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Soft-tissue sarcomas are uncommon, aggressive, and histologically heterogeneous, malignant tumors. Soft-tissue tumor types have different age and anatomical site distributions. This study aimed to assess the prevalence and incidence of soft-tissue sarcomas of the extremities to facilitate early diagnosis and intervention in the Kingdom of Saudi Arabia. This retrospective study included all patients diagnosed with primary soft-tissue sarcomas of the extremities and pelvis between January 2006 and December 2015. The data were obtained from the Saudi Cancer Registry. Means and confidence intervals were reported for numerical variables, whereas the frequencies and percentages were reported for categorical variables. The chi-squared test and analysis of variance were used to test the associations between categorical numerical variables. Cox regression analysis was used to determine the hazard ratios for each sarcoma type. Of the 659 patients, 145 (22%) were aged 19–30 years, 365 (55.4%) were men, and 411 (62.4%) had lower limb sarcoma. Most of the tumors (54.3%) were localized. The incidence of sarcoma ranged from 2 to 2.9 cases per one million (average, 2.4 cases per one million). Moreover, liposarcoma was the most commonly diagnosed (n = 135; 20.5%), followed by undifferentiated pleomorphic sarcoma (n = 87; 13.2%). The incidence of soft-tissue sarcomas in the Kingdom of Saudi Arabia were lower than those in Western countries. However, the distribution of soft-tissue sarcoma subtypes was similar to those in other countries. The survival rates in this study highlight the need for continued research and targeted interventions to improve the outcomes of patients with soft-tissue sarcomas, especially those with more aggressive subtypes, such as sarcoma not otherwise specified and rhabdomyosarcoma. [ABSTRACT FROM AUTHOR]

Abstract Soft-tissue sarcomas are uncommon, aggressive, and histologically heterogeneous, malignant tumors. Soft-tissue tumor types have different age and anatomical site distributions. This study aimed to assess the prevalence and incidence of soft-tissue sarcomas of the extremities to facilitate early diagnosis and intervention in the Kingdom of Saudi Arabia. This retrospective study included all patients diagnosed with primary soft-tissue sarcomas of the extremities and pelvis between January 2006 and December 2015. The data were obtained from the Saudi Cancer Registry. Means and confidence intervals were reported for numerical variables, whereas the frequencies and percentages were reported for categorical variables. The chi-squared test and analysis of variance were used to test the associations between categorical numerical variables. Cox regression analysis was used to determine the hazard ratios for each sarcoma type. Of the 659 patients, 145 (22%) were aged 19–30 years, 365 (55.4%) were men, and 411 (62.4%) had lower limb sarcoma. Most of the tumors (54.3%) were localized. The incidence of sarcoma ranged from 2 to 2.9 cases per one million (average, 2.4 cases per one million). Moreover, liposarcoma was the most commonly diagnosed (n = 135; 20.5%), followed by undifferentiated pleomorphic sarcoma (n = 87; 13.2%). The incidence of soft-tissue sarcomas in the Kingdom of Saudi Arabia were lower than those in Western countries. However, the distribution of soft-tissue sarcoma subtypes was similar to those in other countries. The survival rates in this study highlight the need for continued research and targeted interventions to improve the outcomes of patients with soft-tissue sarcomas, especially those with more aggressive subtypes, such as sarcoma not otherwise specified and rhabdomyosarcoma.

Abstract Background The standard curative treatments for extremity soft tissue sarcoma (ESTS) include surgical resection with negative margins and perioperative radiotherapy. However, the optimal resection margin remains controversial. This study aimed to evaluate the outcomes in ESTS between microscopically positive margin (R1) and microscopically negative margin (R0) according to the Union for International Cancer Control (UICC) (R + 1 mm) classification. Methods Medical records of patients with localized ESTS who underwent primary limb-sparing surgery and postoperative radiotherapy between 2004 and 2015 were retrospectively reviewed. Patients were followed for at least 5 years or till local or distant recurrence was diagnosed during follow-up. Outcomes were local and distal recurrences and survival. Results A total of 52 patients were included in this study, in which 17 underwent R0 resection and 35 underwent R1 resection. No significant differences were observed in rates of local recurrence (11.4% vs. 35.3%, p = 0.062) or distant recurrence (40.0% vs. 41.18%, p = 0.935) between R0 and R1 groups. Multivariate analysis showed that distant recurrences was associated with a Fédération Nationale des Centres de Lutte Contre le Cancer (FNCLCC) grade (Grade III vs. I, adjusted hazard ratio (aHR): 12.53, 95% confidence interval (CI): 2.67–58.88, p = 0.001) and tumor location (lower vs. upper extremity, aHR: 0.23, 95% CI: 0.07–0.7, p = 0.01). Kaplan–Meier plots showed no significant differences in local (p = 0.444) or distant recurrent-free survival (p = 0.161) between R0 and R1 groups. Conclusions R1 margins, when complemented by radiotherapy, did not significantly alter outcomes of ESTS as R0 margins. Further studies with more histopathological types and larger cohorts are necessary to highlight the path forward.

Soft-tissue sarcomas (STSs) are rare neoplasms that can be initially evaluated by fine-needle aspiration cytology (FNAC). Although they are difficult to subtype, these sarcomas can be graded and classified based on cytomorphology. FNAC, with clinical and radiological correlation, can be effectively used in the diagnosis of STSs. Application of ancillary techniques is essential to arrive at the final diagnosis. The aim of the study was to determine the efficacy of FNAC in the initial diagnosis of STSs. Three patients who presented with soft-tissue masses were initially evaluated by ultrasound and/or magnetic resonance imaging and FNAC. Following which, all the three cases were operated on and studied on histopathology and immunohistochemistry to arrive at a final diagnosis. All the three cases were diagnosed as malignant on FNAC even when radiology findings differed or was not confirmatory in one case. FNAC diagnosis was appropriate in case of grading of tumors and classification based on morphology in all the three cases. FNAC of soft-tissue tumors (STTs) is a quick, reliable, and cost-effective mode of initial investigation for STTs. Although it is difficult to subtype the diagnosis, in majority of the cases, a diagnosis of benign or malignant and grade of the tumor can be assessed.

Soft-tissue sarcomas (STSs) are rare neoplasms that can be initially evaluated by fine-needle aspiration cytology (FNAC). Although they are difficult to subtype, these sarcomas can be graded and classified based on cytomorphology. FNAC, with clinical and radiological correlation, can be effectively used in the diagnosis of STSs. Application of ancillary techniques is essential to arrive at the final diagnosis. The aim of the study was to determine the efficacy of FNAC in the initial diagnosis of STSs. Three patients who presented with softtissue masses were initially evaluated by ultrasound and/or magnetic resonance imaging and FNAC. Following which, all the three cases were operated on and studied on histopathology and immunohistochemistry to arrive at a final diagnosis. All the three cases were diagnosed as malignant on FNAC even when radiology findings differed or was not confirmatory in one case. FNAC diagnosis was appropriate in case of grading of tumors and classification based on morphology in all the three cases. FNAC of soft-tissue tumors (STTs) is a quick, reliable, and cost-effective mode of initial investigation for STTs. Although it is difficult to subtype the diagnosis, in majority of the cases, a diagnosis of benign or malignant and grade of the tumor can be assessed. [ABSTRACT FROM AUTHOR]

Background: Rare tumors which are derived from the skin and soft tissue include a wide variety of types, but the knowledge and awareness of these tumors are limited. Besides, the number of specialists with specific training and experience in these tumors is very few. They are frequently associated with problems in the diagnosis and treatment. Purpose: In this study, we shared our experience and compared the outcome with the literature. Methods: Depending on clinicopathological evidence, 45 patients who were treated in our clinic between 2006 and 2021 were included in this descriptive study. Histopathological, etiological, and epidemiological results were analyzed. Tumors are classified as one of two types – either benign (noncancerous) or malignant (cancerous). Malignant tumors are also divided into carcinoma and sarcoma. Radiologic imaging methods and incisional/excisional biopsy were used for the diagnosis and staging. Chemotherapy/radiotherapy was planned after surgical treatment according to the diagnosis. Results: Of 45 cases, 40 (81.25%) were malignant and 5 (18.75%) were benign. The ages of the patients ranged from 10 to 94 years, and the mean age was 54.05 ± 20.1. The male/female ratio was 4.5/1. All patients were from the Aegean region of Turkey. The classification of the malignant tumors was carcinoma and sarcoma. The most common type of sarcomas was leiomyosarcoma and synovial sarcoma. Conclusion: When we consider the growing interest and knowledge in rare tumors, these case series are beneficial for epidemiological and surgical assessments.

Background: Over the years, radiotherapy has been established as a tool to improve local control for high-grade sarcomas. Although the European Society for Medical Oncology guidelines has taken notice of a shift toward a neoadjuvant radiotherapy approach, the American Society for Radiation Oncology guidelines clearly favor a neoadjuvant approach, citing debilitating long-term adverse effects when radiotherapy is applied postoperatively. In this study, we examined these irradiation-associated adverse events for adjuvant radiotherapy and focused on the prognostic factors for disease outcome, including local control. Methods: In this retrospective study, data for 106 patients with extremity soft-tissue sarcomas diagnosed between 1997 and 2021, of which 40 received adjuvant radiotherapy, were collected from the clinical and radiological information systems of a high-volume sarcoma treatment center. These data were then analyzed for radiation-associated side effects as well as predictive factors for overall survival, disease-free survival, local control, and surgical complications. Results: Radiotherapy was beneficial to patients improving local control, especially for high-grade sarcomas, even when those were resected with negative margins. Side effects due to radiotherapy occurred in 87.5% of the patients, and these effects primarily included radiation dermatitis in 67.5%; however, only 40.0% had any adverse event of ≥ grade 2 according to Common Terminology Criteria for Adverse Events. Long-term function-limiting side effects occurred in 45.0% of the patients; 10% exhibited ≥ grade 2 function-limiting adverse events. Greater time between surgery and adjuvant radiotherapy was beneficial for the patients, whereas joint infiltrating sarcomas were associated with more severe long term, function-limiting adverse events. 28.3% of the patients experienced a recurrence at any location (median time 18.35 months) and in 16% the recurrence was local (median time 16.11 months), resulting in 1, 3, and 5 year disease-free survival rates of 74.1, 58.9, and 38.5% and local control rates of 78.7, 61.6, and 42.8% were observed, respectively. Conclusion: Recurrences may be avoided with high-dose radiation, especially for high-grade G2 and G3 sarcomas, even after complete R0 resection. This resulted in a low rate of severe long-term function-limiting adverse events. Thus, adjuvant radiotherapy should be seriously considered when planning patient treatment, especially when treating patients that present with high-grade sarcomas. [ABSTRACT FROM AUTHOR]

Simple Summary: Differentiating atypical lipomatous tumors from lipomas on MR images is a challenging task due to similar imaging characteristics. Given these challenges, it would be highly beneficial to develop a reliable diagnostic tool, thereby minimizing the need for invasive diagnostic procedures. Therefore, the aim of this study was to develop and validate radiogenomic machine-learning models to predict the MDM2 gene amplification status in order to differentiate between ALTs and lipomas on preoperative MR images. The best machine-learning model was based on radiomic features from multiple MR sequences using a LASSO algorithm and showed a high discriminatory power to predict the MDM2 gene amplification. Due to the varying settings in which patients with lipomatous tumors present, this model may enhance the clinical diagnostic workup. Background: The aim of this study was to develop and validate radiogenomic models to predict the MDM2 gene amplification status and differentiate between ALTs and lipomas on preoperative MR images. Methods: MR images were obtained in 257 patients diagnosed with ALTs (n = 65) or lipomas (n = 192) using histology and the MDM2 gene analysis as a reference standard. The protocols included T2-, T1-, and fat-suppressed contrast-enhanced T1-weighted sequences. Additionally, 50 patients were obtained from a different hospital for external testing. Radiomic features were selected using mRMR. Using repeated nested cross-validation, the machine-learning models were trained on radiomic features and demographic information. For comparison, the external test set was evaluated by three radiology residents and one attending radiologist. Results: A LASSO classifier trained on radiomic features from all sequences performed best, with an AUC of 0.88, 70% sensitivity, 81% specificity, and 76% accuracy. In comparison, the radiology residents achieved 60–70% accuracy, 55–80% sensitivity, and 63–77% specificity, while the attending radiologist achieved 90% accuracy, 96% sensitivity, and 87% specificity. Conclusion: A radiogenomic model combining features from multiple MR sequences showed the best performance in predicting the MDM2 gene amplification status. The model showed a higher accuracy compared to the radiology residents, though lower compared to the attending radiologist. [ABSTRACT FROM AUTHOR]

Abstract Background Soft-tissue sarcomas (STS) represent a heterogeneous group of rare tumors including more than 70 different histological subtypes. High throughput molecular analysis (next generation sequencing exome [NGS]) is a unique opportunity to identify driver mutations that can change the usual one-size-fits-all treatment paradigm to a patient-driven therapeutic strategy. The primary objective of the MULTISARC trial is to assess whether NGS can be conducted for a large proportion of metastatic STS participants within a reasonable time, and, secondarily to determine whether a NGS-guided therapeutic strategy improves participant’s outcome. Methods This is a randomized, multicentre, phase II/III trial inspired by the design of umbrella and biomarker-driven trials. The setting plans up to 17 investigational centres across France and the recruitment of 960 participants. Participants aged at least 18 years, with unresectable locally advanced and/or metastatic STS confirmed by the French sarcoma pathological reference network, are randomized according to 1:1 allocation ratio between the experimental arm “NGS” and the standard “No NGS”. NGS will be considered feasible if (i) NGS results are available and interpretable, and (ii) a report of exome sequencing including a clinical recommendation from a multidisciplinary tumor board is provided to investigators within 7 weeks from reception of the samples on the biopathological platform. A feasibility rate of more than 70% is expected (null hypothesis: 70% versus alternative hypothesis: 80%). In terms of care, participants randomized in “No NGS” arm and who fail treatment will be able to switch to the NGS arm at the request of the investigator. Discussion The MULTISARC trial is a prospective study designed to provide high-level evidence to support the implementation of NGS in routine clinical practice for advanced STS participants, on a large scale. Trial registration clinicaltrial.gov NCT03784014 .

Aims: Cell-free DNA (cfDNA) and circulating tumour DNA (ctDNA) are used for prognostication and monitoring in patients with carcinomas, but their utility is unclear in sarcomas. The objectives of this pilot study were to explore the prognostic significance of cfDNA and investigate whether tumour-specific alterations can be detected in the circulation of sarcoma patients. Methods: Matched tumour and blood were collected from 64 sarcoma patients (n = 70 samples) prior to resection of the primary tumour (n = 57) or disease recurrence (n = 7). DNA was isolated from plasma, quantified, and analyzed for cfDNA. A subset of cases (n = 6) underwent whole exome sequencing to identify tumour-specific alterations used to detect ctDNA using digital droplet polymerase chain reaction (ddPCR). Results: Cell-free was present in 69 of 70 samples above 0.5 ng/ml. Improved disease-free survival was found for patients with lower cfDNA levels (90% vs 48% at one-year for ≤ 6 ng/ml and > 6 ng/ml, respectively; p = 0.005). Digital droplet PCR was performed as a pilot study and mutant alleles were detectable at 0.5% to 2.5% of the wild type genome, and at a level of 0.25 ng tumour DNA. Tumour-specific alterations (ctDNA) were found in five of six cases. Conclusion: This work demonstrates the feasibility and potential utility of cfDNA and ctDNA as biomarkers for bone and soft-tissue sarcomas, despite the lack of recurrent genomic alterations. A larger study is required to validate these findings. Cite this article: Bone Joint Res 2021;10(9):602–610.

Soft-tissue sarcomas (STS), with over 100 different histologic subtypes, are rare tumors that account for 1% of all adult malignancies. Immune checkpoint inhibitors (ICIs) display certain benefits in some subtypes, especially in undifferentiated pleomorphic sarcoma (UPS), alveolar soft part sarcoma (ASPS), and leiomyosarcoma (LMS). However, efficacy is difficult to predict. High tumor mutational burden (TMB-H) and programmed death-ligand 1 (PD-L1) expression are the strongest features associated with the efficacy of immunotherapy, although they are rarely found in STS patients. Until now, whether or not PD-L1 expression and TMB are related to the efficacy of immunotherapy has not been determined. In this study, we report data obtained from two STS patients, one ASPS and one UPS with a high TMB, that benefited from anlotinib combined with toripalimab following resistance to anlotinib monotherapy. A 26 year-old female patient was diagnosed with ASPS. PD-L1 was negative. Next generation sequencing (NSG) revealed ASPSCR1-TFE3 fusion and TMB-H. Following eight months of anlotinib monotherapy, the patient's disease progressed but continued to benefit from subsequent use of anlotinib combined with toripalimab for 19 months. Another 63 year-old male patient was diagnosed with UPS. PD-L1 was positive and NGS revealed TMB-H. Following 19 months of anlotinib monotherapy, the patient's disease progressed but continued to benefit from subsequent use of anlotinib combined with toripalimab. DFS is 23 months to follow-up time. The results presented are the first to report the relationship between TMB and the efficacy of immunotherapy in STS. Based on our results, we hypothesis that anlotinib combined with toripalimab is effective for the treatment of some advanced ASPS or UPS. TMB may be a potential predictive biomarker for ICI treatment and deserves additional study. [ABSTRACT FROM AUTHOR]

Soft-tissue sarcomas (STS), with over 100 different histologic subtypes, are rare tumors that account for 1% of all adult malignancies. Immune checkpoint inhibitors (ICIs) display certain benefits in some subtypes, especially in undifferentiated pleomorphic sarcoma (UPS), alveolar soft part sarcoma (ASPS), and leiomyosarcoma (LMS). However, efficacy is difficult to predict. High tumor mutational burden (TMB-H) and programmed death-ligand 1 (PD-L1) expression are the strongest features associated with the efficacy of immunotherapy, although they are rarely found in STS patients. Until now, whether or not PD-L1 expression and TMB are related to the efficacy of immunotherapy has not been determined. In this study, we report data obtained from two STS patients, one ASPS and one UPS with a high TMB, that benefited from anlotinib combined with toripalimab following resistance to anlotinib monotherapy. A 26 year-old female patient was diagnosed with ASPS. PD-L1 was negative. Next generation sequencing (NSG) revealed ASPSCR1-TFE3 fusion and TMB-H. Following eight months of anlotinib monotherapy, the patient’s disease progressed but continued to benefit from subsequent use of anlotinib combined with toripalimab for 19 months. Another 63 year-old male patient was diagnosed with UPS. PD-L1 was positive and NGS revealed TMB-H. Following 19 months of anlotinib monotherapy, the patient’s disease progressed but continued to benefit from subsequent use of anlotinib combined with toripalimab. DFS is 23 months to follow-up time. The results presented are the first to report the relationship between TMB and the efficacy of immunotherapy in STS. Based on our results, we hypothesis that anlotinib combined with toripalimab is effective for the treatment of some advanced ASPS or UPS. TMB may be a potential predictive biomarker for ICI treatment and deserves additional study.

Nodular fasciitis (NF) is a benign yet diagnostically challenging mesenchymal myofibroblastic proliferation that often mimics the histological features of inflammatory myofibroblastic tumors (IMTs) and soft tissue sarcomas. The overlap in histopathological appearance, compounded by the variability in immunohistochemical (IHC) staining, frequently leads to diagnostic uncertainty. In this report, we present a case of a rapidly expanding lesion on the left medial mandible, ultimately diagnosed as NF. Molecular analysis through fluorescence in situ hybridization (FISH) identified a ubiquitin-specific peptidase 6 (USP6; 17p13.2) gene rearrangement, a distinctive marker of NF, which played a critical role in confirming the diagnosis. IHC analysis, including negative staining for cytokeratin and ALK, further helped differentiate this benign entity from other IMTs and malignancies, highlighting the importance of combining molecular diagnostics with traditional histopathological techniques to ensure accurate classification and avoid misdiagnosis., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Coleman et al.)

William Coley was an unacclaimed hero of early cancer treatment. His work is often overshadowed by more recent advancements in immunotherapy. Coley's innovative work in the 1910s and 1930s laid the groundwork for what would become a major field in oncology. His experiments with bacterial vaccines by making use of the immune system to combat cancer preceded contemporary immunotherapy for several decades. This review provides a comprehensive exploration of Coley's life, his groundbreaking research, the socio-scientific challenges he faced, and his lasting impact on cancer treatment. Even though he faced lots of initial resistance and challenges, Coley's work has influenced modern immunotherapy practices., Competing Interests: Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Munir et al.)

Background: Soft-tissue sarcomas (STS) represent a heterogeneous group of rare tumors including more than 70 different histological subtypes. High throughput molecular analysis (next generation sequencing exome [NGS]) is a unique opportunity to identify driver mutations that can change the usual one-size-fits-all treatment paradigm to a patient-driven therapeutic strategy. The primary objective of the MULTISARC trial is to assess whether NGS can be conducted for a large proportion of metastatic STS participants within a reasonable time, and, secondarily to determine whether a NGS-guided therapeutic strategy improves participant's outcome.Methods: This is a randomized, multicentre, phase II/III trial inspired by the design of umbrella and biomarker-driven trials. The setting plans up to 17 investigational centres across France and the recruitment of 960 participants. Participants aged at least 18 years, with unresectable locally advanced and/or metastatic STS confirmed by the French sarcoma pathological reference network, are randomized according to 1:1 allocation ratio between the experimental arm "NGS" and the standard "No NGS". NGS will be considered feasible if (i) NGS results are available and interpretable, and (ii) a report of exome sequencing including a clinical recommendation from a multidisciplinary tumor board is provided to investigators within 7 weeks from reception of the samples on the biopathological platform. A feasibility rate of more than 70% is expected (null hypothesis: 70% versus alternative hypothesis: 80%). In terms of care, participants randomized in "No NGS" arm and who fail treatment will be able to switch to the NGS arm at the request of the investigator.Discussion: The MULTISARC trial is a prospective study designed to provide high-level evidence to support the implementation of NGS in routine clinical practice for advanced STS participants, on a large scale.Trial Registration: clinicaltrial.gov NCT03784014 . [ABSTRACT FROM AUTHOR]

Primary synovial sarcoma (PSS) of the kidney is considered the rarest type of all renal sarcomas with specific chromosomal translocation t (X; 18) (p11.2; q11.2). We report the case of a 65-year-old man with no medical conditions who presented to the emergency department with sudden severe right flank pain associated with haemodynamic instability and haemorrhagic shock. Computed tomography (CT) of the abdomen and pelvis revealed a right renal mass. A right open radical nephrectomy was performed. Histopathology revealed a monophasic synovial sarcoma. The patient received six cycles of docetaxel and gemcitabine as adjuvant chemotherapy. No sign of recurrence was seen on a follow-up CT urogram. This rare tumour often presents atypically, and clear guidelines regarding appropriate treatment are lacking. Our case showed that treatment with docetaxel/gemcitabine after an open radical nephrectomy is promising.

Abstract Background Desmoid tumors, also known as aggressive fibromatosis, are extremely rare, accounting for less than 3% of soft-tissue sarcomas and less than 0,03% of all neoplasms. The diagnosis is usually delayed because of the lack of specific symptoms, and can sometimes lead to serious and, even fatal complications. Case presentation We report the case of a 27-year-old male patient presenting with the clinical picture of acute appendicitis. During the operation, we found a tumor in the jejunum with a necrotic zone and perforation on its surface, causing hemorrhagic effusion into the abdominal cavity and subsequent peritonitis. The tumor was removed with negative margins via resection of the small bowel. The final histological result showed aggressive fibromatosis. Conclusions Aggressive fibromatosis remains a serious problem with the possibility of locally aggressive behavior with high rates of recurrence. Sometimes, its clinical and macroscopic recognition can be immensely tricky. As shown by our patient, on rare occasions, desmoid tumors can lead to acute surgical abdomen requiring an emergency operation.

Primary synovial sarcoma (PSS) of the kidney is considered the rarest type of all renal sarcomas with specific chromosomal translocation t (X; 18) (p11.2; q11.2). We report the case of a 65-year-old man with no medical conditions who presented to the emergency department with sudden severe right flank pain associated with haemodynamic instability and haemorrhagic shock. Computed tomography (CT) of the abdomen and pelvis revealed a right renal mass. A right open radical nephrectomy was performed. Histopathology revealed a monophasic synovial sarcoma. The patient received six cycles of docetaxel and gemcitabine as adjuvant chemotherapy. No sign of recurrence was seen on a follow-up CT urogram. This rare tumour often presents atypically, and clear guidelines regarding appropriate treatment are lacking. Our case showed that treatment with docetaxel/gemcitabine after an open radical nephrectomy is promising. [ABSTRACT FROM AUTHOR]

Soft-tissue sarcomas (STSs) are an uncommon and diverse group of cancers, consisting of more than 80 different kinds, each showing unique mesenchymal differentiation as described by the World Health Organization (WHO). The prognostic factors at the time of diagnosis mostly depend on the size, depth, and histological grade of the lymphatic involvement. Improved prognostic indicators are necessary to identify patients at high risk who may derive advantages from adjuvant therapy and those at low risk who might avoid treatment-related side effects. Over a period of five years, a considerable number of patients experience the recurrence of the tumor in the same area or the metastasis of the disease to other parts of the body, even after the complete removal of the localized tumor through surgery. To further personalize and focus medicines, it is critical to enhance prediction accuracy and uncover new therapy targets. This is particularly important considering the high mortality and morbidity rate associated with metastatic STS. The significant diversity of STS poses difficulty in comprehending its pathobiology and in converting biological progress into therapeutic application. This prospective cohort study was carried out at a major university hospital to ascertain adult patients who were diagnosed with STS of the extremities between the period from 2018 to 2023. The inclusion criteria were individuals who were 18 years of age or older with a histological diagnosis of STS. The exclusion criteria encompassed individuals with malignancies other than STS and those with inadequate data on essential factors. Thorough assessments were conducted to analyze patient demographics, tumor features (including site, size, depth, neurovascular or bone invasion), and histologic type and grade (according to the French Federation of Cancer Centers (FNCLCC) grading system). The purpose was to find predictive markers and evaluate results. The results are consistent with previous research and enhance our current knowledge of STS prognosis. Key prognostic markers for metastasis and mortality risk include tumor size larger than 5 cm, histologic grade, and sarcoma subtype. Radical surgical procedures, such as amputation or disarticulation, did not show any survival advantage, probably because they were used in situations where the disease had already progressed locally and had significant involvement in the blood vessels. Histologic grading has been identified as the most important factor in predicting the likelihood of metastasis in adult STSs. The study found that most tumors were of high grade, and there was a statistically significant association between tumor grade, Ki67 levels, and overall survival. A small proportion of patients experienced prolonged longevity beyond five years, emphasizing the connection between early detection, tumors of lesser severity, and enhanced results. These observations emphasize the significance of accurate prognostic assessments and customized therapeutic approaches in the treatment of STS., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. The Ethics Commission of the Bucharest University Emergency Hospital issued approval 43121. Animal subjects: All authors have confirmed that this study did not involve animal subjects or tissue. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Serban et al.)

Sarcomas are rare tumours arising from mesenchymal tissue. A multimodal management in an expert centre combining surgery and radiotherapy is the current standard of care for localized soft-tissue sarcomas of the extremities, to enable limb-sparing strategies. The delivery of pre- radiotherapy or postoperative radiotherapy offers similar local control and survival rates but the toxicity profile is quite different: preoperative radiotherapy increases the risk of wound complications and postoperative radiotherapy affects long-term functional outcomes. While postoperative radiotherapy has long been the rule, especially in Europe, technical improvements with image-guided- and intensity-modulated radiotherapy associated with a better management of postoperative wounds has tended to change practices with more frequent preoperative radiotherapy. More recently the possibilities of a hypofractionated regimen or potentiation by nanoparticles to increase the therapeutic index plead in favour of a preoperative delivery of radiotherapy. The aim of this paper is to report pros and cons of pre- and post-operative radiotherapy for soft-tissue sarcomas. [ABSTRACT FROM AUTHOR]

Surgery continues to remain an integral component of treatment, especially for nonrhabdomyosarcoma soft tissue sarcoma as compared to rhabdomyosarcoma owing to their general insensitivity to chemotherapy. A key determinant of outcomes, particularly for extremity tumors includes complete tumor resection with negative margins; however, a significant limitation for limb salvage surgery is the adherence of sarcomas to vital vascular structures. Hitherto, vascular involvement constituted an adequate reason for amputation. However, modern reconstructive techniques and availability of prosthetic grafts in addition to autologous venous grafts have rendered limb salvage surgery possible in a substantial majority of patients. Vascular resection and reconstruction for extremity soft tissue sarcoma in children have not been used routinely for reasons like the small-caliber of native vessels, limited options for conduits and rapid somatic growth. The situation is inconceivable in infants owing to the contemporaneous diminutive caliber of the vessels. We report two infants with lower extremity nonrhabdomyosarcoma soft tissue sarcoma who underwent limb salvage surgery with resection of femoral vessels following which vascular reconstruction was successfully performed using the great saphenous vein allograft harvested from the father. [ABSTRACT FROM AUTHOR]

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Objectives/hypothesis: We used the National Cancer Database to identify the patterns of care and prognostic factors in adult patients with head and neck soft-tissue sarcoma (HNSTS).Study Design: Retrospective cohort analysis.Methods: Using the National Cancer Database, we identified patients age ≥ 18 years who were diagnosed with HNSTS between 2004 and 2013. Both χ2 and multivariate logistic regression were used to identify factors associated with radiation therapy (RT) utilization. Kaplan-Meier methods were used to estimate overall survival (OS) and Cox proportional regression was used to determine significant contributors to OS.Results: Our final cohort included 1,282 patients (682 treated with surgery only, 199 treated with RT only, and 401 treated with surgery and RT). Patients with younger age, poor tumor grade, rhabdomyosarcoma histology, and chemotherapy treatment were more likely to receive RT alone without surgery. Among the 1,083 surgical patients, RT utilization was associated with positive margins (odds ratio [OR]: 2.18, 95% confidence interval [CI]: 1.36-3.48), poor grade (OR: 2.92, 95% CI: 1.95-4.38), and chemotherapy use (OR: 1.78, 95% CI: 1.15-2.76). Radiotherapy utilization among surgical patients was not affected by demographic factors (age, sex, or ethnicity) or treatment institution (academic or community). For surgical patients, poor grade, large tumor size, and rhabdomyosarcoma histology were associated with worse OS on multivariate analysis.Conclusions: In this analysis of HNSTS, younger patients with poor tumor grade and rhabdomyosarcoma histology were more likely to receive RT without surgery. Among surgical patients, adjuvant RT was more likely to be used for positive margins and poor grade, with no demographic disparities identified. Poor grade and rhabdomyosarcoma histology were negative prognostic factors for surgical patients.Level Of Evidence: NA Laryngoscope, 130:120-127, 2020. [ABSTRACT FROM AUTHOR]

Background: Desmoid tumors, also known as aggressive fibromatosis, are extremely rare, accounting for less than 3% of soft-tissue sarcomas and less than 0,03% of all neoplasms. The diagnosis is usually delayed because of the lack of specific symptoms, and can sometimes lead to serious and, even fatal complications.Case Presentation: We report the case of a 27-year-old male patient presenting with the clinical picture of acute appendicitis. During the operation, we found a tumor in the jejunum with a necrotic zone and perforation on its surface, causing hemorrhagic effusion into the abdominal cavity and subsequent peritonitis. The tumor was removed with negative margins via resection of the small bowel. The final histological result showed aggressive fibromatosis.Conclusions: Aggressive fibromatosis remains a serious problem with the possibility of locally aggressive behavior with high rates of recurrence. Sometimes, its clinical and macroscopic recognition can be immensely tricky. As shown by our patient, on rare occasions, desmoid tumors can lead to acute surgical abdomen requiring an emergency operation. [ABSTRACT FROM AUTHOR]

Purpose: To explore the role of intratumoral heterogeneity on MRI assessed by histogram analysis in differentiating soft-tissue sarcomas (STS) of different grades.Materials and Methods: Patients with primary STS undergoing MRI prior to iatrogenic procedures were included retrospectively. The histologic grade was assigned according to Federation Nationale des Centres de Lutte Contre le Cancer grading system. T1WI and T2WI were normalized by dividing mean signal intensity (SI) of contralateral/near unaffected muscles. Contrast-enhanced T1WI was normalized by computing enhancement ratio (ER) map as (SIpost-SIpre)/SIpre×100, where SIpre and SIpost represent SI of each pixel before and after enhancement. A region of interest (ROI) was manually drawn to include entire tumor area on axial slice with largest tumor diameter. Mean, mode, standard deviation, kurtosis and skewness on ROIs were extracted with ImageJ software. ANOVA/Kruskal-Wallis test was used to determine the significance of differences. ROC curve was applied for statistically significant parameters. P value ≤0.05 was considered statistically significant.Results: Among involved 67 patients, 8 were assigned to grade 1, 38 to grade 2 and 21 to grade 3. Skewness (P =  0.022) and kurtosis (P =  0.035) on ER maps were significantly different among STS of different grades. The optimal cutoffs of skewness and kurtosis on ER maps were -0.488 (AUC[95% CI] 0.747[0.557-0.937]; sensitivity/specificity, 62.5%/86.4%) and 0.762 (AUC[95% CI] 0.684[0.548-0.821]; sensitivity/specificity, 76.2%/56.5%), respectively.Conclusion: Intratumoral heterogeneity on MRI quantitatively displayed by histogram parameters can differentiate STS of different grades. Skewness and kurtosis on ER maps show the capacity. [ABSTRACT FROM AUTHOR]

Background: Extremity soft-tissue sarcomas (STS) are commonly treated with neoadjuvant radiation therapy followed by surgical resection. However, the pathological near-complete response rate is low (9-25%). Noninvasive imaging assessment that predicts treatment response before and during treatment is desirable to optimize treatment regimens. This pilot study aimed to investigate the application of a quantitative MRI parameter, T 2 *, in assessing neoadjuvant radiation therapy combined with pharmacological ascorbate in extremity STS., Methods: This prospective cohort study included seven patients diagnosed with extremity STS and scheduled to receive neoadjuvant radiation therapy combined with pharmacological ascorbate. T 2 * maps were obtained from each patient before treatment (baseline MRI), two weeks after initiating treatment (on-treatment MRI), and before surgery (pre-surgery MRI). The T 2 * values within the tumor region were transformed into z-scores with respect to the normal- appearing tissue region. The voxel-wise z-scores within the tumor region were thresholded to generate masks representing significantly high (z-score>1.96) and low z-score (z-score<-1.96) voxels. The means of the total z-scores and within each of the significantly high and low z-score mask were computed. Their correlations with percent necrosis from pathological examination were evaluated using Spearman's rank correlation coefficient r. A correlation was considered as moderate or strong when r is higher than 0.6 and 0.8, respectively. A correlation was considered as fair or weak when r is below 0.6., Results: For the baseline and on-treatment MRIs, the means of the significantly high z-scores of the T 2 * measurements showed moderate correlations with percent necrosis (r = 0.68 and 0.6; p = 0.11 and 0.24). For the pre-surgery MRI, the means of the total and significantly high z-scores showed strong correlations with percent necrosis (r = 0.8 and 0.9; p = 0.13 and 0.08). Tumor volume and baseline MRI-based percent necrosis showed fair or weak correlations (r = 0.3-0.54; p = 0.24-0.68)., Conclusion: T 2 * measurements prior to treatment, two weeks after initiating treatment, and before surgery showed moderate to strong correlations with percent necrosis. These results support the potential for using T 2 * mapping to predict and assess response to neoadjuvant radiation therapy combined with pharmacological ascorbate in extremity STS. Level of Evidence: IV ., Competing Interests: Disclosures: The authors report no potential conflicts of interest related to this study., (Copyright © The Iowa Orthopaedic Journal 2023.)

This article proposes a summary of the current status of the research regarding the use of radiomics and artificial intelligence to improve the radiological assessment of patients with soft tissue sarcomas (STS), a heterogeneous group of rare and ubiquitous mesenchymal malignancies. After a first part explaining the principle of radiomics approaches, from raw image post-processing to extraction of radiomics features mined with unsupervised and supervised machine-learning algorithms, and the current research involving deep learning algorithms in STS, especially convolutional neural networks, this review details their main research developments since the formalisation of 'radiomics' in oncologic imaging in 2010. This review focuses on CT and MRI and does not involve ultrasonography. Radiomics and deep radiomics have been successfully applied to develop predictive models to discriminate between benign soft-tissue tumors and STS, to predict the histologic grade (i.e., the most important prognostic marker of STS), the response to neoadjuvant chemotherapy and/or radiotherapy, and the patients' survivals and probability for presenting distant metastases. The main findings, limitations and expectations are discussed for each of these outcomes. Overall, after a first decade of publications emphasizing the potential of radiomics through retrospective proof-of-concept studies, almost all positive but with heterogeneous and often non-replicable methods, radiomics is now at a turning point in order to provide robust demonstrations of its clinical impact through open-science, independent databases, and application of good and standardized practices in radiomics such as those provided by the Image Biomarker Standardization Initiative, without forgetting innovative research paths involving other '-omics' data to better understand the relationships between imaging of STS, gene-expression profiles and tumor microenvironment., Competing Interests: Declaration of Interests The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2023 Société française de radiologie. Published by Elsevier Masson SAS. All rights reserved.)

Patients with unresectable advanced soft-tissue sarcomas (STS) receiving radiotherapy or/and chemotherapy still have a poor prognosis. This study aimed to evaluate retrospectively the efficacy and safety of recombinant adenovirus-p53 (rAd-p53) gene therapy combined with radiotherapy and hyperthermia for advanced STS. A total of 71 patients with advanced unresectable STS treated at the authors' center from April 2007 to November 2014 were included. Of these 71 patients, 36 cases received rAd-p53 therapy combined with radiotherapy and hyperthermia (p53 group), while 35 cases received radiotherapy and hyperthermia alone (control group). Short-term therapeutic efficacies, long-term survival outcomes, and adverse events were evaluated and compared between groups. Compared to the control group, the p53 group had a significantly higher disease control rate (83.33% vs. 54.29%; p = 0.008) and a lower progressive disease rate (16.67% vs. 45.71%; p = 0.018). In addition, rAd-p53 treatment significantly improved the progression-free survival and overall survival of STS patients. Cox regression indicated that rAd-p53 treatment significantly reduced the risks for disease progression or death event for STS patients. Furthermore, there was no significant difference in all adverse events, except for transient fever, which occurred in 89% of patients with rAd-p53 therapy. rAd-p53 combined with radiotherapy and hyperthermia can effectively improve the therapeutic efficacy and survival outcomes in patients with advanced unresectable STS, providing a new therapeutic strategy. [ABSTRACT FROM AUTHOR]

This article provides an overview of the current knowledge regarding diagnostic imaging of patients with soft-tissue sarcomas, which is a heterogeneous group of rare mesenchymal malignancies. After an initial contextualization, diagnostic flow-chart based on initial radiological findings of soft-tissue masses (with specific focus on adipocytic soft-tissue tumors [STTs], hemorragic STTs and retroperitoneal STTs) are provided considering relevant results from novel researches, guidelines, and experts' viewpoints, with the aim to help radiologists and clinicians in their practice. Particularly, the central place of sarcoma reference centers in the diagnostic and therapeutic management is highlighted, as well as the pivotal role that radiologists should play to correctly identify patients with soft-tissue sarcoma at the initial stage of the disease. Indications and methods for performing imaging-guided biopsies are also discussed, as well as clues to improve soft-tissue sarcoma grading with conventional and quantitative imaging., Competing Interests: Declaration of Competing Interest The authors declare that they have no competing interest in relation with this article., (Copyright © 2022 Société française de radiologie. Published by Elsevier Masson SAS. All rights reserved.)

Background: Often, it is difficult to assess the presence of residual disease after an unplanned excision in soft-tissue sarcomas. Inadequate excision leads to disease recurrence and inferior oncological outcomes while unnecessary excision may lead to additional surgical procedures with inherent morbidity and increased cost of treatment. There is a paucity of literature comparing the preoperative imaging findings with the final histopathology report to accurately assess the presence of residual disease.Materials and Methods: The clinical details of 55 patients who had oncological scar excision after unplanned prior excision were retrieved. Histopathological evaluation of scar was compared with presurgery magnetic resonance imaging (MRI) for the presence of residual disease. Sensitivity, specificity, and positive and negative predictive value (NPV) of MRI for detection of residual disease were calculated.Results: On MRI, residual disease was seen in 28 cases, no disease in 24 cases whereas findings of three patients were indeterminate. On final histopathology, residual disease was present in 30 (55%) patients whereas no residual tumor was seen in 25 (45%) patients. Two patients in whom MRI suggested the presence of residual disease had no tumor on final histopathology. No evidence of residual disease was reported in MRI of 24 patients. Of these, twenty patients were confirmed to have no tumor on final histopathology, whereas four patients had a residual tumor. Sensitivity: 86.66%, specificity: 90.90%, positive predictive value (PPV): 92.85%, NPV: 83.33%.Conclusion: MRI can aid in preoperative planning by identifying the site and extent of the previous surgery. It has a high PPV (92%) for detection of residual disease. However, a negative scan (NPV 83%) does not reliably exclude the presence of residual disease. [ABSTRACT FROM AUTHOR]