Search Results (40)

A 54-year-old female patient presented with a gradually enlarging mass on the ulnar side of the left wrist. Magnetic resonance imaging demonstrated a fat-containing lesion deep within the flexor carpi ulnaris that showed heterogeneous signal intensity, as well as weak internal and peripheral enhancement, which are not typical for atypical spindle cell/pleomorphic lipomatous tumors (ASPLTs). The imaging appearance overlapped with spindle cell lipoma (SCL), atypical lipomatous tumor/well-differentiated liposarcoma (ALT/WDL), and hibernoma, which made preoperative diagnosis challenging. The mass was excised en bloc. Histologically, the tumor consisted of mature adipocytes with substantial size variation, mild atypical spindle cells within a myxoid stroma, and scattered lipoblasts. Immunohistochemistry showed CD34 positivity and loss of RB1, while MDM2 amplification was absent on fluorescence in situ hybridization analysis, supporting a diagnosis of ASPLT. ASPLT is known for its broad range of morphological and radiological presentations, and this case further highlights the difficulty inherent in distinguishing it from SCL and ALT/WDL based on imaging alone. Recognition of its diverse features and the use of molecular testing are essential for accurately diagnosing ASPLT. Surgical excision remains the standard treatment. Although recurrence has been reported, metastasis of ASPLT is exceedingly rare. Full article

Background/Objectives: Trabectedin is a standard-of-care chemotherapy for recurrent/metastatic liposarcomas, including myxoid/round cell liposarcoma (MRCLS). This pooled analysis was performed to evaluate trabectedin efficacy in MRCLS across multiple trials to establish a standard-of-care efficacy baseline. Methods: The Yale University Open Data Access (YODA) Project was queried for prospective trabectedin trials. Data on patients with unresectable, locally advanced/metastatic MRCLS who received ≥ 1 trabectedin dose(s) (1.0–1.5 mg/m² administered as a 24-h continuous infusion every 3 weeks) were extracted from three trials: NCT00060944, NCT00210665, and NCT01343277. The primary outcome was the objective response rate as per the Response Evaluation Criteria in Solid Tumors (RECIST) v1.1. Secondary outcomes included disease control rate and overall survival, defined as the time from first trabectedin administration to death. Responses were assessed using RECIST by investigators in NCT01343277 and by independent review in NCT00060944. In NCT00210665, tumor assessment followed institutional standards, so only overall survival was analyzed. Results: Sixty-three patients were included (42 from NCT01343277, 13 from NCT00060944, 8 from NCT00210665): 32% were female, the median age was 50 years, and 79% were White. The objective response rate was 16.3% (8/49) of patients (37 from NCT01343277, 12 from NCT00060944). The disease control rate was 77.6% (38/49). Overall survival data were available for 63 patients: the median overall survival was 22.51 months (95% CI: 16.99–34.33). Conclusions: This analysis represents one of the largest pooled populations to date of patients with advanced MRCLS treated with trabectedin in prospective clinical trials to be reported. Whilst limited by typical factors affecting pooled analyses, including cross-study heterogeneity, nevertheless the results estimate a trabectedin efficacy baseline against which to inform other therapies for MRCLS. Full article

Background/Objectives: The most common soft tissue sarcomas in adults are liposarcomas, a heterogeneous group of malignant tumors. A structured literature search was conducted to better understand the clinical-imaging aspects and molecular behavior underlying the therapeutic approach. Methods: A scoping review was performed according to the PRISMA-ScR guidelines. Searches were conducted in MEDLINE, Web of Science Core Collection, and Google Scholar for the period of 1 January 2016–27 March 2026. Studies that addressed liposarcomas and their subtypes were included. Data were extracted descriptively and synthesized narratively. Results: We identified 113 studies that met the inclusion and exclusion criteria. Most studies have focused on a subtype of liposarcomas or on aspects such as clinical, imaging, histopathological, molecular, therapeutic, or prognostic features. The collected data identify, in each case, the need to develop new techniques useful for their identification and deeper molecular analysis. These needs arise from the need to improve treatment and to provide better prognosis, especially in rare or high-grade subtypes. Conclusions: The heterogeneity of these tumors requires the provision of a diagnosis that takes into account all possible instruments: clinical, imaging, and histo-molecular. Therefore, further research and translational medicine are needed to discover new pathogenic mechanisms in order to develop individualized therapies that increase quality of life. Full article

Background/Objectives: The aim of this study was to develop and validate a robust, radiomics-based classification model that uses pre-treatment MRI to non-invasively differentiate among major soft tissue sarcoma (STS) subtypes and a benign mimic. Methods: In this retrospective study, a cohort of 332 patients with biopsy-proven leiomyosarcoma, myxofibrosarcoma, myxoid liposarcoma, dedifferentiated liposarcoma, and undifferentiated pleomorphic sarcoma, along with the benign mimic intramuscular myxoma, was analyzed. Pre-treatment T1-weighted fat-saturated contrast-enhanced and T2-weighted fat-saturated MRI sequences were used for analysis. Following manual tumor segmentation, 1240 three-dimensional radiomic features were extracted. An XGBoost classifier was trained and validated using a robust 250-iteration bootstrap framework with nested cross-validation to ensure rigorous feature selection and unbiased performance evaluation. The model’s performance was assessed independently on T1-only, T2-only, and combined T1+T2 feature sets. Results: The combined T1 and T2 model achieved superior performance with an accuracy of 0.68 ± 0.04 and an AUC of 0.92 ± 0.02. At the subtype level, balanced accuracy was highest for intramuscular myxoma (0.91 ± 0.05), dedifferentiated liposarcoma (0.84 ± 0.06), and leiomyosarcoma (0.83 ± 0.05). SHAP analysis identified key features driving predictions, such as low T2 GLSZM Zone Size Entropy for myxoma and high T2 GLSZM Gray-Level Variance for leiomyosarcoma, which aligns with known pathological characteristics. Misclassifications predominantly occurred between subtypes with overlapping radiomic profiles. Conclusions: Radiomics applied to pre-treatment MRI enables robust, non-invasive classification of STS subtypes, demonstrating strong clinical potential for improving diagnostic confidence and informing triage strategies. Full article

Background/Objectives: Myxoid liposarcoma (MLS) is a malignant soft-tissue tumor and the second-most common subtype of liposarcoma, often occurring in the lower limbs of middle-aged patients. Case Presentation: A 38-year-old male patient presented to the ultrasound outpatient clinic with a large mass in the right femoral region. It has been present for 15 years and mostly stable in size. Over the last two years, it has been slowly increasing in size, more rapidly in the previous 10 months, and has started to limit his range of motion. After multiparametric ultrasound and magnetic resonance imaging evaluation, the proposed diagnosis was myxoid liposarcoma. Following imaging workup, the patient was referred to the tertiary sarcoma center, where a biopsy was performed, and pathohistological diagnosis was low-grade myxoid liposarcoma. Contrast-enhanced computed tomography (CT) evaluation of the thorax, abdomen, and pelvis showed no signs of dissemination, and CT angiography showed no signs of vessel infiltration. Plastic surgery and vascular surgery specialists performed the extirpation of the mass with the partial resection of the adjacent sartorius muscle and the complete resection of the great saphenous vein. Subsequent pathohistological analysis of the mass and local lymph nodes showed clear surgical margins and no lymphatic or vascular invasion. The patient is currently under regular surveillance by an oncology specialist and awaiting adjuvant radiotherapy. Conclusions: A multidisciplinary approach is essential in the management of patients with MLS, as it provides a tailored, individualized assessment from diagnosis through treatment to ensure the best possible outcome. Full article

Introduction: Myxoid liposarcoma (MLPS) is a rare soft tissue sarcoma comprising 5–10% of adult cases, most often in the thigh. Diagnosis is challenging due to nonspecific imaging findings and resemblance to benign lesions. Case Report: A 42-year-old male presented with a painless, enlarging upper right medial thigh mass. CT and ultrasound suggested a complex solid lesion, possibly benign. Outpatient surgical excision revealed a red, gelatinous, non-encapsulated mass. Frozen section suggested a myxomatous spindle cell tumor. Final pathology confirmed MLPS FNCLCC grade 2 (intermediate grade) with DDIT3 rearrangement on fluorescence in situ hybridization (FISH). Margins were negative but close. Postoperative PET scan and Signatera MRD assay were negative for metastasis. Given the tumor’s size (>10 cm) and known radiosensitivity, adjuvant radiotherapy (60–66 Gy) was initiated. Discussion: MLPS features myxoid stroma, plexiform vasculature, and, in high-grade tumors, a round cell component. The FUS::DDIT3 fusion gene is diagnostic. While MRI offers superior soft tissue characterization, definitive diagnosis requires pathology and molecular testing. Surgical excision with negative margins remains standard, with radiotherapy recommended for large tumors or close margins to reduce recurrence. This case highlights the limitations of preoperative imaging and the value of intraoperative pathology in guiding management. Conclusions: Early recognition, accurate diagnosis, and tailored multimodal treatment are essential for MLPS. Given the potential for recurrence, late extrapulmonary metastases, long-term surveillance with imaging, and molecular assays are critical for optimizing outcomes. Full article

Liposarcomas represent a heterogeneous group of malignant mesenchymal neoplasms, with diverse histological subtypes and molecular alterations. This study aimed to investigate the gene expression profiles of SOX9, GATA3, and GATA4 in liposarcoma subtypes and to assess their associations with clinicopathological parameters. Forty-two formalin-fixed, paraffin-embedded liposarcoma samples were analyzed. Total RNA was extracted, reverse-transcribed, and quantified by qRT-PCR using GAPDH as an endogenous control. Relative quantification (RQ) values were categorized, and statistical analyses included Fisher’s exact test, Kaplan–Meier survival analysis, and Cox proportional hazards modeling. SOX9 expression significantly varied among histological subtypes (p = 0.017), with ALT/WDLS cases showing a predominance of high-level expression (RQ > 50 in 12/15 cases), in contrast to myxoid subtypes clustering mainly in the 10–50 RQ range. GATA4 overexpression correlated with smaller tumor size (<100 mm) (p = 0.049), being more frequent in 15/20 small tumors compared to 10/22 larger ones. GATA3 and GATA4 demonstrated the strongest inter-gene correlation (r = 0.68, p < 0.05), suggesting possible functional interplay. Kaplan–Meier analysis revealed no statistically significant survival differences for individual gene expression, but a high combined GATA3–GATA4 signature was associated with a favorable trend. These findings indicate that SOX9, GATA3, and GATA4 are broadly upregulated in liposarcomas, with subtype- and size-dependent expression patterns. The strong association between GATA3 and GATA4 expression supports their potential synergistic role in tumor biology. Integration of these molecular markers into diagnostic and prognostic workflows may enhance subtype characterization and inform targeted therapeutic strategies. Further studies in larger cohorts are warranted to validate these biomarkers and explore their mechanistic interplay in liposarcoma pathogenesis. Full article

Background and Objectives: Primary mesenteric liposarcoma (LPS) is an exceptionally rare malignancy, with most literature data limited to isolated case reports or small series. This papers aims to evaluate the clinicopathological features, treatment outcomes, and prognostic factors in patients with mesenteric LPS. Materials and Methods: Thirteen patients diagnosed with primary mesenteric LPS between 2010 and 2022 were retrospectively analyzed. Data included demographics, tumor location, histological subtype, surgical treatment, recurrence, and survival. Results: The median age was 56 years (range, 22–74), with a slight male predominance (53.8%). Most tumors arose from the small bowel (53.8%) and colonic (38.5%) mesenteries, with one involving the gastric mesentery. The predominant histological subtypes were myxoid (46.1%) and dedifferentiated (23.1%). R0 resection was achieved in 76.9% of patients. During a median follow-up of 55.2 months, nine patients (69.2%) developed recurrence. Mortality was higher in patients with dedifferentiated LPS (66.7%) than in those with myxoid LPS (40%). Five-year survival rate was 100% in patients without recurrence and 28.6% in those with recurrence (p = 0.112, not significant). Patients who received adjuvant chemoradiotherapy suggested longer survival (110.7 vs. 46.2 months; p = 0.620). Conclusions: This 12-year study highlights the aggressive nature of mesenteric LPS, particularly the dedifferentiated subtype which showed the poorest prognosis. Complete resection remains the primary treatment; however, it has high recurrence rates. To diminish the catastrophic poor results of the postoperative period, multidisciplinary treatment strategies become a keystone. Full article

Background and Clinical Significance: Myxoid liposarcoma (MLS) is a malignant soft tissue tumor that often presents as a painless, slow-growing mass and is known for its atypical extrapulmonary metastatic pattern. Although sciatic nerve involvement is rare, when present, it usually causes neurologic symptoms. In this case, a large MLS silently expanded and completely encased the sciatic nerve without causing deficits, highlighting the importance of early imaging, multidisciplinary planning, and individualized surgical strategy in managing complex soft tissue sarcomas. Case Presentation: This case report describes a 67-year-old male with a 30 cm encapsulated myxoid liposarcoma of the posterior left thigh. The tumor had grown insidiously over one year and completely encased the sciatic nerve without causing pain, paresthesia, or motor impairment. Selective embolization was performed preoperatively to minimize blood loss. A posteromedial surgical approach allowed for en bloc resection with negative margins and preservation of sciatic nerve integrity. Histopathology confirmed a myxoid liposarcoma composed primarily of spindle-shaped tumor cells. The patient experienced no postoperative complications or neurologic deficits. At the two-year follow-up, he remains disease-free with full functional recovery. Conclusions: This case illustrates the potential for large, asymptomatic myxoid liposarcomas to encase critical neurovascular structures without infiltration. Preoperative embolization as part of a multidisciplinary plan was key to achieving safe resection and excellent functional outcomes. Full article

Background: Liposarcoma (LPS) is a rare malignant tumor, but it is also one of the most common adult soft-tissue sarcomas. Myxoid liposarcoma (MLPS) accounts for 30% of all LPS cases. Diagnosis during pregnancy and the puerperium is very rarely reported, and only a few cases have been reported in the thigh. Case presentation: We report the case of a 36-year-old female patient on the 11th day of the puerperium after a cesarean section. The patient presented to the gynecology ward owing to the sudden appearance of a tumor in the medial part of her right thigh. The lesion was non-painful, mobile, soft, approximately 20 cm in diameter, and protruded above the level of the rest of the thigh surface. A suspicion of hematoma was raised. The final diagnosis was high-grade MLPS. Conclusions: An MLPS diagnosis is uncommon in female patients and even rarer during pregnancy. This case represents a novel occurrence, as the first instance in which symptoms manifested during the puerperium. Proper treatment and early detection could improve disease outcomes. Full article

Background: There is an ongoing debate about the role of perioperative chemotherapy in retroperitoneal sarcoma (RPS). The aim of this study was to evaluate the effectiveness of perioperative chemotherapy in subtypes of RPS that are considered chemosensitive, including retroperitoneal angiosarcoma, undifferentiated pleomorphic sarcoma, myxoid liposarcoma, spindle cell sarcoma, and synovial sarcoma. Methods: This is a population-based retrospective cohort study. Patients with localized retroperitoneal sarcoma who underwent surgery were included from the US National Cancer Database (NCDB). After propensity score matching for the factors age, sex, grade, margin status, and tumor size, multivariable logistic and Cox regression analyses were used to identify factors associated with systemic therapy and their potential impact on the survival of patients with localized RPS. Results: We included 851 patients who underwent surgery between 2004 and 2020 (85% white, 41% female, and mean age 62 years). Of those, 227 patients (26.7%) received perioperative chemotherapy. In multivariable logistic regression, age ≤ 60 and tumor grading GIII/IV vs. GI/II were associated with a higher probability of receiving perioperative chemotherapy. After propensity score matching, we detected no difference in overall survival between patients who received chemotherapy and those who did not (HR 0.89, CI 0.55–1.43; and log-rank p = 0.92). Patient age ≤80 and tumor grading GI/II vs. GIII/IV were associated with improved overall survival. Conclusions: In this large analysis, the use of perioperative chemotherapy was not associated with improved survival in rare, chemosensitive subtypes of retroperitoneal sarcoma. However, selection bias must be considered when interpreting these findings. Full article

Purpose: Myxoid liposarcoma (MLPS) is a malignant tumor that occurs predominantly in the deep soft tissues of the extremities. Preoperative radiotherapy (RT) is used to reduce tumor volume to achieve adequate surgical margins. This systematic review aims to evaluate the impact of preoperative RT on surgical margins, local recurrence (LR) rates, metastasis development, and overall survival in patients with MLPS and associated prognostic factors. Methods: A systematic literature search was conducted by two reviewers following PRISMA guidelines on PubMed, Scopus, and the Cochrane Library on 30 November 2024. We included prospective and retrospective cohort studies published in English that evaluate surgical margin status, LR and metastasis rates, and survival outcomes in patients undergoing surgical excision of MLPS following neoadjuvant radiotherapy. Two authors extracted tumor characteristics, percentage of round cells (RCs), change in tumor volume post-RT, surgical margins, postoperative complications, LR and metastasis rates, survival rates, and related prognostic factors. Results: The twelve studies included in this review involved 1483 patients with a mean age of 44.8 years. Tumors were mostly located in the lower limbs, deeply localized, and larger than 5 cm in most cases. The average LR and metastasis rates were 5.2% and 17%, respectively. The mean 5-year and 10-year overall survival rates were 87% and 74%, respectively. Poor prognosis was associated with >5% RC components, tumors larger than 15 cm, deep localization, and inadequate surgical margins. Conclusion: The management of MLPS requires a multidisciplinary approach. Preoperative radiotherapy offers several advantages in reducing tumor volume and facilitating the achievement of adequate surgical margins, finally improving local control and long-term outcomes. Full article

Sarcomas are a heterogeneous group of malignancies with limited therapeutic options, particularly in the metastatic setting. Adoptive cellular therapies (ACTs), including tumor-infiltrating lymphocyte (TIL) therapy, chimeric antigen receptor (CAR) T-cell therapy, and T-cell receptor (TCR) gene-modified T-cell therapy, offer promising novel approaches for these refractory tumors. TIL-based therapy has demonstrated early efficacy in melanoma and myeloma, with ongoing trials exploring its role in sarcoma. CAR T-cell strategies targeting HER2, GD2, and B7-H3 antigens are in development, though challenges such as tumor microenvironment-mediated resistance and antigen escape remain significant. Engineered TCRs, particularly those targeting MAGE-A4 and NY-ESO-1, have shown promising clinical results in synovial sarcoma (SS) and myxoid/round cell liposarcoma (MRCLS), leading to the recent FDA approval of afamitresgene autoleucel (afami-cel) and letetresgene autoleucel (lete-cel). Despite encouraging preliminary data, ACT implementation faces barriers including limited antigen specificity, off-tumor toxicity, immune evasion, and manufacturing scalability. Future research will focus on optimizing lymphodepleting regimens, mitigating toxicity, enhancing in vivo persistence, and combining ACT with other therapeutic agents. As clinical trials expand, ACT holds the potential to revolutionize sarcoma treatment by offering durable, targeted therapies for previously refractory disease. Full article

Sarcomas are a heterogeneous group of neoplasms that develop from bone and soft tissue. Approximately 80% of sarcomas affect soft tissue, with liposarcoma being one of the most common types, accounting for approximately 13–20% of all soft-tissue sarcomas. Per the World Health Organization, liposarcoma can be broadly classified into four different subtypes based on histologic examination: well-differentiated liposarcoma (WDLS)/atypical lipomatous tumors (ALT), dedifferentiated liposarcoma (DDLS), myxoid liposarcoma (MLS), and pleomorphic liposarcoma (PLS). WDLS/ALT is the most common liposarcoma subtype, accounting for approximately 31–33% of liposarcomas; DDLS accounts for 20%; MLS accounts for 19%; and PLS, the least common subtype, represents 7–8% of liposarcomas. Sarcoma diagnosis is challenging because of its rarity, intrinsic complexity, and diagnostic technological complexity. Sarcomas are misdiagnosed in approximately 30% of cases, leading to delays in diagnosis and access to appropriate therapy and clinical trials. Furthermore, treatment options are limited for those diagnosed with liposarcoma. This review discusses the epidemiology, pathology, and treatment options currently available for liposarcoma. Full article

The FUS::DDIT3 fusion protein, formed by the chromosomal translocation t (12;16) (q13;p11), is found in over 90% of myxoid liposarcoma (MLS) cases and is a crucial protein in its development. Many studies have explored the role of FUS::DDIT3 in MLS, and the prevailing view is that FUS::DDIT3 inhibits adipocyte differentiation and promotes MLS growth and invasive migration by functioning as an aberrant transcription factor that affects gene expression and regulates its downstream molecules. As fusion proteins are gradually showing their potential as targets for precision cancer therapy, FUS::DDIT3 has also been investigated as a therapeutic target. Drugs that target FUS::DDIT3 and its downstream molecules for treating MLS are widely utilized in both clinical practice and experimental studies, and some of them have demonstrated promising results. This article reviews the findings of relevant research, providing an overview of the oncogenic mechanisms of the FUS::DDIT3 fusion protein in MLS, as well as recent advancements in its therapy. Full article