UNASSIGNED: Desmoid tumor (DT) is a rare locally aggressive but non-metastatic mesenchymal soft tissue neoplasm that predominantly occurs in the abdominal wall, abdominal cavity, and extremities. Its occurrence in the mesentery is relatively uncommon.
UNASSIGNED: This article reports two cases of desmoid tumor treated at the Department of Gastrointestinal Surgery, Weifang People\'s Hospital. The first case was a 59-year-old male patient who had previously undergone surgery for esophagogastric junction cancer. Postoperatively, he developed an intra-abdominal mass that rapidly increased in size within three months. The second case was a 60-year-old male patient who incidentally discovered a mass in the left lower abdomen. Both patients underwent surgical treatment, and the postoperative pathological diagnosis was mesenteric desmoid tumor.
UNASSIGNED: The treatment of desmoid tumor remains challenging. Simple surgical resection often yields unsatisfactory outcomes, and the efficacy of adjuvant radiotherapy and chemotherapy is also limited. Further research and clinical practice are necessary to improve diagnostic and therapeutic strategies, aiming to enhance patient survival and quality of life.

OBJECTIVE: To systematically evaluate the application value of radiomics and deep learning (DL) in the differential diagnosis of benign and malignant soft tissue tumors (STTs).
METHODS: 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.
RESULTS: 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.
CONCLUSIONS: 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.

Objective: To evaluate the diagnostic performance of standard diffusion-weighted imaging (DWI), intravoxel incoherent motion (IVIM), and diffusion kurtosis imaging (DKI), for differentiating benign and malignant soft tissue tumors (STTs). Materials and methods: A thorough search was carried out to identify suitable studies published up to September 2020. The quality of the studies involved was evaluated using Quality Assessment of Diagnostic Accuracy Studies-2 (QUADAS-2). The pooled sensitivity (SEN), specificity (SPE), and summary receiver operating characteristic (SROC) curve were calculated using bivariate mixed effects models. A subgroup analysis was also performed to explore the heterogeneity. Results: Eighteen studies investigating 1319 patients with musculoskeletal STTs (malignant, n=623; benign, n=696) were enrolled. Thirteen standard DWI studies using the apparent diffusion coefficient (ADC) showed that the pooled SEN and SPE of ADC were 0.80 (95% CI: 0.77-0.82) and 0.63 (95% CI: 0.60-0.67), respectively. The area under the curve (AUC) calculated from the SROC curve was 0.806. The subgroup analysis indicated that the percentage of myxoid malignant tumors, magnet strength, study design, and ROI placement were significant factors affecting heterogeneity. Four IVIM studies showed that the AUCs calculated from the SROC curves of the parameters ADC and D were 0.859 and 0.874, respectively. The AUCs for the IVIM parameters pseudo diffusion coefficient (D*) and perfusion fraction (f) calculated from the SROC curve were 0.736 and 0.573, respectively. Two DKI studies showed that the AUCs of the DKI parameter mean kurtosis (MK) were 0.97 and 0.89, respectively. Conclusion: The DWI-derived ADC value and the IVIM DWI-derived D value might be accurate tools for discriminating musculoskeletal STTs, especially for non-myxoid SSTs, using more than two b values, with maximal b value ranging from 600 to 800 s/mm2, additionally, a high-field strength (3.0 T) optimizes the diagnostic performance.

EWSR1 belongs to the FET family of RNA-binding proteins including also Fused in Sarcoma (FUS), and TATA-box binding protein Associated Factor 15 (TAF15). As consequence of the multifunctional role of EWSR1 leading to a high frequency of transcription of the chromosomal region where the gene is located, EWSR1 is exposed to aberrations such as rearrangements. Consecutive binding to other genes leads to chimeric proteins inducing oncogenesis. The other TET family members are homologous. With the advent of widely used modern molecular techniques during the last decades, it has become obvious that EWSR1 is involved in the development of diverse benign and malignant tumors with mesenchymal, neuroectodermal, and epithelial/myoepithelial features. As oncogenic transformation mediated by EWSR1-fusion proteins leads to such diverse tumor types, there must be a selection on the multipotent stem cell level. In this review, we will focus on the wide variety of soft tissue and bone entities, including benign and malignant lesions, harboring EWSR1 rearrangement. Fusion gene analysis is the diagnostic gold standard in most of these tumors. We present clinicopathologic, immunohistochemical, and molecular features and discuss differential diagnoses.

Perhaps the rarest cause of osteomalacia is that caused by a neoplasm, so-called \"tumor-induced osteomalacia\" (TIO). Although very rare cases of TIO have been associated with carcinomas and syndromes such as neurofibromatosis type-1 and McCune-Albright syndrome, the overwhelming majority of TIO is caused by tumors of mesenchymal origin. Although it was historically felt that almost any mesenchymal tumor type could occasionally result in TIO, it has become increasingly clear over the past several decades that almost all cases of mesenchymal tumor-associated TIO are caused by a single entity, known as \"phosphaturic mesenchymal tumor\" (PMT). This article will review historical aspects of this tumor, as well as its clinical, morphological, immunohistochemical and molecular genetic features. The distinction of PMT from its many potential morphological mimics is discussed in detail.

The majority of peripheral musculoskeletal soft tissue masses encountered in routine clinical practice are benign and associated with an excellent prognosis, whereas a small proportion of soft tissue masses are malignant and are associated with higher morbidity and mortality. Owing to the excellent tissue contrast resolution provided, magnetic resonance imaging (MRI) is a primary modality for the diagnostic evaluation of soft tissue masses, particularly for defining the anatomic extent. MRI is also useful for the characterization of soft tissue masses; however, after the exclusion of cysts and lipomas, it is challenging to accurately categorize neoplastic lesions as benign or malignant with conventional anatomic MRI. Quantitative MRI biomarkers obtained with nonconventional pulse sequences are becoming increasingly available, and can improve the accuracy of determining the character of soft tissue masses. In this article we discuss established qualitative and emerging quantitative MRI-based biomarkers available with dynamic contrast-enhanced MRI, diffusion-weighted imaging, and MR spectroscopy and their utility for the characterization of untreated soft tissue masses. Level of Evidence: 5 Technical Efficacy Stage: 5 J. Magn. Reson. Imaging 2019;50:11-27.

Mesenchymal neoplasms of the genitourinary (GU) tract often pose considerable diagnostic challenges due to their wide morphologic spectrum, relative rarity, and unexpected incidence at GU sites. Soft tissue tumors arise throughout the GU tract, whether from adventitia surrounding or connective tissues within the kidneys, urinary bladder, and male and female genital organs. This selected article focuses on a subset of these lesions, ranging from benign to malignant and encompassing a range of patterns of mesenchymal differentiation, where recent scholarship has lent greater insight into their clinical, molecular, or diagnostic features.

The case involves a 10-year-old child who underwent a left radical nephrectomy for what was believed to be a Wilms\' tumor. Histopath examination indicated a benign vascular lesion, subsequently determined to be an anastomosing hemangioma of the kidney. A comparison with the previously cited pediatric patients with renal vascular tumors is provided, and the inconsistent diagnostic terminologies for these conditions are highlighted. The therapeutic implications of these predominantly benign renal tumors, in the context of the much more frequently encountered malignant neoplasms in children, are additionally discussed.

Infantile myofibroma is a rare benign mesenchymal tumor that presents as solitary or multiple lesions (myofibromatosis) in the skin, soft tissue, bone, or internal organs. It most commonly affects the head and neck of infants and young children, but it can also affect adults. Intracranial involvement is reported to be extremely rare, and its clinical picture has been poorly characterized. Recently, it has been demonstrated that germline and somatic mutations in the platelet-derived growth factor receptor beta (PDGFRB) are associated with familial infantile myofibromatosis. We report a case of infantile myofibromatosis with predominant posterior fossa extradural involvement in a 14-year-old adolescent girl with a confirmed mutation in the PDGFRB gene.

OBJECTIVE: Clear cell sarcoma (CCS) is a rare soft tissue tumor thought to originate from tendons and aponeuroses. This is the largest and most comprehensive study of CCS to the best of our knowledge. In addition, this is the first study to determine the estimated 10-year overall survival, specific treatment modalities including neoadjuvant and adjuvant combinations, and sites of distant metastasis in CCS utilizing a national database.
METHODS: The National Cancer Database (NCDB) was used to study 489 patients diagnosed with CCS from 2004 to 2014. Kaplan-Meier methods were used to estimate 5- and 10-year overall survival, and log-rank tests were used to compare survival amongst stage.
RESULTS: Median age at diagnosis was 39 years. Males and females were equally affected. Race distribution was 78% Caucasian and 15% Black. Most common primary site was lower limb or hip (53%). Percentage of patients with distant organ metastases was 15%, with lung being the most common site (4%). Median overall survival was 57.2 months. Overall estimated 5- and 10-year survival was approximately 50 and 38%, respectively. Approximate 5-year survival for Stages I-IV was 75, 65, 35, and 15%, respectively. Stages at disease presentation were 34% at Stage I, 13% at Stage II, 16% at Stage III, and 38% at Stage IV. Surgery was the most common form of treatment (83%); 34% received radiation and 20% received chemotherapy.
CONCLUSIONS: The 5-year overall survival for CCS in Stages III and IV was much worse than Stages I and II. Overall estimated 5- and 10-year survival was approximately 50 and 38%, respectively. Men and women were equally affected and had a median age at diagnosis of 39 years. The most common tumor location was lower limb and hip and the most common site of metastases was the lung.