 % returned to theatre for evacuation of haematoma or infected seroma and 18.1 % had outpatient seroma aspiration. Surgical margins were R0/R1 in 93.7 % of patients and R2 in 6.3 % with a LR rate of 8.4 % and 75 % respectively at median time of 54 months. One- and 5-year LDFS was 100 % and 88.4 %, respectively. DR rate was 0.8 % (1/127) this patient had pleomorphic liposarcomatous transformation on recurrence and subsequently developed distant metastases. No patients died of disease. CONCLUSION: Function-preserving marginal resection of non-coelomic ALTs has low morbidity, low LR and extremely low rates of distant relapse. Patients with lower limb ALT were found to have significantly lower LR, which may impact follow-up protocols.

Lipid profiling of electrosurgical vapors for real-time assistance of soft tissue sarcoma resection.

BACKGROUND: Soft tissue sarcomas (STS) constitute a heterogeneous group of rare tumor entities. Treatment relies on challenging patient-tailored surgical resection. Real-time intraoperative lipid profiling of electrosurgical vapors by rapid evaporative ionization mass spectrometry (REIMS) may aid in achieving successful surgical R0 resection (i.e., microscopically negative-tumor margin resection). Here, we evaluate the ex vivo accuracy of REIMS to discriminate and identify various STS from normal surrounding tissue. METHODS: Twenty-seven patients undergoing surgery for STS at Maastricht University Medical Center+ were included in the study. Samples of resected STS specimens were collected and analyzed ex vivo using REIMS. Electrosurgical cauterization of tumor and surrounding was generated successively in both cut and coagulation modes. Resected specimens were subsequently processed for gold standard histopathological review. Multivariate statistical analysis (principal component analysis-linear discriminant analysis) and leave-one patient-out cross-validation were employed to compare the classifications predicted by REIMS lipid profiles to the pathology classifications. Electrosurgical vapors produced during sarcoma resection were analyzed in vivo using REIMS. RESULTS: In total, 1200 histopathologically-validated ex vivo REIMS lipid profiles were generated from 27 patients. Ex vivo REIMS lipid profiles classified STS and normal tissues with 95.5% accuracy. STS, adipose and muscle tissues were classified with 98.3% accuracy. Well-differentiated liposarcomas and adipose tissues could not be discriminated based on their respective lipid profiles. Distinction of leiomyosarcomas from other STS could be achieved with 96.6% accuracy. In vivo REIMS analyses generated intense mass spectrometric signals. CONCLUSION: Lipid profiling by REIMS is able to discriminate and identify STS with high accuracy and therefore constitutes a potential asset to improve surgical resection of STS in the future.

Preoperative radiation therapy increases adherence in patients with high-risk extremity soft tissue sarcoma.

BACKGROUND: Surgery and radiation therapy remain the standard of care for patients with high-grade extremity soft tissue sarcoma that are >5 cm. Radiation therapy is time and labor-intensive for patients, and social determinants of health may affect adherence. The aim of this study was to define demographic, clinical, and treatment factors associated with the completion of radiation therapy and determine if preoperative radiation therapy improved adherence compared to postoperative radiation therapy. METHODS: The cohort included patients in the National Cancer Database with high-grade extremity soft tissue sarcoma >5 cm without nodal or distant metastases who received limb-sparing surgery and radiation therapy with microscopically negative R0 margins. Multivariable logistic regression analyses identified factors associated with radiation therapy sequencing and adherence (defined as completion of 50 Gy preoperative radiation therapy or at least 60 Gy postoperative radiation therapy). A multivariable Cox Proportional Hazards model assessed overall survival. RESULTS: Among 2,145 patients, 47.1% received preoperative radiation therapy (n = 1,010), and 52.9% (n = 1135) received postoperative radiation therapy. A greater proportion of patients treated with preoperative (77.2%) versus postoperative radiation therapy (64.9%, P < .0001) received the recommended dose. More patients with private insurance (49.8% vs 35.3% Medicaid vs 44.9% Medicare, P = .011) and patients treated at an academic medical center (52.6% vs 47.4%, P < .001) received preoperative radiation therapy. Patients who received preoperative radiation therapy had lower odds of receiving insufficient doses of radiation therapy (odds ratio 0.34 [95% CI 0.27-0.47]). Neither radiation therapy adherence nor sequencing were independent predictors of overall survival. CONCLUSIONS: Patients who received preoperative radiation therapy were more likely to complete therapy and receive an optimal dose than patients treated with postoperative radiation therapy. Preoperative radiation therapy improves adherence and should be widely considered in patients with high-grade extremity soft tissue sarcoma, particularly in patients at risk for not completing therapy.

Emerging innovations and advancements in the treatment of extremity and truncal soft tissue sarcomas.

In this special edition update on soft tissue sarcomas (STS), we cover classifications, emerging technologies, prognostic tools, radiation schemas, and treatment disparities in extremity and truncal STS. We discuss the importance of enhancing local control and reducing complications, including the role of innovative imaging, surgical guidance, and hypofractionated radiation. We review advancements in systemic and immunotherapeutic treatments and introduce disparities seen in this vulnerable population that must be considered to improve overall patient care.