Primary liposarcoma (LPS) of the pancreas is extremely rare. The present report describes an additional case of primary pancreatic LPS and reviews the current literature. A 40-year-old female patient that initially presented with a fever was subsequently found to have a pancreatic tumor. CT was used to identify a heterogeneous mass in the pancreatic head and body, which had a notable absence of fat components. The tumor exhibited slightly heterogeneous enhancement during the arterial phase, moderate enhancement in the venous phase and persistent enhancement in the delayed phase, with encapsulation of the common hepatic artery, splenic vein and portal vein. The patient underwent a successful distal pancreatectomy and splenectomy. Pathological examination and next-generation sequencing confirmed the diagnosis of dedifferentiated LPS (DDLPS). The present report elucidated the CT findings of this rare case of DDLPS of the pancreas, characterized by vascular invasion and progressive enhancement but without fat components, which can serve as a reference for the diagnosis of this uncommon tumor. The uncommon CT manifestation makes differentiation from pancreatic neuroendocrine tumors particularly challenging based on imaging characteristics.

Liposarcoma, as a complex disease, is characterized by intricate interactions between distinct histopathological subtypes and corresponding clinical outcomes, emphasizing the necessity of personalized approaches in diagnosis and treatment strategies. This malignant tumor originating from adipose tissue is classified into different subtypes with specific molecular markers, which not only distinguish them but also guide treatment directions. The main approach for treating liposarcoma is surgical resection, with the aim of complete excision and achieving clean margins (R0 resection) to minimize the risk of recurrence. This surgical principle emphasizes the critical need for precise preoperative planning, and in certain cases, the integration of neoadjuvant therapy may be needed to reduce the tumor to a surgically manageable size. In addition to surgery, systemic therapy plays a key role in the advanced stages of the disease, especially when resistance to traditional treatment arises. The emergence of novel systemic therapies, including chemotherapy, targeted therapy, and immunotherapy, has opened new avenues for treating this challenging malignancy. These systemic therapies are selected on the basis of the specific molecular features of the tumor, highlighting the importance of detailed molecular diagnostics. As our understanding of the molecular basis of liposarcoma deepens, integrating clinical and molecular features is crucial for optimizing treatment outcomes. This comprehensive approach, which combines surgical precision with systemic therapy innovations, will change the treatment landscape for patients with liposarcoma, advancing toward more personalized and effective treatment strategies.

Dedifferentiated liposarcoma (DDLPS) is one of the most common types of soft tissue sarcoma (STS) characterized by liposarcomatous differentiation and a predilection for the retroperitoneum. Despite the growing number of histology-specific immune checkpoint blockade (ICB) trials in STS, it is still difficult to identify the radiographic objective response rate (ORR) for DDLPS in the real world setting. This study aimed to evaluate the ORR and survival of patients with DDLPS treated with ICB at a single center.

We conducted a retrospective study of 31 patients with pathologically confirmed DDLPS treated with ICB at MD Anderson Cancer Center between 2018 and 2023. Patient demographics, disease characteristics, treatment history, and response to ICB were analyzed. Immunohistochemical analysis was performed on tumor samples to assess immune-related markers.

ORR by RECIST 1.1 was 3.2% (n=1/31). Among all patients (n=31), 6% achieved partial radiographic response, while 39% had stable disease, and 55% showed progressive disease. Median progression-free survival (PFS) was 3.5 (95%CI:1.9, 4.7) months, and overall survival (OS) after ICB initiation was 19.7 (95%CI: 8.8, not reached) months. Patients without prior systemic therapy demonstrated better OS (p=0.004). Immunohistochemistry revealed no relationship between pre- or post-ICB expression of CD8, CD20, CD21 and PDL-1 and response.

While the response to ICB in DDLPS remains limited, specific immune markers may influence treatment outcomes. CD20/21 post-ICB appear more important for prognosis. Further research is warranted to identify predictive factors for ICB efficacy in DDLPS.

Liposarcoma with retroperitoneal location is a rare malignant tumor that poses significant diagnostic and therapeutic challenges due to its often asymptomatic nature in early stages and potential for substantial growth before detection.

We present the case of a 46-year-old female patient who experienced a 7-year history of intermittent left flank pain and was found to have a large, well-defined abdominal mass.

Urgent computed tomography scans revealed a retroperitoneal mass with characteristics suggestive of liposarcoma. Histopathological examination confirmed the diagnosis, showing features of both well-differentiated and dedifferentiated liposarcoma.

The patient underwent successful surgical resection of the mass, which measured 18 × 16 × 10 cm.

The patient had an uneventful postoperative recovery and remained disease-free during the 6-month follow-up period, demonstrating the effectiveness of timely surgical intervention.

This case underscores the importance of accurate diagnosis and complete surgical resection in managing liposarcoma with retroperitoneal location. The high rate of local recurrence necessitates careful follow-up and consideration of adjuvant therapies. Further research is warranted to explore the optimal treatment strategies for recurrent cases.

This population-based study examined pediatric liposarcoma (LPS) prognosis and developed a nomogram to predict overall survival (OS).

Data from 145 pediatric LPS patients (2000-2019) were analyzed. Survival curves were compared using log-rank tests and generated via Kaplan-Meier estimation. Cox proportional-hazards regression identified OS-associated variables, and a nomogram was constructed.

Myxoid LPS (72.5%) was the most common subtype, predominantly in extremities (66.8%). Most cases were localized (77.9%), with regional (17.2%) and distant (4.9%) stages less frequent. Surgery alone was the primary treatment (69.0%). OS rates were 90.5% (3-year) and 86.9% (5-year). Localized disease had significantly better survival than distant disease (96.2% vs. 14.3%, p < 0.001), and myxoid LPS showed superior OS (p < 0.001). Cox analysis identified primary site (HR 7.78, p = 0.004) and SEER stage (HR 30.44, p < 0.001) as independent prognostic factors.

Pediatric LPS typically presents as localized, extremity-based myxoid tumors with favorable outcomes. High-grade or distant-stage disease, though rare, correlates with poorer survival. The nomogram provides individualized OS prediction to guide clinical management.

Dedifferentiated liposarcoma (DDLPS) accounts for 15-20% of liposarcoma (LPS) and has high rates of local recurrence and distant metastasis. Hyperactivation of yes-associated protein (YAP) has been implicated in DDLPS development. However, the mechanisms that drive aberrant YAP signaling remain largely unknown. Here, we show that tumor protein p53 inducible nuclear protein 2 (TP53INP2) is a potential negative modulator of the malignant progression of DDLPS. The TP53INP2 protein expression level in tumor tissues from 79 patients with DDLPS decreased progressively. Compared with primary tumors, recurrent tumors also exhibited reduced TP53INP2 expression. More importantly, low TP53INP2 expression is correlated with poor prognosis. TP53INP2 gain- or loss-of-function experiments in DDLPS cell lines showed profound inhibitory effects on processes and properties linked with cancer malignancy, such as proliferation, migration, stemness and dedifferentiation. Mechanistically, TP53INP2 is located mainly in mitochondria and promotes mitophagic degradation of YAP in a VDAC1-dependent manner. The WW domain in YAP and the PPTY motif in VDAC1 are required for their interaction. Taken together, these data demonstrate that TP53INP2 represses the malignant progression of DDLPS by inactivating YAP via a mitophagy-dependent mechanism and that TP53INP2 may constitute a novel prognostic biomarker for advanced DDLPS.

Liposarcoma is one of the most prevalent forms of soft tissue sarcoma, and its prognosis is highly dependent on its molecular subtypes. Non-coding RNAs (ncRNAs) like microRNAs (miRNAs) and long non-coding RNAs (lncRNAs) can bind various cellular targets to regulate carcinogenesis. By affecting the expressions and activities of their downstream targets post-transcriptionally, dysregulations of miRNAs can alter different oncogenic signalling pathways, mediating liposarcoma progression. On the contrary, lncRNAs can sponge miRNAs to spare their downstream targets from translational repression, indirectly affecting miRNA-regulated oncogenic activities. In the past 15 years, multiple fundamental and clinical research has shown that different ncRNAs play essential roles in modulating liposarcoma development. Yet, there is a lack of an effective review report that could summarize the findings from various studies. To narrow this literature gap, this review article aimed to compare the findings from different studies on the tumour-regulatory roles of ncRNAs in liposarcoma and to understand how ncRNAs control liposarcoma progression mechanistically. Additionally, the reported findings were critically reviewed to evaluate the translational potentials of various ncRNAs in clinical applications, including employing these ncRNAs as diagnostic and prognostic biomarkers or as therapeutic targets in the management of liposarcoma. Overall, over 15 ncRNAs were reported to play essential roles in modulating different cellular pathways, including apoptosis, WNT/β-catenin, TGF-β/SMAD4, EMT, interleukin, and YAP-associated pathways to influence liposarcoma development. 28 ncRNAs were reported to be upregulated in liposarcoma tissues or circulation, whereas 11 were downregulated, making them potential candidates as liposarcoma diagnostic biomarkers. Among these ncRNAs, measuring the tissues or circulating levels of miR-155 and miR-195 was reported to help detect liposarcoma, differentiate liposarcoma subtypes, and predict the survival and treatment response of liposarcoma patients. Overall, except for a few ncRNAs like miR-155 and miR-195, current evidence to support the use of discussed ncRNAs as biomarkers and therapeutic targets in managing liposarcoma is mainly based on a single-center study with relatively small sample sizes or cell-based studies. Hence, more large-scale multi-center studies should be conducted to further confirm the sensitivity, specificity, and safety of ncRNAs as biomarkers and therapeutic targets. Instead of furthering investigation to confirm the translational values of all the discussed ncRNAs, which can be time- and cost-consuming, it would be more practical to focus on a few ncRNAs, including miR-155 and miR-195, to evaluate if they are sensitive and safe to be used as liposarcoma biomarkers and therapeutic agents or targets.

Amplification of chromosomal material derived from 12q13-15 is common in human cancer and believed to result in overexpression of multiple collaborating oncogenes. To define the oncogenes involved, we overexpressed genes recurrently amplified in human liposarcoma using a zebrafish model of the disease. We found several genes whose overexpression collaborated with AKT in sarcomagenesis, including the tRNA methyltransferase METTL1. This was surprising, because AKT phosphorylates METTL1 to inactivate its enzymatic activity. Indeed, phosphomimetic S27D or catalytically dead alleles phenocopied the oncogenic activity of wild-type METTL1. We found that METTL1 binds the multi-tRNA synthetase complex, which contains many of the cellular aminoacyl-tRNA synthetases and promotes tRNA aminoacylation, polysome formation, and protein synthesis independent of its methyltransferase activity. METTL1-amplified liposarcomas were hypersensitive to actinomycin D, a clinical inhibitor of ribosome biogenesis. We propose that METTL1 overexpression promotes sarcomagenesis by stimulating tRNA aminoacylation, protein synthesis, and tumor cell growth independent of its methyltransferase activity.

Liposarcoma of the gallbladder is an exceptionally rare malignancy originating from adipose tissue. Its rarity and diagnostic challenges make this case noteworthy. Liposa rcomas exhibit diverse histological subtypes, each with distinct biological behaviors, and there is limited consensus on optimal treatment approaches. This report emphasizes the importance of accurate diagnosis, effective therapeutic strategies, and detailed analysis of clinical outcomes in managing such rare cases.

A 35-year-old woman presented with a two-month history of a palpable abdominal mass accompanied by mild bloating. She reported no significant discomfort, systemic symptoms, or changes in bowel habits. Imaging revealed a large abdominal mass displacing adjacent organs. Magnetic resonance imaging suggested a mixed-signal lesion originating from the gallbladder, and laboratory tests showed elevated inflammatory markers. The patient underwent successful surgical excision of the mass and cholecystectomy. Pathological examination confirmed a well-differentiated liposarcoma closely associated with the gallbladder. Immunohistochemistry indicated positivity for CDK4, MDM2, P16, S-100, and CD34, with a low proliferation index (Ki-67 ~ 10%). Postoperative recovery was uneventful, and the patient showed significant improvement. Long-term management, including genetic testing and follow-up, was planned to monitor recurrence risk and explore potential targeted therapies.

This case underscores the importance of considering rare malignancies like liposarcoma of the gallbladder in the differential diagnosis of abdominal masses. Early diagnosis through imaging and histopathological confirmation is crucial for optimal management. Complete surgical excision remains the cornerstone of treatment, particularly for well-differentiated subtypes, which generally have favorable prognoses. The findings highlight the need for multidisciplinary care and further research into genetic and molecular mechanisms to guide future targeted treatments.

Liposarcomas are malignant soft tissue tumours with heterogeneous features and variable prognosis. Each entity comprised in this group displays distinct morphology and harbours specific genetic alterations, which correlate with clinical behaviour and therapy response. The aim of this study is to analyse the clinical and histopathological features that can influence the prognosis of liposarcoma. We also present a newly designed scoring system that could be useful for predicting the risk of disease progression and death in patients with different liposarcoma subtypes.

We carried out a retrospective multicentric study on 77 liposarcomas diagnosed between 2009 and 2023 that were followed up to assess the presence of metastases and survival of the patients. We evaluated the age, gender, tumour location and dimensions, histological subtype, mitotic index, presence and percentage of necrosis, and their association with disease progression and survival.

In this respect, progression-free survival was positively associated with lower mitotic index, somatic soft-tissue localization, well-differentiated and myxoid subtypes and absence of necrosis. Overall survival was negatively influenced by older age, higher mitotic index, dedifferentiated and pleomorphic subtypes and the presence of necrosis. Therefore, several clinical and histopathological features of liposarcomas, such as tumour location, mitotic index, and tumour necrosis can strongly predict the disease evolution.

This study focuses on developing a new scoring system that considers histologic subtype, mitotic index, and tumour necrosis as indicators that could predict the risk of disease progression and overall survival in patients with liposarcoma. The system classifies liposarcomas of any histological subtype into low-risk and high-risk tumours. Diagnosing liposarcomas using this two-tiered system could be useful for providing personalized therapy, in order to avoid relapses, metastases and improve the disease's prognosis.

Liposarcoma is a rare malignant tumor of mesenchymal origin. It accounts for ~7% of all mesenchymal sarcomas and 1% of cancers in general. Its incidence does not exceed 2.5 cases per million inhabitants per year. This tumor is generally locally invasive, often diagnosed at an advanced stage, and can grow to a considerable size and weight, culminating in a locally advanced form. We report a case of a 65-year-old patient with retroperitoneal liposarcoma treated by open surgical resection. The patient was admitted for the management of an abdomino-pelvic mass measuring 40 × 27 × 17 cm, progressively evolving over 5 years. The diagnosis was established by computed tomography scan. The postoperative course was uneventful, and after 1 year of chemotherapy, the patient died of respiratory distress.

To evaluate the efficacy and safety of anlotinib combined with envafolimab in the treatment of unresectable or metastatic liposarcoma.

This single-center, retrospective study enrolled 15 patients with unresectable or metastatic liposarcoma, who were treated at the Retroperitoneal Tumor Surgery Research Center of Qingdao University Affiliated Hospital between April 2022 and November 2023. The treatment regimen consisted of anlotinib combined with envafolimab. Treatment efficacy was evaluated using the Response Evaluation Criteria in Solid Tumors version 1.1. Treatment-related adverse events (TRAEs) were assessed using Common Terminology Criteria for Adverse Events version 5.0.

A total of 15 patients with unresectable or metastatic liposarcoma were included; among them, seven were male (46.7%) and eight were female (53.3%), with a median age of 55 years. The pathological subtype distribution was as follows: three (20.0%) patients with well-differentiated liposarcoma, 11 (73.3%) patients with dedifferentiated liposarcoma, and one (6.7%) patient with myxoid liposarcoma. At 12 weeks post-diagnosis, none of the patients achieved a complete response. The objective response rate was 6.7%, with one patient (6.7%) achieving a partial response. Disease stability was observed in 10 (66.6%) patients, which corresponded to a disease control rate of 73.3%. Disease progression occurred in four (26.7%) patients. The median follow-up time was 16.9 months and the median progression-free survival time was 14.2 months. Seven patients experienced TRAEs, of whom three (42.2%) had grade 3-4 TRAEs. The most common TRAEs were liver function abnormalities, hypertension, and fatigue.

Anlotinib combined with envafolimab demonstrates promising efficacy and manageable safety in treating unresectable or metastatic liposarcoma.

Rapidly progressing orbital liposarcomas, while rare, pose significant diagnostic challenges due to their varied clinical and radiological presentations. A 76-year-old female presented with a suspected well-differentiated orbital liposarcoma 16 months after the onset of proptosis and diplopia. Initial magnetic resonance imaging (MRI) revealed a homogeneous, high-intensity mass in the left superior orbit. Although oral corticosteroids were administered, the patient's condition worsened over the following 13 months, with subsequent MRI revealing a heterogeneous mass. Orbital exenteration was performed, and histopathological analysis confirmed the diagnosis of a well-differentiated liposarcoma despite the rapid progression and imaging changes. This case highlights that rapid clinical and radiological changes in orbital liposarcomas do not necessarily indicate dedifferentiation. The discrepancy between imaging progression and histopathological findings emphasizes the critical role of pathological evaluation in making a definitive diagnosis. Treatment decisions, including aggressive surgical approaches, should be based on a comprehensive assessment of the clinical presentation, imaging features, and histopathological characteristics tailored to the individual patient's condition and disease progression.

Liposarcoma is the most prevalent sarcoma in adults representing 20% of all sarcomas with well-differentiated/dedifferentiated among the most common subtypes represented. Despite multimodality treatment approaches, there has not been any appreciable change in survival benefit in the past 10 years. The future of targeted therapy for WD/DDLPS is promising with the intention to spare multi-visceral removal due to radical surgical resection. Therefore, there is a need to expand upon the molecular landscape of WDLPS and DDLPS which can help identify potential therapeutic targets for the treatment of this disease. Targeted transcriptome analysis using the NanoString tumor signaling 360 panel revealed a dysregulation in glucose metabolism and HIF1 signaling pathways in both WDLPS and DDLPS when compared to normal fat controls. WDLPS, however, demonstrated upregulation of HIF-1A and TGF-β when compared to DDLPS by targeted transcriptome analysis and orthogonal validation by RT-qPCR suggesting activation of EMT pathway in WDLPS when compared to DDLPS. Our findings implicate a putative role for dysregulation in glucose metabolism, TGF-β and HIF1 signaling in the pathogenesis of both WD/DDLPS suggesting a possible proinflammatory tumor environment within WDLPS and subsequent activation of the TGF-β signaling pathway.

Myxoid liposarcoma, a rare type of tumor, accounts for approximately 30% of all liposarcomas. Myxoid liposarcomas harboring the FUS/CHOP fusion gene have shown promising results with trabectedin in basic research and some clinical experiments. However, the efficacy and safety of trabectedin in chemotherapy-naive soft tissue sarcomas or FUS/CHOP fusion gene-positive myxoid liposarcomas have not yet been established. Therefore, we evaluated the effectiveness and safety of trabectedin monotherapy in four cases of myxoid liposarcoma harboring the FUS/CHOP fusion gene at our hospital.

We analyzed four patients with metastatic myxoid liposarcoma who underwent surgery at Okayama University and received chemotherapy at Kawasaki Medical School. These patients had positive test results for the FUS/CHOP fusion gene as an aid to pathological diagnosis by RT-PCR. RNA was extracted from tumor tissue sliced from frozen tumor specimens. Following reverse transcription, PCR was performed using TLS/FUS-CHOP primers. The resulting products were electrophoresed, and then the nucleotide sequences were confirmed.

Case 1: A 44-year-old male started trabectedin as second-line therapy after initial chemotherapy, which included doxorubicin. To date, he has completed 9 cycles, showing a response for 6 months. Case 2: A 71-year-old male, deemed intolerant to doxorubicin, started trabectedin as his first-line treatment. He has undergone 50 cycles to date, maintaining a response for 56 months. Case 3: A 59-year-old female began trabectedin as second-line therapy after initial chemotherapy, including doxorubicin. She responded for 6 months before experiencing disease progression. Case 4: A 79-year-old male developed new lesions after one course of initial chemotherapy, including doxorubicin. He then began trabectedin and has maintained a response for 10 months to date.

Compared to other chemotherapies, trabectedin demonstrated potentially higher efficacy and a favorable safety profile for patients with myxoid liposarcoma harboring the FUS/CHOP fusion gene.

Liposarcoma (LPS) is a kind of malignancy of soft tissue usually found in the retroperitoneal, limb, or neck region, and some may be detected with delayed symptoms (pain or palpable mass), and less frequently occurs in organs of the digestive system. In contrast, Dedifferentiated liposarcoma (DDLPS) is a common histological subtype of LPS. The present study reported a case of dedifferentiated liposarcoma originating in the gallbladder. Differentiated liposarcoma originating from the gallbladder is rarely reported.

A 64-year-old female patient presented to our hospital with a painless abdominal mass. Abdominal computed tomography (CT) showed that the gallbladder had lost its normal shape, and a 9.1 cm × 7.1 cm × 12.1 cm mass was seen in the area of the gallbladder fossa and the right upper abdomen below it, which had an irregular morphology, inhomogeneous density, and nodular calcification, with marked inhomogeneous enhancement on enhancement scan. Preoperative tumor markers and liver function indicators were not abnormal. With suspicion of a giant malignant tumor of the gallbladder, she underwent a cholecystectomy combined with abdominal mass resection. After surgery, the tumor and gallbladder, were completely resected, and postoperative pathological results confirmed the diagnosis of dedifferentiated liposarcoma deriving from gallbladder. After surgery, the patient and his family refused to continue treatment. After 15 months follow-up, the patient remains asymptomatic and does not show any signs of recurrence. And she is now under continued follow - up.

Treatment of dedifferentiated liposarcoma is still at exploratory stage, and a lack of clinical evidence for this condition might hinder access to clinical trials and studies. Currently, the treatment of choice for dedifferentiated liposarcoma remains radical resection. In the available clinical studies, there are no robust data to support clinical use of neoadjuvant and adjuvant radiochemotherapy. As with other diseases, the use of radiotherapy and chemotherapy before and after surgery may be a potential future treatment.

MicroRNAs (miRNAs) are pivotal regulators of gene expression, influencing key cellular processes such as proliferation, differentiation, apoptosis, and metastasis. In the realm of sarcomas-a diverse group of malignant tumors affecting soft tissues and bone sarcomas-miRNAs have emerged as crucial players in tumorigenesis and tumor progression. This review delves into the intricate roles of miRNAs across various soft tissue sarcoma subtypes, including rhabdomyosarcoma, liposarcoma, leiomyosarcoma, synovial sarcoma, fibrosarcoma, angiosarcoma, undifferentiated pleomorphic sarcoma (UPS), and malignant peripheral nerve sheath tumor (MPNST). We explore how dysregulated miRNAs function as oncogenes or tumor suppressors, modulating critical pathways that define the aggressive nature of these cancers. Furthermore, we discuss the diagnostic and prognostic potential of specific miRNAs and highlight their promise as therapeutic targets. By understanding the miRNA-mediated regulatory networks, this review aims to provide a comprehensive overview of current research while pointing towards future directions for miRNA-based therapies. Our findings underscore the potential of miRNAs to transform the landscape of sarcoma treatment, offering hope for more precise, personalized, and effective therapeutic strategies.

Liposarcoma is one of the most common soft tissue malignancies. We previously discovered upregulation of minichromosome maintenance 2 (MCM2) expression in liposarcoma tissues. Hereon, we attempt to clarify the biological influence and mechanisms of MCM2 in liposarcoma. The mRNA level of MCM2 expression was detected through the use of quantitative real-time PCR. Immunohistochemistry staining and western blot were employed to detect protein expression of MCM2. The protein expression of fibroblast-activation protein and α-smooth muscle actin was examined by immunofluorescence. Protein concentrations of interleukin (IL)-6, transforming growth factor β, and IL-8 were measured via ELISA. Furthermore, liposarcoma cell viability was assessed through cell counting kit-8 assay, and liposarcoma cell invasiveness and migration were evaluated through transwell assay. For assessing proliferation and apoptosis of liposarcoma cells, colony formation assay and flow cytometry were used. For constructing a mouse tumor model, SW872 cells were introduced into mouse flank via subcutaneous injection. MCM2 expression was boosted in liposarcoma tissues and cells when compared with the controls. MCM2-activated cancer-associated fibroblasts (CAFs)-like phenotype, presenting as increased fibroblast-activation protein expression, α-smooth muscle actin expression, cell migration, IL-6 concentration, IL-8 concentration, and transforming growth factor β concentration. Functional experiments indicated that MCM2-activated-CAFs facilitated proliferation, migration, and invasion of liposarcoma cells. Additionally, 1 μM doxorubicin treatment could not affect proliferation and apoptosis of liposarcoma cells, whereas combined use of MCM2 knockdown and 1 μM doxorubicin evidently repressed cell proliferation and promoted apoptosis. In vivo, silencing of MCM2 impaired tumor growth in mice. MCM2 overexpression promoted CAFs formation and tumor progression, showing potential value in treatment of liposarcoma.

Pulmonary nodules in patients with soft tissue sarcomas are likely pulmonary metastases, whereas synchronous primary pulmonary sarcomas are rare. Without surgery, determining whether a solitary pulmonary nodule is a primary or metastatic nodule is difficult. Herein, we report a rare case of a primary pulmonary sarcoma that presented synchronously with a primary dedifferentiated liposarcoma.

A 77-year-old man presented to another hospital with left inguinal swelling and a suspected recurrent inguinal hernia. Computed tomography revealed a left inguinal mass and pure-solid nodule in the left lung and the patient was referred to our hospital for detailed examination and treatment. The inguinal mass was pathologically diagnosed as a dedifferentiated liposarcoma using needle biopsy, whereas bronchoscopic biopsy revealed histological findings suggestive of a sarcoma; however, the primary site could not be determined. Positron emission tomography-computed tomography revealed no high-accumulation lesions except for the two sarcomas. We decided to perform surgery on both sarcomas for diagnostic and curative purposes. The surgical specimens showed that the two sarcomas were different. Based on the immunohistochemical staining findings of MDM2, a left inguinal dedifferentiated liposarcoma and primary pulmonary unclassified sarcoma were diagnosed. The patient displayed no evidence of recurrence 1 year after surgery.

We encountered a rare case of synchronous multiple primary sarcomas, one presenting in the lung and the other in the soft tissue. Surgery was required to achieve a definitive diagnosis for the patient, who achieved disease-free survival at 1 year. This case suggests that proactive resection of pulmonary nodules in patients with soft tissue sarcomas may be feasible as a diagnostic treatment if complete resection is achieved.

Liposarcomas (LPSs) are rare malignant tumors of adipocytic origin, primarily occurring in the extremities and retroperitoneum, with thoracic involvement being exceptionally rare. This case report details the surgical management and outcomes of a recurrent intrathoracic LPS in a 65-year-old male with a history of previous mediastinal tumor resection. CT imaging revealed a recurrent tumor extending into the left pleura. The patient underwent a posterolateral thoracotomy for complete tumor excision and limited replacement of the descending aorta. Postoperative recovery was smooth, and histology confirmed dedifferentiated LPS (G2) with areas of highly differentiated LPS. LPSs encompass a heterogeneous group of tumors with various subtypes, including atypical lipomatous tumor/well-differentiated liposarcoma (ALT/WDLPS), dedifferentiated liposarcoma (DDLPS), myxoid liposarcoma (MLPS), and pleomorphic liposarcoma (PLPS). Treatment primarily involves complete surgical resection, while the roles of radiotherapy and chemotherapy remain debated. Immunotherapy shows potential benefits, particularly for DDLPS patients expressing PD-L1. Prognosis varies significantly by subtype, with DDLPS and PLPS associated with poorer outcomes compared to MLPS and ALT/WDLPS. Long-term follow-up is crucial for managing LPSs due to their high recurrence rate. This case highlights the effectiveness of surgical intervention in recurrent intrathoracic LPSs and underlines the need for continued research into adjuvant therapies to improve patient outcomes.

Soft tissue sarcomas account for less than 1% of new cancer diagnoses, approximately one in five of which are liposarcomas. These tumors typically arise in the deep tissues of the proximal extremity or retroperitoneum, with just under 3% presenting as primary intrathoracic neoplasms. We present an exceedingly rare and particularly unique presentation of primary lung liposarcoma which traversed the mediastinum into the contralateral hemithorax. This report highlights the primary characteristics of the disease and underscores the importance of a multidisciplinary approach to its successful treatment.

As a common soft tissue sarcoma, liposarcoma (LPS) is a heterogeneous malignant tumor derived from adipose tissue. Due to the high risk of metastasis and recurrence, the prognosis of LPS remains unfavorable. To improve clinical treatment, a robust risk prediction model is essential to evaluate the prognosis of LPS patients.

By comprehensive analysis of data derived from GEO datasets, differentially expressed genes (DEGs) were obtained. Univariate and Lasso Cox regressions were subsequently employed to reveal distant recurrence-free survival (DRFS)-associated DEGs and develop a prognostic gene signature, which was assessed by Kaplan-Meier survival and ROC curve. GSEA and immune infiltration analyses were conducted to illuminate molecular mechanisms and immune correlations of this model in LPS progression. Furthermore, a correlation analysis was involved to decipher the therapeutic significance of this model for LPS.

A six-gene signature was developed to predict DRFS of LPS patients and showed higher precision performance in more aggressive LPS subtypes. Then, a nomogram was further established for clinical application based on this risk model. Via GSEA, the high-risk group was significantly enriched in cell cycle-related pathways. In the LPS microenvironment, neutrophils, memory B cells and resting mast cells exhibited significant differences in cell abundance between high-risk and low-risk patients. Moreover, this model was significantly correlated with therapeutic targets.

A prognostic six-gene signature was developed and significantly associated with cell cycle pathways and therapeutic target genes, which could provide new insights into risk assessment of LPS progression and therapeutic strategies for LPS patients to improve their prognosis.

Liposarcomas are some of the most challenging soft tissue tumors and are subclassified into multiple subtypes with special histologic and molecular features. The peculiarities of each histopathological subtype influence the clinical behavior, management, and treatment of these neoplasms. For instance, well-differentiated liposarcomas are common soft tissue malignancies and usually display a favorable outcome. On the other hand, pleomorphic liposarcoma is the rarest, yet the most aggressive subtype of liposarcoma. This histopathological diagnosis may be challenging due to the scarce available data and because pleomorphic liposarcomas can mimic other pleomorphic sarcomas or other neoplasms of dissimilar differentiation. Nevertheless, the correct diagnosis of pleomorphic liposarcoma is of utmost importance as such patients are prone to develop local recurrences and metastases. Treatment usually consists of surgical excision along with radiotherapy and follow-up of the patients. Therefore, this review aims to assess the complex clinical, histological, and immunohistochemical features of liposarcomas in order to establish how these characteristics influence the management and prognosis of the patients, emphasizing the particularities of pleomorphic liposarcoma.

To describe a series of eight adult patients with primary orbital sarcoma and to review the existing literature on orbital sarcoma and post-irradiation sarcoma.

Report of eight cases and literature review.

We report eight cases of primary orbital sarcoma, three of which were radiation-induced. Only one patient had a history of retinoblastoma. The most common presentations were painful proptosis and reduced vision. Most tumours arose in the extraconal compartment. The overall median age at diagnosis was 50 years. The pathology comprised a diverse group of tumours. Treatment and outcome varied between patients and their clinical circumstances.

Adult primary orbital sarcomas are rare. They can comprise a variety of tumour types and are difficult to treat. Irradiation is a significant risk factor, and the incidence of post-irradiation sarcoma of the orbit may be increasing due to the widespread use of radiotherapy and improved survival of patients with cancer. Post-irradiation sarcoma should be considered in the differential diagnosis of an orbital space-occupying lesion in a patient with a history of radiotherapy.

Myxoid/round cell liposarcomas (MRCLPS) are a rare soft tissue sarcoma. We report the largest sarcoma in our institutional history. We discuss the patient's surgical management and treatment of the tumor and challenges given its dimensions. Several complications arose following primary resection that were managed by a multidisciplinary team. Although MRCLPS can vary in size, large MRCLPS must be treated cautiously given the potential for complications. Additionally, multidisciplinary treatment of MRCLPS is essential in diagnosing and treating these complex cases.

Gastric liposarcoma (GL) is extremely rare and radical surgery has been the conventional treatment, even in small tumors. Laparoscopic wedge resection has been reported worldwide for subepithelial tumors of the stomach.

The patient was an asymptomatic 63-year-old man presenting with a subepithelial gastric tumor. The esophagogastroduodenoscopy showed a 3 cm ulcerated soft tumor located in the posterior wall of the antrum just above the pylorus. Two preoperative biopsies were performed with a negative result for malignant neoplasm. Dynamic computerized tomography revealed 35 × 35 mm well-defined pyloric mass with fat density. Despite the difficult location of the tumor, function-preserving surgery was performed. Surgery was initiated by a laparoscopic approach with four trocars. After the dissection of the greater omentum, the greater curvature and the posterior wall of the stomach were exposed. A gastrostomy was performed in the anterior wall of the antrum. Due to the difficulty in identifying the tumor location, a mini-laparotomy was conducted. After assessing the pylorus and section parameters, the tumor was extracted by gastrostomy and resected with a linear stapler. The patient was discharged after five days with no complications. The histological diagnosis was a well-differentiated liposarcoma. Resection margins were clear. The tumor cells tested negative for MDM2. No adjuvant therapy was indicated. The patient is alive without recurrence.

Despite its rarity, gastric liposarcoma should be respected for differential diagnosis in submucosal tumors. The main diagnostic method is histological, and surgery is the conventional treatment without yet having a consensus. Minimally invasive wedge resection might be a suitable treatment even if the location is close to the pylorus. Multicenter studies are required to obtain better results in the management of this pathology.

Large mediastinal lipomas are rare. Complete surgical resection can be difficult due to the intricate anatomy in the mediastinum. We report the case of a 75-year-old man with worsened retrosternal pressure, decline in performance and syncope episodes. Computed tomography revealed a large retrocardiac low-attenuated mediastinal lesion measuring 10 × 8 cm, compressing the left atrium and pulmonary veins bilaterally. Surgical exploration was achieved through a right anterolateral thoracotomy with a successful en bloc resection without any intraoperative complications. The total operation time was 185 min with a total blood loss of <250 ml. Stand-by extracorporeal life support was present throughout the procedure, but its use was not required. The postoperative course was uneventful. The pathological examination revealed a mature mediastinal lipoma without any evidence of malignancy. In the 12-month control the patient was completely free of symptoms and in a good general condition.

Paratesticular liposarcoma (PTL) is a rare condition, with fewer than 200 cases reported worldwide. It is a malignant tumor that originates from fat tissue with high risk of recurrence. Herein, the authors present a contralateral recurrence of a treated PTL. To the best of the authors' knowledge, in the current literature, there are few cases reported with recurrent PTL.

The authors report the case of a 62-year-old man who presented with a rapidly growing painless right hemiscrotal swelling. Clinical and radiographic evidence suggested the presence of two paratesticular tumors. The patient underwent a radical orchidectomy with resection of the two tumors through an inguinal approach. The histologic examination revealed a sclerosing, well-differentiated liposarcoma. The decision of the multidisciplinary consultation meeting was not to do adjuvant treatment. A follow-up of 12 months showed recurrence of the contralateral scrotum revealed by an FDG-PET/scan.

PTL, a rare spermatic cord tumor, affects adults aged 50-60, often presenting with scrotal swelling. Diagnosis involves ultrasound, computed tomography, and magnetic resonance imaging. Surgical intervention, including radical orchiectomy and adjuvant radiotherapy, is common for management, while the role of chemotherapy is inconclusive. High-grade subtypes carry a higher recurrence risk.

PTL is often misdiagnosed preoperatively. It is typically managed through radical orchidectomy, which includes wide excision and high ligation to ensure free surgical margins and avoid recurrence. The role of adjuvant therapy remains debatable. Despite a generally favorable prognosis, long-term follow-up is crucial because of the elevated risk of recurrence.

Soft tissue sarcoma (STS) is a relatively rare malignancy, accounting for about 1% of all adult cancers. It is known to have more than 70 subtypes. Its rarity, coupled with its various subtypes, makes early diagnosis challenging. The current standard treatment for STS is surgical removal. To identify the prognosis and pathophysiology of STS, we conducted untargeted metabolic profiling on pre-operative and post-operative plasma samples from 24 STS patients who underwent surgical tumor removal. Profiling was conducted using ultra-high-performance liquid chromatography-quadrupole time-of-flight/mass spectrometry. Thirty-nine putative metabolites, including phospholipids and acyl-carnitines were identified, indicating changes in lipid metabolism. Phospholipids exhibited an increase in the post-operative samples, while acyl-carnitines showed a decrease. Notably, the levels of pre-operative lysophosphatidylcholine (LPC) O-18:0 and LPC O-16:2 were significantly lower in patients who experienced recurrence after surgery compared to those who did not. Metabolic profiling may identify aggressive tumors that are susceptible to lipid synthase inhibitors. We believe that these findings could contribute to the elucidation of the pathophysiology of STS and the development of further metabolic studies in this rare malignancy.

Malignancies represent a persisting worldwide health burden. Tumor treatment is commonly based on surgical and/or non-surgical therapies. In the recent decade, novel non-surgical treatment strategies involving monoclonal antibodies (mAB) and immune checkpoint inhibitors (ICI) have been successfully incorporated into standard treatment algorithms. Such emerging therapy concepts have demonstrated improved complete remission rates and prolonged progression-free survival compared to conventional chemotherapies. However, the in-toto surgical tumor resection followed by reconstructive surgery oftentimes remains the only curative therapy. Breast cancer (BC), skin cancer (SC), head and neck cancer (HNC), and sarcoma amongst other cancer entities commonly require reconstructive surgery to restore form, aesthetics, and functionality. Understanding the basic principles, strengths, and limitations of mAB and ICI as (neo-) adjuvant therapies and treatment alternatives for resectable or unresectable tumors is paramount for optimized surgical therapy planning. Yet, there is a scarcity of studies that condense the current body of literature on mAB and ICI for BC, SC, HNC, and sarcoma. This knowledge gap may result in suboptimal treatment planning, ultimately impairing patient outcomes. Herein, we aim to summarize the current translational endeavors focusing on mAB and ICI. This line of research may serve as an evidence-based fundament to guide targeted therapy and optimize interdisciplinary anti-cancer strategies.

A man in his 60s presented to an outside hospital with persistent groin pain and a scrotal mass which was thought to be a recurrent hernia. Three months after initial presentation, the patient was found to have dedifferentiated liposarcoma (LPS) of the spermatic cord. LPS of the spermatic cord is a rare entity; however, clinicians should have LPS on the differential diagnosis especially in men with recurrent scrotal pain and mass. If unrecognised, LPS is associated with a high degree of morbidity and mortality. LPS can be subdivided into well-differentiated LPS, dedifferentiated LPS, myxoid LPS and pleomorphic LPS. In patients with advanced or metastatic LPS, chemotherapy consisting of Adriamycin, ifosfamide and mesna is used despite LPS being relatively chemoresistant. Therapies inhibiting mouse double minute 2 homologue, an oncoprotein that is a negative regulator of the tumour suppressor p53, appear to be promising in preclinical trials.

A 42-year-old man visited our hospital due to a gradually swelling subcutaneous mass on the back of the right shoulder. The biopsy specimen was diagnosed pathologically as pleomorphic liposarcoma. Systemic computed tomography and 18F-fluorodeoxyglucose positron emission tomography revealed multiple organ metastases, including involvement of the heart, skin, liver, bone, and lung. Six cycles of doxorubicin plus ifosfamide initially controlled the disease. However, newly developed lung metastases grew rapidly during subsequent cycles of chemotherapy, and the patient died 10 months after the initial diagnosis. The initial presentation of multiple organ involvement, including the heart, is a rare clinical manifestation of pleomorphic liposarcoma.

Liposarcomas are described as soft tissue sarcomas derived from adipose tissue. The finding of this tumor in the mandibular region is exceedingly rare. As of now, it has been described mainly in case reports and small series. A multidisciplinary approach is required to offer optimal treatment and may involve surgery, radiation and systemic therapies. Surgical repair of these defects represents a major challenge in oral and maxillofacial reconstructive surgery. We present the case of a 54-year-old man referred to our center with a progressively increasing mass in the anterior portion of the mandible. Biopsy revealed a well-differentiated myxoid liposarcoma. Resection of the tumor was performed with an additional primary reconstruction.

Dedifferentiated liposarcoma (DDL), a member of malignant mesenchymal tumors, has a high local recurrence rate and poor prognosis. Pyroptosis, a newly discovered programmed cell death, is tightly connected with the progression and outcome of tumor.

The aim of this study was to explore the role of pyroptosis in DDL.

We obtained the RNA sequencing data from The Cancer Genome Atlas (TCGA) and Genotype-Tissue Expression databases to identify different pyroptosis-related genes (PRGs) expression pattern. An unsupervised method for clustering based on PRGs was performed. Based on the result of cluster analysis, we researched clinical outcomes and immune microenvironment between clusters. The differentially expressed genes (DEGs) between the two clusters were used to develop a prognosis model by the LASSO Cox regression method, followed by the performance of functional enrichment analysis and single-sample gene set enrichment analysis. All of the above results were validated in the Gene Expression Omnibus (GEO) dataset.

Forty-one differentially expressed PRGs were found between tumor and normal tissues. A consensus clustering analysis based on PRGs was conducted and classified DDL patients into two clusters. Cluster 2 showed a better outcome, higher immune scores, higher immune cells abundances, and higher expression levels in numerous immune checkpoints. DEGs between clusters were identified. A total of 5 gene signatures was built based on the DEGs and divided all DDL patients of the TCGA cohort into low-risk and high-risk groups. The low-risk group indicates greater inflammatory cell infiltration and better outcome. For external validation, the survival difference and immune landscape between the two risk groups of the GEO cohort were also significant. Receiver operating characteristic curves implied that the risk model could exert its function as an outstanding predictor in predicting DDL patients' prognoses.

Our findings revealed the clinical implication and key role in tumor immunity of PRGs in DDL. The risk model is a promising predictive tool that could provide a fundamental basis for future studies and individualized immunotherapy.

Liposarcoma (LPS) is one of the most common adult soft-tissue sarcomas (STS), characterized by a high diversity of histopathological features as well as to a lesser extent by a spectrum of molecular abnormalities. Current targeted therapies for STS do not include a wide range of drugs and surgical resection is the mainstay of treatment for localized disease in all subtypes, while many LPS patients initially present with or ultimately progress to advanced disease that is either unresectable, metastatic or both. The understanding of the molecular characteristics of liposarcoma subtypes is becoming an important option for the detection of new potential targets and development novel, biology-driven therapies for this disease. Innovative therapies have been introduced and they are currently part of preclinical and clinical studies. In this review, we provide an analysis of the molecular genetics of liposarcoma followed by a discussion of the specific epigenetic changes in these malignancies. Then, we summarize the peculiarities of the key signaling cascades involved in the pathogenesis of the disease and possible novel therapeutic approaches based on a better understanding of subtype-specific disease biology. Although heterogeneity in liposarcoma genetics and phenotype as well as the associated development of resistance to therapy make difficult the introduction of novel therapeutic targets into the clinic, recently a number of targeted therapy drugs were proposed for LPS treatment. The most promising results were shown for CDK4/6 and MDM2 inhibitors as well as for the multi-kinase inhibitors anlotinib and sunitinib.

Myxoid liposarcoma (LPS) has a unique tendency to spread to extrapulmonary sites, including osseous sites such as the spine, and adjacent sites such as the paraspinous tissue. No clear consensus exists to guide the approach to imaging in these patients.

The aim of this study was to investigate the rate and distribution of spine metastases in patients with myxoid LPS and detection modality.

Records of all patients with myxoid LPS evaluated at our sarcoma center were retrospectively reviewed. Disease patterns and imaging modality utilization were analyzed.

Between 2000 and 2020, 164 patients with myxoid LPS were identified. The majority (n = 148, 90%) presented with localized disease, with half (n = 82, 50%) of all patients developing metastases or recurrence during their disease course. With a median follow-up of 69.2 months, spine/paraspinous metastases developed in 38 patients (23%), of whom 35 (92%) already had synchronous, non-spine metastases. Spine disease was only visible on magnetic resonance imaging (MRI), as opposed to other imaging modalities, for over one-quarter of patients with spine metastases (n = 10). For patients with metastatic disease, spine metastases were associated with worse median overall survival (2.1 vs. 8.7 years, p < 0.001).

Spine metastases occurred in nearly one-quarter of patients with myxoid LPS and represented an advanced disease state, as they primarily presented in the setting of synchronous, non-spine metastases, and were associated with worse overall survival. Routine surveillance with spine MRI in patients with localized disease likely provides no benefit but may be considered in those with known metastatic disease.

Primary liposarcoma of the breast is an uncommon soft tissue malignant tumor, comprising only 0.003% of all malignant breast tumors. The main differential diagnosis of this mass consists of malignant phyllodes tumor and metaplastic breast carcinoma. The objective of this paper is to report a case of dedifferentiated breast liposarcoma, therapeutic approach and outcome. We present a case of a 79-year-old woman complaining of a large mass in her left breast which had increased in size over the last 6 months. Physical examination revealed an enlarged left breast, and a total body CT scan showed a large tumor in contact with the musculature of the anterior thoracic wall, with no metastatic lesions. The histopathology report of a fine needle biopsy described a high-grade sarcoma. The Oncological Tumor Board recommended neoadjuvant radiotherapy sessions and reevaluation by MRI and CT scans. The patient underwent radical mastectomy with latissimus dorsi myo-cutaneous flap reconstruction. The final histopathology diagnosis was a grade 3 dedifferentiated liposarcoma (FNCLCC), with certain response to radiotherapy and positive MDM2, CDK4 markers. The postoperative period was uneventful; 12 months after surgery, the follow-up CT scan showed multiple pulmonary lesions with metastatic characteristics. Liposarcoma is a very rare type of breast cancer, and the most important treatment for breast sarcoma is surgery, the role of axillary lymph node removal, chemotherapy and radiotherapy still being controversial. Considering such cases are scarce and the development of surgical guidelines is difficult, reporting any new case is crucial.

Many patients with high-risk soft tissue sarcoma (STS) develop distant metastases. Meta-analyses suggest that chemotherapy confers a small survival benefit, though few studies focus on neoadjuvant chemotherapy (NCT). There has been more frequent use of neoadjuvant radiation therapy (NRT) in STS, but the utility of NCT for these patients remains unclear.

Patients with stage II-III trunk/extremity STS who underwent NRT and resection were identified using the National Cancer Database (2006-2019). Predictors of NCT were analyzed using logistic regression. Change in rate of NCT use over time was assessed using log-linear regression modeling. Survival was examined using Kaplan-Meier (KM) and Cox proportional hazard modeling.

Of 5740 patients, 25% underwent NCT. The overall median age was 62, 55% of patients were male, and 67% had stage III disease. The most common histological subtypes were fibrosarcoma/myxofibrosarcoma (39%) and liposarcoma (16%). Use of NCT decreased by 4.0% per year throughout the study period (p < 0.01). Predictors of NCT included younger age (median 54, IQR 42-64 vs. median 65, IQR 53-75, p < 0.01), treatment at an academic center (odds ratio [OR] 1.5, p < 0.01), and stage III disease (OR 2.2, p < 0.01). Histologic predictors of NCT included synovial sarcoma (52%) and angiosarcoma (45%). With a median follow-up time of 77 months, NCT was associated with improved 5-year survival compared to NRT alone on KM analysis (70% vs. 63%, p < 0.01). This difference persisted on multivariate analysis (hazard ratio 0.86, p = 0.027) and after propensity matching (70% vs. 65%, p = 0.0064).

Despite risk of distant failure in high-risk STS, use of NCT has decreased over time in patients receiving NRT. In this retrospective analysis, NCT was associated with a modestly improved overall survival.