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Objectives: The aim of this study is to develop an ultrasound-based fusion model of clinical, radiomics and deep learning (CRDL) for accurate diagnosis of benign and malignant soft tissue tumors (STTs) Methods: In this retrospective study, ultrasound images and clinical data of patients with STTs from two hospitals were collected between January 2021 and December 2023. Radiomics features and deep learning features were extracted from the ultrasound images, and the optimal features were selected to construct fusion models using support vector machines. The predictive performance of the model was evaluated based on three aspects: discrimination, calibration and clinical usefulness. The DeLong test was used to compare whether there was a significant difference in AUC between the models. Finally, two radiologists who were unaware of the clinical information performed an independent diagnosis and a model-assisted diagnosis of the tumor to compare the performance of the two diagnoses. Results: A training cohort of 516 patients from Hospital-1 and an external validation cohort of 78 patients from Hospital-2 were included in the study. The Pre-FM CRDL showed the best performance in predicting STTs, with area under the curve (AUC) of 0.911 (95%CI: 0.894-0.928) and 0.948 (95%CI: 0.906-0.990) for training cohort and external validation cohort, respectively. The DeLong test showed that the Pre-FM CRDL significantly outperformed the clinical models (P< 0.05). In addition, the Pre-FM CRDL can improve the diagnostic accuracy of radiologists. Conclusion: This study demonstrates the high clinical applicability of the fusion model in the differential diagnosis of STTs. [ABSTRACT FROM AUTHOR]

Introduction: Activated vascular endothelial growth factor receptors (VEGFRs), platelet-derived growth factor receptors (PDGFRs) and c-Kit have been shown to be involved in the growth, invasion and metastasis of non-rhabdomyosarcoma soft tissue sarcoma tumor (NRSTS) with promising results for targeted therapy. Our aim was to assess the expression of these markers among different histological types and correlate with outcomes. Material and methods: This retrospective study included pediatric patients aged ≤ 18 years diagnosed with high-grade NRSTS who were treated at Children Cancer Hospital Egypt 57357 as per the COG NRSTS protocol (ARST0332). Expression of VEGFR2, PDGFRs (α and β) and c-Kit in tumor tissue was assessed by immunohistochemistry and correlated with clinical outcome. Results: Of 113 patients, 96 were eligible for the analysis with a median age of 11 years. Overall, 32.3% demonstrated high expression of PDGFRα, 17.7% for PDGFRβ, 19.8% for VEGFR2 and 8.3% exhibited positive expression for c-kit on the tumor cells. Most cases of synovial sarcoma (45.8%) and 43.7% of patients with undifferentiated sarcoma exhibited high expression of PDGFRα while 41.6% of MPNST showed high expression to PDGFRβ. The 5-year overall survival (OS), event free survival and relapse free survival (RFS) for the whole cohort were 59%, 54% and 60% respectively. In univariate analyses, only PDGFRα had a negative prognostic impact on relapse free survival (RFS) (p =0.03). In multivariate analyses, VEGFR2 was found to have a negative prognostic impact for OS (p = 0.02). Conclusion: Our findings indicated that tyrosine kinase receptors are upregulated in NRSTS and exhibited a distinct expression pattern within various subgroups. High expression of VEGFR2 and PDGFRα significantly correlated with reduced survival and may guide targeted therapy approaches for this poor prognosis group of patients. [ABSTRACT FROM AUTHOR]

Background and Objectives: This study aims to translate and culturally adapt the Toronto Extremity Salvage Score (TESS) for Latin American Spanish–speaking patients, enhancing the tool's accessibility for evaluating postsurgical functional outcomes in sarcoma patients across Latin America. Methods: The TESS questionnaires for lower extremity (LE) and upper extremity (UE) were translated and adapted following international guidelines. The process included forward and backward translation, expert committee review, and pretesting with cognitive interviewing. Patients treated for bone or soft tissue tumors in LE or UE were recruited to complete the adapted questionnaires. Test–retest reliability was evaluated by having participants complete the questionnaire again 2 weeks after the initial assessment. Results: A total of 89 participants completed the questionnaires. The study found high internal consistency, with Cronbach's alpha values reaching 0.9437 for LE and 0.9402 for UE. An agreement rate of 98.4% for the global score of TESS‐LE (95% confidence interval [CI]: 0.909–1.059) and 93.9% for TESS‐UE (95% CI: 0.882–0.995) was observed, demonstrating strong test–retest reliability. Conclusions: The Latin American Spanish version of TESS for both lower and upper extremities is a reliable and culturally appropriate tool for assessing physical function in limb sarcoma patients. Further validation across diverse Latin American populations is encouraged to strengthen its broad applicability. [ABSTRACT FROM AUTHOR]

Objective: Increased survival rates in childhood cancer have led to an emphasis on the importance of treatment-related infertility. Fertility preservation methods should be explained to every patient and their families (PaFs) before treatment. Establishing good communication with PaFs is crucial in this regard despite many barriers such as cultural and financial barriers. Routine feasibility of sperm preservation (SP) in adolescent males newly diagnosed with cancer was evaluated after the implementation of reimbursement for the procedure and storage by the national healthcare system. Materials and Methods: Males <18 years of age planned to undergo cancer treatment between 2021 and 2023 were included. Patient and their families were informed by the treating physician about the disease, treatment modality, side effects, the importance of SP, and the method. Information about the purpose and technique of SP was provided to the patient alone in a comfortable environment. Questions from PaFs were answered, and consent was obtained. The procedure and storage cost was covered by the social security institution. Results: Seventeen patients (median age 15) (15 with bone/soft tissue sarcoma, 1 brain tumor, and 1 Hodgkin lymphoma) were included. There were no refusals for SP. Eleven patients were able to provide sperm samples. Of the 6 patients who could not provide sperm, 3 stated embarrassment, 2 anxiety, and 1 cancer-related pain. Conclusion: Increasing awareness among physicians about the importance of treatment-related infertility, allocating sufficient time to inform PaFs about fertility preservation, and providing information taking into account societal or socio-cultural factors will contribute to preventing treatment-related infertility. Establishing close communication with the Reproductive Health Center (RHC), along with the rapid and cost-free nature of the procedure, enhances success. [ABSTRACT FROM AUTHOR]

Head and neck cancers (HNCs) arise from anatomically adjacent sites and subsites, with varying etiological factors, diagnostic strategies, prognoses, and treatment approaches. While conventional squamous cell carcinoma (SCC) is the most common histology in the head and neck district, HNCs encompass a variety of rare histopathological entities, categorized into epithelial tumors such as salivary gland cancers, sinonasal tumors, neuroendocrine tumors, malignant odontogenic tumors, and SCC variants versus non-epithelial tumors including soft tissue sarcomas, mucosal melanomas, and hematological malignancies. Rare HNCs (R-HNCs) represent a diagnostic and clinical challenge, requiring histopathological expertise, the availability of peculiar molecular analysis, and the personalization of local and systemic treatments, all guided by a multidisciplinary tumor board. Here, we provide a comprehensive literature review on R-HNCs, emphasizing key histopathological and molecular characteristics that are crucial for guiding treatment decisions. An insight about the latest developments in systemic treatments is also reported. [ABSTRACT FROM AUTHOR]

Simple Summary: This study examines how care pathways impact local recurrence (LR) rates in patients with soft tissue sarcomas (STS). It compares outcomes between those managed entirely within a comprehensive care pathway (CCP) at the Swiss Sarcoma Network (SSN) and those with a fragmented care pathway (FCP) where initial treatment occurred outside specialized centers. Patients in FCPs had higher LR rates, unplanned "whoops" resections, and positive surgical margins, highlighting the critical role of referral patterns and early, coordinated care. The findings underscore the need for better education and standardized early referrals to improve outcomes and establish quality benchmarks in specialized sarcoma care. This study evaluated the impact of care pathways on the incidence of local recurrence (LR) in patients with soft tissue sarcomas (STS) and identified factors predictive of LR. It compared outcomes between patients managed entirely within a comprehensive care pathway (CCP) at the Swiss Sarcoma Network (SSN) and those who experienced fragmented care pathways (FCPs), where initial treatment occurred outside specialized centers. This prospective study utilized real-world-time data from the SSN-Sarconnector, capturing quality indicators through weekly Multidisciplinary Team/Sarcoma-Board (MDT/SB) meetings. The overall incidence of LR was 17.6% (n = 68/386), higher than rates typically reported in sarcoma center-based studies due to the inclusion of patients with prior inadequate management from real-world referrals. In a univariable logistic regression analysis, the FCP was significantly associated with higher LR rates, unplanned "whoops" resections (25.4%, n = 96), and positive surgical margins, emphasizing the detrimental impact of suboptimal initial management outside of specialized centers. Multivariable analysis confirmed that the FCP (aOR 2.7, 95% CI [1.41, 5.12], p = 0.003), tumor size (aOR 1.49, 95% CI [1.1, 2.02], p = 0.01), and biological behavior (aOR 5.84 95% CI [1.8, 18.86], p = 0.0003) are independent predictors of LR. Notably, patients referred to sarcoma centers after an initial FCP presented with inadequately managed disease, such as incomplete resections and unplanned surgeries, leading to increased complexity of subsequent treatments. These findings underscore the critical role of referral patterns on sarcoma center outcomes, highlighting the significant disparity in LR rates between institutions. The need for improved education and standardized early referral strategies at the spoke level is paramount to optimize patient outcomes and reduce the burden of LR. Enhanced spoke-level education and standardized referral protocols are critical to ensuring effective initial management and optimizing patient outcomes within specialized sarcoma networks like the SSN. [ABSTRACT FROM AUTHOR]

Simple Summary: Surgical metastasectomy has been the primary choice for local therapy in patients with lung metastasis secondary to sarcoma. Over recent decades, however, stereotactic body radiation therapy (SBRT) has been employed more extensively in the treatment of lung metastasis. SBRT is associated with less toxicity and similar outcomes compared with surgical metastasectomy. When used with chemotherapy and surgery in a multimodal treatment plan, SBRT can improve the cure rate and prolong survival. Background: Stereotactic body radiotherapy (SBRT) is increasingly used to treat lung metastasis (LM) in patients with soft tissue sarcoma (STS) and bone sarcoma (BS). Methods: This retrospective study evaluated the outcomes of patients with BS and STS treated with SBRT for LM between 2010 and 2023. Results: We enrolled 102 patients (51 each with STS and BS), of whom 71 were males and 31 were females (median age, 40 years; range, 11–81 years). At diagnosis, 76 and 26 patients had localized and metastatic disease, respectively, with a median of 4 recurrences (range, 1–12). Before SBRT, 75 patients received chemotherapy and 52 underwent surgery for LM, with 276 nodules treated with SBRT (median dose, 48 Gy; range, 40–52). Local control of irradiated LM was 86% at 1 year and 78% at 2 years. By 31 December 2023 (median follow-up, 4.8 years), 60 patients had died and 42 survived (20 without ongoing disease). From the first LM relapse, the median overall survival (OS) was 4.8 years and the 5-year OS was 49% (95% confidence interval, 39–60%), with no difference between STS and BS; the median OS was 2.9 years and the 5-year OS was 36% after SBRT. Chemotherapy before SBRT was a negative prognostic factor by multivariate analysis. Conclusions: Long-term follow-up shows that SBRT as part of a multimodal treatment approach has reasonable survival rates in patients with LM due to sarcoma. Compared with historical results using only surgery and chemotherapy, SBRT has improved the 5-year OS. [ABSTRACT FROM AUTHOR]

Bladder embryonal rhabdomyosarcomas (ERMS) are malignant soft tissue tumors that rarely present in the perinatal period. Herein, we report 2 cases that were diagnosed in the newborn period (one with concomitant posterior urethral valves [PUV]), managed with chemotherapy and surgical resection to minimize the need for radiation. [ABSTRACT FROM AUTHOR]

Mantle cell lymphoma (MCL) is frequently diagnosed at advanced stages and is characterized by multiple extranodal sites of disease, most notably the bone marrow, peripheral blood, and gastrointestinal tract. Historically the prognosis of mantle cell lymphoma has been poor with median survival of four to five years. With new treatment regimens, however, patients have been able to achieve prolonged remissions and require special attention while being evaluated for relapse. This report describes four patients treated for stage IV mantle cell lymphoma at the University of Miami who developed soft tissue relapse presenting as non-tender large masses of the extremities, including one patient who presented without associated nodal involvement. Average time to soft tissue relapse was 99 months (range: 28–240) following initial diagnosis. Providers who care for patients with mantle cell lymphoma should be aware of soft tissue lesions as a presentation of mantle cell lymphoma that merits evaluation for disease relapse. [ABSTRACT FROM AUTHOR]

Objective: This study aimed to assess the complication rate during and 24 hours after cryoablation in lung, bone, and soft tissue tumors. Methods: We reviewed complications in a total of 85 consecutive patients who underwent cryoablation using a liquid nitrogen-based system in various lesions between April 2017 and October 2022. There were no liver and renal lesions. Complications were categorized using the Society of Interventional Radiology classification. Results: Eighty-five patients were treated for 96 lesions in the bone (36.4%; 35 of 96), lung (18.8%; 18 of 96), and soft tissue (44.8%; 43 of 96). The primary technical success rate was 97.7% (83 of 85). The total grade 2 and 1 complication rates were 5.2% (5/96) and 20.8% (20/96), respectively. Two patients had asymptomatic pulmonary embolisms incidentally noted at the 24-hour follow-up computed tomography (grade 2). The most frequent complications were simple and hemorrhagic pleural effusions (18.7%; 18 of 96). Lung procedures had the highest complication rate, where 13 patients (72.2%; 13 of 18) reported complications, including 2 cases of symptomatic hydropneumothorax requiring drainage (grade 2) and an additional 2 days of hospital stay. Eight patients (24.2%; 8 of 33) with bone lesions and 4 (9.3%; 4 of 43) with soft tissue lesions experienced complications. Conclusion: Cryoablation using a liquid nitrogen-based system is safe, with only minor complications observed. Advances in knowledge: This study provides data on the safety of liquid nitrogen-based percutaneous cryoablation in tumors located in lung, in bones and in soft tissues. Despite using larger diameter cryoprobes than those typically reported with argon-based system, our experience shows that complications are mostly low and comparable in frequency and severity. [ABSTRACT FROM AUTHOR]

This article reports on two cases of testicular sclerosing stromal tumors (SSTs) that show GLI1 gene alterations. SSTs are neoplasms that were first described in the ovary in 1973 and are characterized by lobulated microscopic appearance. The patients in these cases were aged 35 and 65 years and presented with testicular masses. The tumors demonstrated large areas of hypocellular myxoid/oedematous stroma with scattered hypercellular areas. Both tumors expressed nuclear GLI1. The article suggests that these findings support the existence of rare SSTs in the testis and their potential pathogenesis related to ovarian SSTs. [Extracted from the article]

PRRX1‐fused mesenchymal neoplasm is a recently identified, rare subcutaneous soft tissue neoplasm that is characterized by fusion of PRRX1 (exon 1) with NCOA1 (exon 13) in the majority of reported cases. Although initially considered to be fibroblastic, a possibility of neural or neuroectodermal differentiation has been suggested in a subset of cases. We report a 26‐year‐old female with a 4.0 cm painless mass located in the subcutis of the left thigh. Microscopically, the tumor was well‐circumscribed and multinodular and was composed of relatively monomorphic ovoid to spindle cells arranged in loose fascicles, trabeculae, and cords within alternating myxoid and fibrous matrix, and vascularized stroma. Mitotic figures were scarce and necrosis was not observed. By immunohistochemistry, the neoplastic cells demonstrated focal co‐expression of S100 protein and SOX10 and were negative for epithelial membrane antigen, smooth muscle actin, desmin, CD34, STAT6, HMB45, Melan‐A, and MUC4. The expression of Rb1 was retained. Targeted RNA‐sequencing identified a novel transcript fusion of PRRX1 (exon 1)::NCOA1 (exon 15), which was further confirmed by reverse transcription polymerase chain reaction and Sanger sequencing. The tumor was narrowly excised and no tumor recurrence or metastasis was identified after 13 months of follow‐up. In summary, we report a new case of PRRX1‐fused mesenchymal neoplasm, expanding the molecular genetic spectrum and providing further support for possible neural or neuroectodermal differentiation of this emerging soft tissue tumor entity. [ABSTRACT FROM AUTHOR]

The AMSIG Annual Scientific Meeting 2024 was held in Noosa, Queensland, with both in-person and online attendees. The meeting focused on imaging techniques for athletes and featured presentations from local and international speakers. Topics covered included muscle and bony injuries, interventions for athletes, and injury management in professional athletes. The meeting also included research talks and electronic posters, with the Best E-Poster Presentation Prize awarded to Dr. Dong Yu for his work on combining CT and MRI in spine tumor surgery planning. The document accompanying the meeting discusses various topics related to sports injuries and their management, including ACL injuries, stress injuries in female athletes, and hip morphology in women. It also covers topics such as ACL reconstruction, follow-up imaging for lower limb muscle injuries, pain management in shoulder injuries, and interdisciplinary pain management for lower limb injuries. The document presents clinical scenarios and discusses conservative measures, image-guided therapies, and operative management options for each case. The panel of experts engages with the audience in a healthy and entertaining manner. [Extracted from the article]

This document discusses a case of a painless knee mass in a young adult. The MRI showed a soft tissue lesion in the posterior recess of the knee, closely related to the anterior cruciate ligament. A provisional diagnosis of a complex ACL ganglion was made based on the MRI findings. However, an aspiration biopsy revealed a synovial sarcoma, a rare and aggressive soft tissue sarcoma. The document emphasizes the importance of histopathological analysis in cases of ganglion aspiration to avoid misdiagnosis. The management of synovial sarcoma involves surgical resection and, in some cases, radiotherapy or neoadjuvant chemoradiotherapy. [Extracted from the article]

This article discusses a case study of a young female patient with a painful mass in the quadriceps compartment. The diagnosis was fibro-adipose vascular anomaly (FAVA), a rare condition characterized by intramuscular replacement with fibro-fatty tissue, complex vascular malformation, venous thrombosis, and lymphatic involvement. The article presents various imaging findings, including radiographs, ultrasounds, MRIs, and CT scans, to support the diagnosis. The treatment options for FAVA include surgical removal, minimally invasive ablative procedures, and the use of sirolimus. The article emphasizes the importance of collaboration among specialists in diagnosing and managing FAVA. [Extracted from the article]

A glomus tumor is a benign mesenchymal tumor comprised of cells that resemble the perivascular modified smooth muscle cells of the glomus body. Glomus tumors typically appear in the superficial lesions of the soft tissue in the extremities, such as the subungual region. However, their occurrence in the bone is rare, with only about 30 cases reported to date. Half of these cases involved the distal phalanges of the fingers or toes, with only three reported cases involving the long bones. Here, we present the first case, a primary glomus tumor in the humerus of a 14-year-old female. An osteolytic and cystic lesion was detected after a pathological fracture occurred during exercise. Despite the tumor's large size, no pathological findings indicated malignancy. The fracture healed through conservative treatment, while the tumor was effectively managed with curettage. Appropriate medical care can be provided to patients by focusing on pathological findings. [ABSTRACT FROM AUTHOR]

Liposarcoma is the commonest soft tissue neoplasm, usually located in the deep soft tissue of the limbs. The central nervous system (CNS) as the primary site is exceedingly rare. We report a 35-year-old male patient who presented with seizure and weakness of the left half of the body for three months. The magnetic resonance imaging of the brain revealed a large solid partially necrotic lesion in the right posterior frontal lobe with perilesional edema. The tumour was firm, intra-axial, not adherent to the dura, and was completely excised. Based on the morphology, immunohistochemistry, and imaging findings, the diagnosis of primary intracerebral pleomorphic liposarcoma was rendered. Post-operatively, the patient had an uneventful follow-up. Pleomorphic liposarcoma is a rare CNS tumor with aggressive behavior, high potential for local recurrence, and distant metastasis. However, the acquaintance with intracranial liposarcoma is limited because of its rarity, and thus, no standard treatment protocol is of consensus. [ABSTRACT FROM AUTHOR]

Introduction: Heterotopic ossification (HO), acquired or hereditary, is a diverse pathological condition defined by the production of extraskeletal bone in muscles, soft tissues, and connective tissues. Acquired HO is relatively prevalent and develops mostly in response to trauma, although its etiology is unknown. Genetic forms provide insight into the pathobiological mechanisms of this disorder. Fibrodysplasia ossificans progressiva (FOP) is a rare hereditary form of HO that can have a significant impact on affected individuals. FOP steadily weakens affected subjects and increases their risk of death. Areas covered: The U.S. Food and Drug Administration has recently approved the retinoid palovarotene as the first compound to treat heterotopic ossification in patients with FOP. This review provides a comprehensive overview of current and potential future pharmacotherapeutic options and their modes of action. The online databases PubMed, Cochrane Library, Web of Science, and ClinicalTrials.gov were searched using the terms 'heterotopic ossification' and 'fibrodysplasia ossificans progressiva' or synonyms, with a special focus over the last 5 years of publications. Expert opinion: Approval of palovarotene, as the first retinoid indicated for reduction in the volume of new HO, may revolutionize the therapeutic landscape. However, long-term safety and efficacy data for palovarotene are currently lacking. [ABSTRACT FROM AUTHOR]

The document discusses the increasing prevalence of transpleural inferior phrenic artery to pulmonary artery fistula (IPA-PAF), a rare but important vascular anomaly often detected incidentally on routine CT scans. The cases presented highlight the radiological features of IPA-PAFs and emphasize the need for recognition by medical professionals to avoid misdiagnosis and inappropriate treatments. The text also mentions the potential complications of IPA-PAFs, such as hemoptysis, aneurysmal changes, and right heart failure, and suggests conservative management for asymptomatic cases. Advanced imaging techniques like CT angiography are recommended for accurate diagnosis and evaluation of IPA-PAFs. [Extracted from the article]

Myofibroblastomas are benign mesenchymal tumors that frequently occur in the groin. They show variable morphology, and the differential histopathological diagnoses are broad, including lipomatous to myxoid tumors. In addition, both pathological and imaging findings may be complex, which makes diagnosis challenging. We herein present a case of a mixed-variant myofibroblastoma of the wrist in a 73-year-old woman. Considering the long clinical course of more than 20 years and the imaging findings, a benign myxoid tumor including a schwannoma was suspected; however, the histopathological findings from resected specimens suggested a diagnosis of myxofibrosarcoma. Additional histopathological findings led to a diagnosis of mixed-variant myofibroblastoma. The differential diagnosis of myofibroblastoma extends beyond imaging to pathological findings because of the number of possible variants. This case reinforces the notion that the gold standard treatment for soft tissue tumors is to perform surgery only after determining the correct diagnosis by biopsy. [ABSTRACT FROM AUTHOR]

CREB gene family (ATF1, CREB1, CREM) fusions with either EWSR1 or FUS gene partners drive the pathogenesis of a wide range of neoplasms, including various soft tissue tumors, intracranial myxoid mesenchymal tumors (IMMTs), hyalinizing clear cell carcinoma (HCCC), and rare mesotheliomas. Recently, a SMARCA2::CREM fusion was reported in one case each of IMMT and HCCC. In this study, we expand the clinicopathologic and molecular spectrum of these neoplasms by describing three additional cases with SMARCA2::CREM and one with a novel SMARCA4::CREM fusion, highlighting the recurrent potential of additional CREB gene fusion partners beyond FET family members. To evaluate if these fusions define a new pathologic entity, we performed a comprehensive genomic and methylation analysis and compared the results to other related tumors. Tumors occurred in children and young adults (median age 20 years) and spanned a broad anatomic distribution, including soft tissue, intracranial, head and neck, and prostatic urethra. Microscopically, the tumors shared an undifferentiated round to epithelioid cell phenotype and a hyalinized fibrous stroma. Immunohistochemically, a polyphenotypic profile was observed, with variable expression of SOX10, desmin, and/or epithelial markers. No targetable genomic alterations were found using panel‐based DNA sequencing. By DNA methylation and transcriptomic analyses, tumors grouped closely to FET::CREB entities, but not with SMARCA4/SMARCB1‐deficient tumors. High expression of CREM by immunohistochemistry was also documented in these tumors. Patients experienced local recurrence (n = 2), locoregional lymph node metastases (n = 2), and an isolated visceral metastasis (n = 1). Overall, our study suggests that SMARCA2/4::CREM fusions define a distinct group of neoplasms with round cell to epithelioid histology, a variable immunoprofile, and a definite risk of malignancy. Larger studies are needed to further explore the pathogenetic relationship with the FET::CREB family of tumors. © 2024 The Pathological Society of Great Britain and Ireland. [ABSTRACT FROM AUTHOR]

High survivin expression has been correlated with poor outcomes in several canine tumors but not in soft tissue tumors (STTs). Survivin is a target gene of the Wnt/β-catenin pathway, which is involved in human STT oncogenesis. Immunohistochemistry for survivin, β-catenin, and Ki-67 was performed on 41 canine perivascular wall tumors (cPWTs), and statistical associations of protein expression and histopathologic and clinical variables with clinical outcomes were investigated. Immunohistochemically, there was nuclear positivity (0.9%–12.2% of tumor cells) for survivin in 41/41 (100%), cytoplasmic positivity (0 to > 75% of tumor cells) for survivin in 31/41 (76%), nuclear positivity (2.9%–67.2% of tumor cells) for β-catenin in 24/41 (59%), and cytoplasmic positivity (0% to > 75% of tumor cells) for β-catenin in 23/41 (56%) of cPWTs. All tumors expressed nuclear Ki-67 (2.2%–23.5%). In univariate analysis and multivariate analysis (UA and MA, respectively), every 1% increase of nuclear survivin was associated with an increase of the instantaneous death risk by a factor of 1.15 [hazard ratio (HR) = 1.15; P =.007]. Higher nuclear survivin was associated with grade II/III neoplasms (P =.043). Expression of cytoplasmic survivin, nuclear and cytoplasmic β-catenin, and nuclear Ki-67 were not significantly associated with prognosis in UA nor MA. Tumor size was a significant prognostic factor for local recurrence in UA [subdistribution HR (SDHR) = 1.19; P =.02] and for reduced overall survival time in MA. According to UA and MA, a unitary increase of mitotic count was associated with an increase of the instantaneous death risk by a factor of 1.05 (HR = 1.05; P =.014). Nuclear survivin, mitotic count, and tumor size seem to be potential prognostic factors for cPWTs. In addition, survivin and β-catenin may represent promising therapeutic targets for cPWTs. [ABSTRACT FROM AUTHOR]

Background: Solitary fibroous tumors (SFTs) are distinctive soft tissue tumors characterized by rearrangements of NAB2-STAT6 gene, which are associated with thin-walled, branching, "staghorn"-shaped vessels. SFTs are originally classified as a type of hemangiopericytoma (HPC). Classical SFTs are composed of spindle to ovoid cells arranged haphazardly or in fascicles. Rarely, SFTs exhibit unusual morphological variants such as fat formation, giant cells, dedifferentiation, or epithelioid variant. The epithelioid cell variant, which is composed almost entirely of epithelioid cells and arranged in solid or nest patterns, is extremely rare and frequently malignant. Case presentation: In this study, we reported three cases of epithelioid SFTs (ESFTs) located in extrathoracic sites (right lateral ventricle, right lumbar, left pelvis). All the subjects in this study were elderly, with a predominance of female patients, accounting for two out of the three cases, and only one case involved a male patient. The tumor cells were entirely composed of epithelioid cells and exhibited positive for CD34 and STAT-6 markers. Ultimately, the majority of cases (two out of three) were diagnosed as malignant SFTs. Conclusion: This study aims to enhance the awareness of ESFTs. In these cases, irrespective of the onset location, the arrangement patterns of tumor cells, such as papillary structures and the morphology of epithelial-like cells, conspicuously lack the hallmark histological characteristics of Solitary Fibrous Tumors (SFTs). Consequently, it requires differential diagnosis from a plethora of malignant neoplasms. Moreover, the elevated malignancy level of this cohort of cases poses substantial diagnostic challenges to pathologists, compounding the complexity of accurate interpretation. [ABSTRACT FROM AUTHOR]

Cervical aggressive fibromatosis is a rare intermediate tumor characterized by invasive growth. Aggressive fibromatosis (AF), also referred to as desmoid-type fibrosarcoma or grade I fibrosarcoma, is a clonal proliferative lesion of fibroblasts located in deep soft tissues. While many cases have been reported, there are relatively few involving aggressive fibromatosis in the anterior inferior margin muscle space of the neck trapezius muscle. We present a case of pathologically confirmed left cervical aggressive fibromatosis, admitted to the Fourth Affiliated Hospital of Zhejiang University. The initial ultrasound and magnetic resonance imaging (MRI) scan of the patient revealed a mass in the left cervical dorsal muscle space, which slowly increased after one year. An enhanced MRI scan initially diagnosed the mass as a left cervical schwannoma. The patient underwent neck soft tissue lesion resection surgery, with postoperative confirmation of cervical aggressive fibromatosis. Our case suggests that fibromatosis cannot be ruled out, and the low-signal cord-like non-enhanced areas, representing collagen fiber characteristics after enhanced scanning, are significant imaging features in diagnosing cervical fibromatosis. Based on the available literature, we have conducted preliminary research on the clinical presentation, imaging manifestations, diagnosis, and differential diagnosis of cervical aggressive fibromatosis to improve clinical understanding and ensure timely clinical treatment. [ABSTRACT FROM AUTHOR]

Nerve tumors in the retroperitoneal space are a rarity. Radical surgery according to soft tissue tumors can lead to persistent pain and neurological deficits. This study aims to evaluate clinical outcomes of patients treated by a visceral- / neurosurgical approach. 33 patients with a retroperitoneal nerve tumor underwent surgery between 01/2002 and 12/2022 at our department. A visceral surgeon provided access to the retroperitoneal space, followed by micro-neurosurgical tumor preparation under neuromonitoring. Clinical examination and MRI were performed 12 weeks after surgery and further 3 months (WHO grade > 1) or 12 months (WHO grade 1). Further examinations were based on MRI findings and residual symptoms with median follow-up time of 24 months. One patient was treated for two distinct masses resulting in a total of 34 histological findings. Schwannomas (n = 15; 44.1%) and neurofibromas (n = 10; 29.4%) were the most common tumors. Long-term improvements were noted in radicular pain (15/18 patients; 83.3%), motor deficits (7/16 patients; 43.8%), abdominal discomfort and pain (5/7 patients; 71.4%). Recurrences were observed in 3/33 (9,1%) patients. This study represents the largest series of retroperitoneal BPNSTs treated with microsurgical techniques. Prospective multicenter studies are warranted to establish standardized treatment guidelines. [ABSTRACT FROM AUTHOR]

Background: Extraskeletal mesenchymal chondrosarcoma (ESMC), an uncommon and highly aggressive form of chondrosarcoma, is characterized by its mesenchymal origin and absence of skeletal involvement. Only a few cases of primary ESMC with metastasis to the pancreas have been reported so far. In this study, we present a case of ESMC in the left thigh with a solitary pancreatic metastasis in a 45-year-old woman. Additionally, we provide a thorough overview of ESMC, encompassing its entire clinical progression and radiographic observations. Furthermore, we reviewed all thirteen cases of pancreatic metastasis, including this present case, analyzing patient attributes, clinical management, and prognosis. Case presentation: A 45-year-old woman has had a painless mass in her left thigh for one year. X-ray, computed tomography (CT), andmagnetic resonance imaging of the left thigh were performed. Positron emission tomography-CT imaging showed a high accumulation in the left thigh tumor and the pancreatic neck lesion. A diagnosis of extraskeletal chondrosarcoma with pancreatic metastasis was determined based on the radiological examinations. A final diagnosis of ESMC was confirmed by histopathological and immunohistochemical examinations after surgical resection. The patient presentedmetastasis in the lung, right groin, and tail of the pancreas successively, and mostly received complete surgical excision during a 39-month follow-up with postoperative chemotherapy. Conclusion: We present a highly uncommon case of ESMC spreading to the pancreas and highlight the importance of recognizing the distinctive imaging features of ESMC for diagnosis and prognosis assessment. [ABSTRACT FROM AUTHOR]

Background: Capicua transcriptional repressor-double homeobox 4 sarcoma (CDS) is a rare and aggressive malignant soft tissue tumor that typically arises within the soft tissues. We report an exceptionally rare case of a gastric CDS successfully resected despite its extensive invasion into surrounding organs. Case presentation: A 48-year-old male presented with a progressively enlarging abdominal mass. Upper gastrointestinal endoscopy revealed a large ulcerative tumor on the posterior gastric wall. Biopsy results initially suggested a neuroendocrine cell carcinoma. Contrast-enhanced computed tomography showed a 20 cm tumor protruding from the posterior stomach wall, directly invading the pancreas and colon. We performed a multivisceral resection (stomach, pancreatic tail, spleen, and transverse colon) achieving an R0 resection. Pathological examination of the permanent specimen revealed small round cells with high nuclear-to-cytoplasmic ratios. Immunohistochemical staining confirmed the diagnosis of CDS. The patient recovered well and was discharged on postoperative day 33. Conclusions: This case report describes the first detailed account of a surgically resected aggressive CDS originating from the stomach. [ABSTRACT FROM AUTHOR]

Purpose: Loddo et al. (Br J Cancer 100:959–70, 2009) established the prognostic significance of cell cycle markers and "Cell-Cycle Phenotypes" in breast carcinoma. This study aims to 1) identify prognostic cell-cycle markers in sarcoma, and 2) assess the prognostic potential of specific cell-cycle phenotypes in sarcoma. Methods: Tissue samples from 128 soft tissue sarcomas were stained for four cell cycle-specific markers: Mcm2, Geminin, Plk1, and H3S10ph. Only primary soft tissue tumors (liposarcoma, leiomyosarcoma, synovial sarcoma, and undifferentiated pleomorphic sarcoma) were included in the analysis. Any tumor coming from a recurrent or metastatic lesion were excluded from the analysis. Three cell-cycle phenotypes (I, II, III) were derived from marker expression patterns. Prognostic significance was evaluated in a subset of primary soft tissue sarcomas using Cox regression for survival analysis. Results: Compared to phenotype I, the phenotype III tumors had a decreased 5-year overall survival (HR 6.81 [2.36–19.61]; p = < 0.001), 5-year disease-free survival (HR 1.07 (1.02–1.18); p = 0.004), and 5-year metastasis-free survival (HR 4.34 [1.58–11.93]; p = 0.004). High expression of Plk1 was associated with decreased 5-year overall survival (HR: 4.04 CI [1.21–6.67; p = 0.02) and 5-year metastasis-free survival (HR: 2.91 CI [1.15–7.37]; p = 0.03). Geminin was also found to have a decreased 5-year overall survival (HR:2.84 CI [1.21–6.67]; p = 0.02). No statistical difference in prognostication were noted between phenotypes and the AJCC system. Conclusions: We identified three unique sarcoma cell cycle phenotypes that have prognostic significance. This performs similarly to the AJCC staging system. [ABSTRACT FROM AUTHOR]

Simple Summary: The pathologic response after neoadjuvant radiotherapy in soft tissue sarcoma of the extremities and trunk was evaluated using the EORTC-STBSG response score. The median percentages of viable cells, necrosis and fibrosis/hyalinization were 20%, 11% and 40%, respectively. A pathologic complete response, defined as ≤5% viable tumor cells, was achieved in 25% of cases. Local recurrence occurred in 33% of cases, with a significantly higher rate of 64% after R1 excision compared to 22% after R0 resection. Distant metastases were observed in 42% of patients, primarily in the lungs. The local recurrence free survival, distant metastasis free survival and overall survival rates were 65%, 54%, and 67% at 3-years, respectively. A correlation between tumor histological subtype, size and grade with outcome was observed. While the EORTC-STBSG response score did not correlate with clinical outcomes, resection specimens with ≤5% viable tumor cells were linked to improved outcomes. Background/Objectives: The relationship between pathologic findings in soft tissue sarcoma (STS) after neoadjuvant treatment and oncological outcomes remains uncertain due to varying evaluation methods and cut-off values. This study aims to assess pathologic findings after neoadjuvant radiotherapy in STS using the EORTC-STBSG response score and evaluate its prognostic value. Methods: Clinical and outcome data from 44 patients were reviewed. Resected specimens were re-evaluated to measure viable cells, necrosis, fibrosis, and hyalinization. Local recurrence-free survival (LRFS), distant metastasis-free survival (DMFS), and overall survival (OS) were analyzed using Kaplan–Meier survival analysis. Cox proportional hazards regression was used for univariate and multivariate analyses to correlate outcomes with pathologic response. Results: The median percentages of viable cells, necrosis, and fibrosis/hyalinization were 20%, 11%, and 40%, respectively. A pathologic complete response (pCR), defined as ≤5% viable cells, was achieved in 25% of cases. Local recurrence occurred in 33% of cases, with a significantly higher rate of 64% after R1 resection compared to 22% after R0 resection. Distant metastases were observed in 42% of patients, primarily in the lungs. The 3-year rates for LRFS, DMFS, and OS were 65%, 54%, and 67%, respectively. A correlation between outcomes and tumor size, grade and histological subtype was observed. Classifying pathologic response by the EORTC-STBSG score failed to show an association with outcomes. Patients achieving pCR showed lower risk of LR and improved OS. Conclusions: While the EORTC-STBSG score did not show a prognostic value, resection specimens with ≤5% viable cells were linked to improved LRFS and OS. [ABSTRACT FROM AUTHOR]

Doege–Potter syndrome (DPS) is a very rare paraneoplastic condition that is marked by hypoglycemia brought on by a solitary fibrous tumor rather than an islet cell tumor. Soft tissue neoplasms termed as solitary fibrous tumors (SFTs) are rare and these tumors vary in the site of origin, from the pleural cavity, mediastinum, pericardium, retroperitoneal spaces, liver, thyroid, orbit, bladder, intestines, and soft tissues, while pelvic‐derived fibrous tumors are incredibly unusual. There are currently extremely few documented cases and literature reviews both domestically and internationally. In this case study, we present an 82‐year‐old woman who developed DPS as a result of malignant pelvic SFTs. Her hypoglycemia was clinically healed after she underwent laparoscopic retroperitoneal tumor resection in our institution, and thereafter, her quality of life improved. [ABSTRACT FROM AUTHOR]

Background: Aspergillomas are globular growths of Aspergillus fumigatus, a benign aspergillosis of the lungs. It usually affects patients who are immunocompromised and have anatomically defective lung structures. The majority of aspergilloma cases are asymptomatic, despite the fact that 10% of cases spontaneously resolve. Most patients do not have any symptoms from their lesions. Direct serological or microbiological evidence of an Aspergillus species along with radiologic evidence is required for the diagnosis of an aspergilloma. Case: We describe a 35-year-old adult Oromo male patient who had been experiencing night sweats, an intermittent productive cough with sparse whitish sputum, loss of appetite, and easy fatigability for 3 months. At 5 years prior, he received treatment for pulmonary tuberculosis that was smear-positive and was subsequently certified healed. Objectively, he was tachypneic and had intercostal, subcostal, and supraclavicular retractions with symmetric chest movement. A high-resolution computed tomography scan revealed bilateral apical cavitary lesions with core soft tissue attenuating spherical masses and an air crescentic sign suggestive of aspergillomas, which were confirmed by sputum light microscopic examination. The patient was managed with antibiotics and antifungals. Conclusion: Aspergilloma is a symptom of chronic pulmonary aspergillosis, a category of lung disorders caused by a persistent Aspergillus infection. Primary aspergillomas are uncommon and frequently occur in people with compromised immune systems. A prolonged cough, fever, chest pain, and hemoptysis are all symptoms of pulmonary aspergillomas. The majority of the time, pulmonary aspergillosis is difficult to identify. Despite high mortality and morbidity rates, surgery is still the most effective treatment for pulmonary aspergilloma. [ABSTRACT FROM AUTHOR]

Myofibromatosis is a proliferative disorder characterized by the development of soft tissue neoplasms referred to as myofibromas that predominantly occur in infants and young children. These lesions have been described using varied terminologies since they were first reported in 1951. However, these neoplasms are histopathologically characterized by benign fibroblast and myofibroblast proliferation containing a biphasic presentation of spindle-shaped cells surrounding a central zone of less differentiated cells arranged in a hemangiopericytoma-like pattern in all cases. Usually, myofibromatosis occurs in children aged <2 years and is observed at birth in nearly 60% of cases. We report a rare case of myofibroma in an 8-year-old boy who presented with an approximately 6-month history of a left postauricular mass. Histopathological evaluation of the resected mass confirmed the diagnosis. [ABSTRACT FROM AUTHOR]

A wide spectrum of tumors may affect the periungual spaces of the digits. Superficial acral fibromyxoma (SAF) is a rare, benign soft tissue tumor with diverse clinical presentations. We present a case of a 55‐year‐old woman with a 2‐year history of a solitary periungual tumor on the left thumb, subjected to multiple episodes of trauma. Initially suspected to be a periungual squamous cell carcinoma (SCC) based on clinical and dermoscopic features, the tumor was confirmed to be a CD34− SAF through histopathology and immunohistochemistry. Although CD34 immunoreactivity is common in SAF, one‐third of these tumors, including this case, do not stain for this marker. Periungual SCC considered a "great mimicker of nail tumors," may resemble other benign nail tumors such as SAF. The patient underwent complete surgical excision with primary closure, resulting in no recurrence after 1 year. This case highlights SAF as an underrecognized benign entity that may manifest with features suspicious of malignancy, potentially leading to unnecessarily aggressive interventions. Recognizing SAF through accurate biopsy techniques and thorough histopathologic evaluation, even in the absence of CD34 reactivity, is crucial for appropriate treatment and preservation of hand function and appearance. [ABSTRACT FROM AUTHOR]

Background: The primary aim of this study is to evaluate the role of magnetic resonance imaging (MRI) in the assessment of soft tissue tumors with histopathological correlation at a tertiary care center. Methods: An observational study was conducted on 75 patients (n = 75) in the Department of Radiodiagnosis over 18 months. The targeted population comprised patients who presented to the Radiodiagnosis Department for radiological imaging of soft tissue tumors. Results: Out of 75 cases, 20% were found to have benign tumors, while 80% were found to have malignant tumors. The most frequent benign tumor was fibromatosis, with n = 10 cases (13.33%), and the most common malignant tumor was synovial sarcoma, with n = 14 cases (18.66%). The benign age group ranged from 11 to 20 years. T2-weighted heterogeneous hyperintensity was noted more frequently in malignant lesions, demonstrating a high positive predictive value; that is, 83% of malignant tumors exhibited changes on diffusion-weighted imaging/apparent diffusion co efficient (DWI/ADC). Low-grade malignant lesions showed no restrictions. Most benign lesions displayed restrictions, with a high positive predictive value of 98.14%, specificity of 93.33%, and sensitivity of 88.33%. Conclusions: Soft tissue tumors can be detected and locally staged using MRI; thus, this technique has proven its value. Intralesional hemorrhage and calcification are two parameters that have been shown to have no substantial association with cancer. Due to its high sensitivity, MRI is a viable option for evaluating soft tissue tumors. [ABSTRACT FROM AUTHOR]

Introduction: Extraskeletal Ewing sarcoma (EEwS) is a rare malignant tumor, and current international recommendations indicate systemic and local treatment like bone Ewing sarcoma (BEwS); to the best of our knowledge, very few studies tried to explore the clinical and genetic characteristics of this tumor, and the most appropriate treatment strategy remains uncertain. Methods: We reviewed 35 EEwS cases enrolled at Rizzoli Orthopedic Institute in Bologna, Italy, between 1988–2022. We performed RNA sequencing in 18 Ewing sarcoma cases, including 12 BEwSs and 6 EEwSs. We analyzed overall survival (OS), local relapse-free survival (LRFS), and metastasis-free survival (MFS) and the risk factors associated to survival. Results: Unsupervised hierarchical clustering showed no differences in the transcriptional profile between EEwS and BEwS. Five-year OS was 67% (95% confidence interval [CI]: 47–80), 5-year LRFS was 61% (95% CI: 43–75), and 5-year MFS was 55% (95% CI: 38–70). Recurrent tumors, larger than 8 cm, and elevated lactate dehydrogenase (LDH) serum value resulted to be negative prognostic factors. Conclusions: The finding/detection of a genetic profile that is indistinguishable between EEwS and BEwS confirms the view that the two subgroups belong to the same tumor entity and supports the use of a single therapeutic approach for Ewing sarcoma, regardless of the site of origin. Statistical evaluation showed that size bigger than 8 cm, elevated LDH, and recurrent tumors had a worse prognosis, suggesting a risk-stratification method for identifying patients for specific therapy treatment. However, larger, multicenter, prospective trials are called for to validate our findings. [ABSTRACT FROM AUTHOR]

Background: Soft tissue sarcoma (STS) represents highly multifarious malignant tumors that often occur in adolescents and have a poor prognosis. The basement membrane, as an ancient cellular matrix, was recently proven to play a vital role in developing abundant tumors. The relationship between basement membrane‐related genes and STS remains unknown. Methods: Consensus clustering was employed to identify subgroups related to differentially expressed basement membrane‐related genes. Cox and least absolute shrinkage and selection operator regression analyses were utilized to construct this novel signature. Then, we established a nomogram and calibration curve, including the risk score and available clinical characteristics. Finally, we carried out functional enrichment analysis and immune microenvironment analysis to investigate enriched pathways and the tumor immune microenvironment related to the novel signature. Results: A prognostic predictive signature consisting of eight basement membrane‐related genes was established. Kaplan–Meier survival curves demonstrated that the patients in the high‐risk group had a poor prognosis. Independent analysis illustrated that this risk model could be an independent prognostic predictor. We validated the accuracy of our signature in the validation data set. In addition, gene set enrichment analysis and immune microenvironment analysis showed that patients with low‐risk scores were enriched in some pathways associated with immunity. Finally, in vitro experiments showed significantly differential expression levels of these signature genes in STS cells and PSAT1 could promote the malignant behavior of STS. Conclusions: The novel signature is a promising prognostic predictor for STS. The present study may improve the prognosis and enhance individualized treatment for STS in the future. [ABSTRACT FROM AUTHOR]

Simple Summary: The characterization of molecular alterations is continuously gaining relevance in pathology as it can contribute to explaining tumors' pathogenesis and address specific targeted therapies. As for the kidney, in recent years, much scientific research has been focused on the MiTF family genes, particularly on the TFE3 gene. In this setting, while initially just accustomed to a subtype of renal cell carcinoma, currently classified as TFE3-rearranged renal cell carcinoma, TFE3 fusions have been identified in a few mesenchymal neoplasms of the kidney viewed as PEComas. In this work, we gather the available data regarding the key clinical and pathological features of these TFE3-rearranged renal epithelial and mesenchymal neoplasms. We seek to propose a comprehensive solution regarding their pathogenesis and sort out their classification conundrum. Background: Identical translocations involving the TFE3 gene and various partners have been found in both renal and soft tissue tumors, like alveolar soft part sarcoma (ASPSCR1), ossifying fibromyxoid tumor (PHF1), epithelioid hemangioendothelioma, and the clear cell stromal tumor of the lung (YAP1). Methods: Herein, we review in detail the clinicopathologic and molecular data of TFE3-rearranged renal tumors and propose our perspective, which may shed light on this emerging conundrum. Results: Among the kidney tumors carrying TFE3 translocations, most are morphologically heterogeneous carcinomas labeling for the tubular marker PAX8. The others are mesenchymal neoplasms known as PEComas, characterized by epithelioid cells co-expressing smooth muscle actin, cathepsin-K, melanogenesis markers, and sometimes melanin pigment deposition. Over the past 30 years, numerous TFE3 fusion partners have been identified, with ASPL/ASPSCR1, PRCC, SFPQ/PSF, and NONO being the most frequent. Conclusions: It is not well understood why similar gene fusions can give rise to renal tumors with different morpho-immunophenotypes, which may contribute to the recent disagreement regarding their classification. However, as these two entities, respectively, epithelial and mesenchymal in nature, are widely recognized by the pathology community and their clinicopathologic features well established, we overall believe it is still better to retain the names TFE3-rearranged renal cell carcinoma and TFE3-rearranged PEComa. [ABSTRACT FROM AUTHOR]

Objective: Malignant soft tissue sarcoma (MSTS) is a rare disease, but is seen in patients undergoing orthopedic surgery. Although the association of periodontal disease with various cancers occurring in the oral cavity, gastrointestinal tract, lungs, and prostate, has been reported, the association between periodontal disease and MSTS remains unclear. This study investigated the association between periodontal disease and MSTS in patients undergoing orthopedic surgery. Subjects and Methods: One hundred fifteen patients who underwent orthopedic surgery between 2017 and 2021 were retrospectively enrolled (mean age = 66.8 ± 10.7 years). The patient background was adjusted by the propensity score (PS). Subsequently, the association of periodontal disease with MSTS was analyzed using PS inverse probability of treatment weighting (IPTW). Periodontal status was determined by evaluating the periodontal inflamed surface area, which was calculated by measuring the periodontal probing pocket depth and detecting bleeding on probing. Results: Multivariate logistic regression analysis after adjustment by the PS showed that severe periodontitis was significantly associated with MSTS (odds ratio 2.81, p = 0.04). Furthermore, IPTW showed that severe periodontitis was significantly associated with MSTS (odds ratio 3.21, p = 0.01). Conclusion: The results indicate an association between periodontal inflammation and MSTS. [ABSTRACT FROM AUTHOR]

Rhabdomyosarcoma (RMS) is a malignant soft tissue tumor of skeletal muscle origin. The head and neck, urinary tract, and extremities are the common sites of origin. Embryonal, alveolar, pleomorphic, and spindle/sclerosing are subtypes. It is more common in childhood and rare among adults. The incidence and risk factors for this disease are mainly largely unknown. RMS is sporadic in most instances; however, it is attributed to familial syndromes in some situations--its metastasis to the lungs, bone marrow, and lymph nodes. Breast and ovary involvement is scarce. Diagnostic workup mainly includes contrast-enhanced computed tomography (CECT) and magnetic resonance imaging (MRI). However, 18F-fluro-deoxyglucose positron emission tomography (18F-FDG-PET/CT) and PET/MRI are increasing contribution to providing functional insights about tumor biology and improving the diagnostic accuracy of the imaging workup. This report presents a case of the neck's embryonal RMSmetastasizing simultaneously to the breast and ovary. PET/CT imaging revealed the unusual pattern, further validated by histopathology. [ABSTRACT FROM AUTHOR]

Anaplastic lymphoma kinase (ALK)-rearranged mesenchymal neoplasms (non-inflammatory myofibroblastic tumor and non-epithelioid fibrous histiocytoma) have been recently described which tend to occur in the superficial and deep soft tissues. Occurrence as a primary sinonasal neoplasm has not been reported thus far. Herein, we describe the first case of sinonasal ALK-rearranged mesenchymal tumor that harbored remarkable epithelioid and spindle cell morphology. The tumor affected a 40-year-old man who presented with flu-like symptoms and was thought to have influenza A. However, computed tomography demonstrated a nasal polypoid lesion causing curvature of the nasal septum. Histological examination revealed a heterogeneous tumor composed of round to epithelioid cells with foci of spindle cells. The tumor cells exhibited moderate pleomorphism and mitotic activity. By immunohistochemistry, they showed diffuse staining of CD34, S100, ALK (D5F3) and CD30. Fluorescence in situ hybridization analysis demonstrated ALK rearrangement. Subsequent next-generation sequencing (RNA-seq) identified a rare PLEKHH2exon6::ALKexon20 fusion. This study further demonstrates the importance of molecular profiling in identifying kinase fusion-positive soft tissue tumors, particularly for those that arise at unusual sites and display atypical cytomorphology. [ABSTRACT FROM AUTHOR]

To systematically evaluate the application value of radiomics and deep learning (DL) in the differential diagnosis of benign and malignant soft tissue tumors (STTs). A systematic review was conducted on studies published up to December 11, 2023, that utilized radiomics and DL methods for the diagnosis of STTs. The methodological quality and risk of bias were evaluated using the Radiomics Quality Score (RQS) 2.0 system and Quality Assessment of Diagnostic Accuracy Studies-2 (QUADAS-2) tool, respectively. A bivariate random-effects model was used to calculate the summarized sensitivity and specificity. To identify factors contributing to heterogeneity, meta-regression and subgroup analyses were performed to assess the following covariates: diagnostic modality, region/volume of interest, imaging examination, study design, and pathology type. The asymmetry of Deeks' funnel plot was used to assess publication bias. A total of 21 studies involving 3866 patients were included, with 13 studies using independent test/validation sets included in the quantitative statistical analysis. The average RQS was 21.31, with substantial or near-perfect inter-rater agreement. The combined sensitivity and specificity were 0.84 (95% CI: 0.76–0.89) and 0.88 (95% CI: 0.69–0.96), respectively. Meta-regression and subgroup analyses showed that study design and the region/volume of interest were significant factors affecting study heterogeneity (P < 0.05). No publication bias was observed. Radiomics and DL can accurately distinguish between benign and malignant STTs. Future research should concentrate on enhancing the rigor of study designs, conducting multicenter prospective validations, amplifying the interpretability of DL models, and integrating multimodal data to elevate the diagnostic accuracy and clinical utility of soft tissue tumor assessments. [ABSTRACT FROM AUTHOR]

Objective To present a new treatment for ingrown toenail with CO2 laser that is easy to perform, without damage the nail, nail matrix and nail bed. Methods A total of 21 ingrown toenail (IGT) lesions of the big toe in 16 patients who were diagnosed as ingrown toenail were treated with CO2 laser, while tissues along nail groove around the lesion was ablated, ingrown nail plate was exposed and separated. Results A total of 16 patients, with a mean age was (23.38 ± 13.61) years. All the 21 lesions were regressed except 2 with relapse (the cure rate was 90.48%). The time of return to walking, and return to daily activity were (2.25 ±0.66) days and (5.13 ± 0.86) days, respectively. During the 6-35 months follow-up, the cosmetic effect was remarkable with normal appearance and function. There were no complications. Conclusion Ingrown toenail can be surgically treated without matriceetomy and preserved the nail. All necrotic tissue and certain volume of soft tissue surrounding the lesion should be ablated to decompress the nail and reduce inflammation. [ABSTRACT FROM AUTHOR]

Intratumoral microbiota, which affects the physiological and pathological processes of the host, has attracted increasing attention from researchers. Microbials have been found in normal as well as tumor tissues that were originally thought to be sterile. Intratumoral microbiota is considered to play a significant role in the development of tumors and the reduction of clinical benefits. In addition, intratumoral microbiota are heterogeneous, which have different distribution in various types of tumors, and can influence tumor development through different mechanisms, including genome mutations, inflammatory responses, activated cancer pathways, and immunosuppressive microenvironments. Therefore, eliminating the intratumoral microbiota is considered one of the most promising ways to slow down the tumor progression and improve therapeutic outcomes. In this review, we systematically categorized the intratumoral microbiota and elucidated its role in the pathogenesis and therapeutic response of cancer. We have also described the novel strategies to mitigate the impact of tumor progression. We hope this review will provide new insights for the anti‐tumor treatment, particularly for the elderly population, where such insights could significantly enhance treatment outcomes. [ABSTRACT FROM AUTHOR]

Myoepithelioma is an exceptionally rare tumor, primarily arising in glandular tissues but occasionally found in soft tissues, including the hand. Its occurrence in the hand is particularly uncommon, presenting unique clinical challenges due to the limited number of documented cases and the unusual location. We conducted a literature review in June 2024, with the aim to evaluate the current understanding of hand myoepithelioma, recent diagnostic advances, treatment options, and the diverse presentations of this neoplasm. Articles confirmed that patients present with a painless, slow-growing mass in the hand, often misdiagnosed as more common soft tissue tumors like lipomas or fibromas. Imaging, particularly MRI and ultrasound, aids in assessing the tumor, but definitive diagnosis relies on histopathology, including immunophenotyping. Managing spindle cell myoepithelioma in the hand requires a multidisciplinary approach, with surgical excision being the primary treatment. Achieving clear margins is critical yet challenging due to the hand's complex anatomy. In some cases, adjuvant therapies such as radiation or chemotherapy may be necessary. The prognosis depends on factors like tumor size, location, and the success of surgical removal, with complete excision typically leading to a favorable outcome. [ABSTRACT FROM AUTHOR]

Background: Tenosynovial giant cell tumor is a rare soft tissue tumor of the synovium of joint, bursae, or tendon sheath. It is divided into localized or diffuse types on the basis of the growth pattern. Localized tenosynovial giant cell tumors are usually benign and treated successfully by excision. Diffuse tenosynovial giant cell tumors, in contrast to localized type, can destroy bone and cartilage and are associated with frequent local recurrences and distant metastasis. Localized type tenosynovial giant cell tumors rarely metastasize to distant organs. Here, we report a case of localized tenosynovial giant cell tumor presenting with lung metastases and systematically review literature. Case presentation: A 55-year-old Asian male presented with a dry cough, right-sided chest pain and progressive dyspnea for 1 month. At 18 months before this presentation, he had undergone excision of a painless swelling on his right index finger. The swelling recurred within 3 months of excision, and a biopsy was then suggestive of a giant cell tumor. Given the suspicion of a giant cell tumor, a wide excision of the lesion was performed and the excisional biopsy was consistent with a diagnosis of tenosynovial giant cell tumor, localized type. At admission to our hospital, the patient had tachypnoea and absent breath sounds on the right side. A chest radiograph showed a right-sided pleural effusion with a homogenous opacity in the left mid-zone. A contrast-enhanced computed tomography of the chest and abdomen showed right massive pleural effusion and bilateral multiple lobulated heterogeneously enhancing pleural-based masses with areas of internal calcification. Pleural fluid analysis revealed an exudate with no malignant cells on cytology. A lung biopsy showed osteoclast-like giant cells and mononuclear spindle cells with areas of hemorrhage and necrosis, suggesting tenosynovial giant cell tumor metastasis. A final diagnosis of localized type tenosynovial giant cell tumor of the right index finger with metastases to the lungs and pleura was made. The patient passed away after receiving three cycles of denosumab injection owing to progressive disease. Conclusion: Lung metastasis is extremely rare in patients with localized tenosynovial giant cell tumor. The survival is usually poor in patients with lung metastasis. A close follow-up of patients with localized type tenosynovial giant cell tumor is necessary for early detection of pleuropulmonary complications. [ABSTRACT FROM AUTHOR]

Congenital fibrous soft tissue tumors in the eyelids are exceptionally rare, with only seven cases of neonatal myofibromas reported. Myopericytoma, a variant with pericytic differentiation, has not previously been described in neonatal eyelid tumors. This report details a case of a massive congenital myopericytoma in a newborn’s lower eyelid. The lesion had a broad area of adhesion to the anterior lamella of the lid. After resection, when the child was 11 days old, the resulting defect was successfully reconstructed with a V-Y type perforator-based flap. [ABSTRACT FROM AUTHOR]

Musculoskeletal traumatic injuries (MTI) involve soft tissue lesions adjacent to a bone fracture leading to fibrous nonunion. The impact of MTI on the inflammatory response to fracture and on the immunomodulation of skeletal stem/progenitor cells (SSPCs) remains unknown. Here, we used single-nucleus transcriptomic analyses to describe the immune cell dynamics after bone fracture and identified distinct macrophage subsets with successive pro-inflammatory, pro-repair and anti-inflammatory profiles. Concurrently, SSPCs transition via a pro- and anti-inflammatory fibrogenic phase of differentiation prior to osteochondrogenic differentiation. In a preclinical MTI mouse model, the injury response of immune cells and SSPCs is disrupted leading to a prolonged pro-inflammatory phase and delayed resolution of inflammation. Macrophage depletion improves bone regeneration in MTI demonstrating macrophage involvement in fibrous nonunion. Finally, pharmacological inhibition of macrophages using the CSF1R inhibitor Pexidartinib ameliorates healing. These findings reveal the coordinated immune response of macrophages and skeletal stem/progenitor cells as a driver of bone healing and as a primary target for the treatment of trauma-associated fibrosis. Hachemi et al. report the immune cell atlas of bone repair revealing macrophages as pro-fibrotic regulators and a therapeutic target for musculoskeletal regeneration. Genetic depletion or pharmacological inhibition of macrophages improves bone healing in musculoskeletal trauma. [ABSTRACT FROM AUTHOR]

Introduction: Magnetic Resonance Elastography (MRE) allows the non-invasive quantification of tumor biomechanical properties in vivo. With increasing incidence of brain metastases, there is a notable absence of appropriate preclinical models to investigate their biomechanical characteristics. Therefore, the purpose of this work was to assess the biomechanical characteristics of B16 melanoma brain metastases (MBM) and compare it to murine GL261 glioblastoma (GBM) model using multifrequency MRE with tomoelastography post processing. Methods: Intracranial B16 MBM (n = 6) and GL261 GBM (n = 7) mouse models were used. Magnetic Resonance Imaging (MRI) was performed at set intervals after tumor implantation: 5, 7, 12, 14 days for MBM and 13 and 22 days for GBM. The investigations were performed using a 7T preclinical MRI with 20 mm head coil. The protocol consisted of single-shot spin echo-planar multifrequency MRE with tomoelastography post processing, contrast-enhanced T1- and T2- weighted imaging and diffusion-weighted imaging (DWI) with quantification of apparent diffusion coefficient of water (ADC). Elastography quantified shear wave speed (SWS), magnitude of complex MR signal (T2/T2*) and loss angle (j). Immunohistological investigations were performed to assess vascularization, blood-brain-barrier integrity and extent of glucosaminoglucan coverage. Results: Volumetric analyses displayed rapid growth of both tumor entities and softer tissue properties than healthy brain (healthy: 5.17 ± 0.48, MBM: 3.83 ± 0.55, GBM: 3.7 ± 0.23, [m/s]). SWS of MBM remained unchanged throughout tumor progression with decreased T2/T2* intensity and increased ADC on days 12 and 14 (p<0.0001 for both). Conversely, GBM presented reduced j values on day 22 (p=0.0237), with no significant alterations in ADC. Histological analysis revealed substantial vascularization and elevated glycosaminoglycan content in both tumor types compared to healthy contralateral brain. Discussion: Our results indicate that while both, MBM and GBM, exhibited softer properties compared to healthy brain, imaging and histological analysis revealed different underlying microstructural causes: hemorrhages in MBM and increased vascularization and glycosaminoglycan content in GBM, further corroborated by DWI and T2/T2* contrast. These findings underscore the complementary nature of MRE and its potential to enhance our understanding of tumor characteristics when used alongside established techniques. This comprehensive approach could lead to improved clinical outcomes and a deeper understanding of brain tumor pathophysiology. [ABSTRACT FROM AUTHOR]

Objective: The purpose of this study is to determine the prevalence and features of oral and maxillofacial lesions found in the residents of Al‐Qassim region, Saudi Arabia. Methods: A retrospective study was conducted at King Fahad Specialist Hospital, Buraidah, Qassim, KSA. The data for all biopsied oral and maxillofacial lesions were retrieved from January 2014 until August 2022. All patients' data including age, gender, location of the lesion, and histopathologic diagnosis were reviewed and analyzed using IBM SPSS version 23 and Microsoft Excel. Results: A total of 381 oral pathology biopsies for individuals aged 18 and above were included in a descriptive analysis. One hundred ninety five (51.18%) of patients were male, and 186 (48.82%) were female. The site most commonly biopsied was the oral mucosa (26%). The diagnosis was categorized according to the histopathological diagnosis into 13 categories including all pathological lesions in the oral and maxillofacial area. The frequently biopsied category was soft tissue pathological lesion category (26%), second to that is the odontogenic cyst category (22%), and third is the immunological‐mediated lesion category (13%). The sub‐diagnosis that was mostly observed was radicular cyst, lichen planus, and focal fibrous hyperplasia with the percentages of 13.6%, 10.8%, and 9.4%, respectively. Conclusion: The findings provide important information about the oral and maxillofacial pathology in Al‐Qassim region, Saudi Arabia. This study found that biopsied oral lesions were more prevalent in males and in patients in the fourth decade of life. The oral mucosa was the most biopsied site, and the majority of the biopsies were soft tissue pathological lesions and radicular cyst was the most frequent diagnosis. Knowledge of such demographic and clinical features of oral and maxillofacial pathology cases helps in prediction of disease incidence and subsequent proper patient care in the region. [ABSTRACT FROM AUTHOR]