Subtype Of Liposarcoma Research Articles

Comprehensive Analysis of a Six-Gene Signature Predicting Survival and Immune Infiltration of Liposarcoma Patients and Deciphering Its Therapeutic Significance.

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.

Pleomorphic Liposarcoma Unraveled: Investigating Histopathological and Immunohistochemical Markers for Tailored Diagnosis and Therapeutic Innovations.

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.

Mixed liposarcoma: A study of socioeconomic and demographic factors.

e23527 Background: Mixed Liposarcoma (LS) is an extremely rare subtype of LS, with characteristics often spanning 2 or more of the other LS subtypes such as myxoid, round cell, or well-differentiated LS. To date, the cytologic features of this uncommon tumor has yet to be described. Analyzing trends in diagnoses may produce valuable information about its epidemiology. The National Cancer Database (NCDB) was analyzed to uncover the demographic factors in patients diagnosed with mixed LS. Methods: A retrospective cohort study from 2004 to 2020 was conducted using the NCDB. 199 patients were found with a histologically confirmed diagnosis of mixed LS (ICD-8855-3). Race, gender, Hispanic origin, type of treatment facility, insurance coverage, and distance from patient residence to treatment facility were analyzed via descriptive statistics, and trends in incidence were interpreted using regression analysis. Results: The majority of patients were white (89%) and non-Hispanic (83%) with a relatively equal split of males (56%) and females (44%). The most common primary site of mixed LS was the retroperitoneum (72%), and the frequency of diagnosis decreased from 2004 to 2020 (R2 = 0.4568). Patients were treated at academic/research programs (45%), comprehensive community cancer programs (25%) and integrated network cancer programs (18%) that were generally located in the Middle Atlantic (19%), South Atlantic (18%), and East North Central (16%). The two primary payers at diagnosis include private insurance/managed care (45%) and Medicare (43%). Most patients were from counties in metro areas with a population of at least 1 million (57%) followed by metro counties with a population of 250,000 to 1 million (18%). The largest median household income quartile was $74,063 or more (38%), followed by $46,227 - $57,856 (22%). Most patients had a Charlson-Deyo Score of 0 (83%) with a NCDB analytic stage of either I (28%), III (26%), or not applicable (21%). Conclusions: To the best of our knowledge this NCDB analysis on mixed LS is the first of its kind and therefore addresses a significant knowledge gap. Most patients tend to be white and non-Hispanic. Mixed LS most commonly is found in the retroperitoneum, which is similar to case reports. We found that mixed LS patients have a relatively high household income, most often live in high populated metro areas, and are most frequently treated at academic/research programs. These findings are significant because these socioeconomic factors have not been noted before in literature. Further examination of these demographic and socioeconomic factors is required to understand how they impact treatment, diagnosis of, and survival of patients with mixed LS.

Maintenance treatment with toripalimab and anlotinib after anthracycline-based chemotherapy in patients with advanced soft tissue sarcoma (TORAN): A single-arm, phase 2 trial.

11572 Background: In patients with advanced soft tissue sarcoma (STS) who have achieved disease control after anthracycline-based chemotherapy, maintenance therapy is being explored. This study evaluated the efficacy and safety of toripalimab (a monoclonal antibody against the programmed cell death-1 receptor) combined with anlotinib (a multitargeted tyrosine kinase inhibitor) as a maintenance treatment. Methods: Patients with advanced STS who have achieved disease control after at least four cycles of first-line anthracycline-based chemotherapy were enrolled in this single-arm, phase 2 trial. Fifty patients are planned to receive treatment with toripalimab and anlotinib. The primary endpoint was progression-free survival rate at 24 weeks (PFR24w) during maintenance therapy. Secondary endpoints were median progression-free survival (PFS), median overall survival (OS), objective response rate (ORR), disease control rate (DCR) and safety. Results: By the cut-off date of Jan 25, 2024, 22 patients were enrolled, including patients with leiomyosarcoma (n =5), synovial sarcoma (n =3), epithelioid sarcoma (n =3), dedifferentiated liposarcoma (n =2) and other different subtypes of sarcomas (n =9). The median follow-up time was 11 months. Response has been evaluated in 18 patients. The ORR and DCR were 22.2% and 100%. The PFR24w was 67%, the median PFS was 11.8 months, and the median OS was not reached. The grade 3 or higher adverse events were hypertension (n =4), hemorrhage (n =1), adrenal insufficiency (n =1) and hypertriglyceridemia (n =1). Conclusions: Maintenance therapy with toripalimab and anlotinib is a promising treatment option for patients with advanced STS after first-line anthracycline-based chemotherapy. Clinical trial information: ChiCTR2100054901.

Sclerosing well-differentiated liposarcoma: two diagnostically challenging mimicker cases and a literature review.

Liposarcoma is a malignant soft tissue tumor with several subtypes, the most common of which is well-differentiated liposarcoma (WDL) or atypical lipomatous tumor (ALT). WDL/ALTs are further divided into three histological subtypes, including lipoma-like, sclerosing, andinflammatory. While the majority of these tumors are predominantly fatty, the sclerosing variant demonstrates diverse histologic and radiographic characteristics, including variable amounts of fibrosis and fat. Because of this histological variability and relative rarity, the sclerosing WDL/ALT can present diagnostic dilemmas. We present two cases of sclerosing WDL/ALT, both of which demonstrated high degrees of fibrosis and a paucity of fat, mimicking desmoid fibromatosis and other fibrotic soft tissue tumors. Thus, it is important for radiologists to be aware of the subtypes of liposarcoma and their unique characteristics, and to consider sclerosing WDL/ALT in cases of fibrotic soft tissue tumors.

An overview on liposarcoma subtypes: Genetic alterations and recent advances in therapeutic strategies.

Liposarcoma (LPS) is a rare malignancy of adipocytic differentiation. According to World Health Organization classification, LPS comprises of four principle subtypes Atypical lipomatous tumor/Well-differentiated liposarcoma (ATL/WDLPS), Dedifferentiated liposarcoma (WDLPS), Myxoid liposarcoma (MLPS), and Pleomorphic liposarcoma (PLPS). Each subtype can develop at any location and shows distinct clinical behavior and treatment sensitivity. ATL/ WDLPS subtype has a higher incidence rate, low recurrence, and is insensitive to radiation and chemotherapy. DDLPS is the focal progression of WDLPS, which is aggressive and highly metastasizing. MLPS is sensitive to radiation and chemotherapy, with a higher recurrence rate and metastasis. PLPS subtype is highly metastasizing, has a poor prognosis, and exhibiting higher recurrence rate. Initial histological analysis provides information for the characterization of LPS subtypes', further molecular and genetic analysis provides certain subtype specifications, such as gene amplifications and gene fusions. Such molecular genetic alterations will be useful as therapeutic targets in various cancers, including the LPS subtypes. A wide range of novel therapeutic agents based on genetic alterations that aim to target LPS subtypes specifically are under investigation. This review summarizes the LPS subtype classification, their molecular genetic characteristics, and the implications of genetic alterations in therapeutics.

Outcome of patients with liposarcoma: A retrospective review over 12 ​Years in a single center

BackgroundLiposarcoma is the most common soft tissue sarcoma, which comprises around 20% of soft tissue sarcoma. While diverse therapeutic approaches exist for liposarcoma, the primary goal remains the achievement of effective local disease control. Wide resection of liposarcoma is the cornerstone of treatment. Radiotherapy is often employed as an adjuvant measure to enhance the local disease control. At Hospital Kuala Lumpur, the treatment protocol includes R0 resection and followed by adjuvant radiotherapy. This study aims to review the demographic characteristic of liposarcoma patients and evaluate the efficacy of their treatment based on the treatment protocol. Method and materialThis is a retrospective cohort study. A total of 72 patients (male/female ​= ​43/29) were included in this study. Recorded parameters included the histologic subtype, tumor location, treatment modality, resection margin, radiotherapy status, and occurrences of local recurrence and metastasis. ResultHistopathological examination revealed a varied spectrum of liposarcoma subtypes, with the predominant subtype being atypical lipomatous tumor (n ​= ​35), followed by myxoid liposarcoma (n ​= ​32), two cases of dedifferentiated liposarcoma, and three cases of pleomorphic liposarcoma. Of these patients, 69 underwent resection, while three necessitated amputations. Total 62 achieving negative and 10 cases had R1 margins. Adjuvant radiotherapy was administered in 58 cases. Overall, the local recurrence was observed in only six cases. ConclusionThis study emphasizes that achieving R0 resection is the fundamental approach in the treatment of liposarcoma and is able to minimize the risk of local recurrence.

Best Overall Response-Associated Signature to Doxorubicin in Soft Tissue Sarcomas: A Transcriptomic Analysis from ANNOUNCE.

This exploratory analysis evaluated the tumor samples of the patients treated with doxorubicin (with or without olaratumab) in a negative phase III ANNOUNCE trial to better understand the complexity of advanced soft tissue sarcomas (STS) and to potentially identify its predictive markers. RNA sequencing was performed on pretreatment tumor samples (n = 273) from the ANNOUNCE trial to evaluate response patterns and identify potential predictive treatment markers for doxorubicin. A BOR-associated signature to doxorubicin (REDSARC) was created by evaluating tumors with radiographic response versus progression. An external cohort of doxorubicin-treated patients from the Spanish Group for Research on Sarcomas (GEIS) was used for refinement and validation. A total of 259 samples from the trial were considered for analysis. Comparative analyses by the treatment arm did not explain the negative trial. However, there was an association between the BOR signature and histologic subtype (χ2P = 2.0e-7) and grade (P = 0.002). There were no associations between the BOR signature and gender, age, ethnicity, or stage. Applied to survival outcomes, REDSARC was also predictive for progression-free survival (PFS) and overall survival (OS). Using the GEIS cohort, a refined 25-gene signature was identified and applied to the ANNOUNCE cohort, where it was predictive of PFS and OS in leiomyosarcoma, liposarcoma, and other sarcoma subtypes, but not in undifferentiated pleomorphic sarcoma. The refined REDSARC signature provides a potential tool to direct the application of doxorubicin in sarcomas and other malignancies. Validation and further refinement of the signature in other potentially subtype specific prospective cohorts is recommended.

Adult genitourinary sarcoma: analysis using hospital-based cancer registry data in Japan

BackgroundGenitourinary sarcomas are rare in adults and few large-scale studies on adult genitourinary sarcoma are reported. We aimed to elucidate the clinical characteristics, survival outcomes, and prognostic factors for overall survival of adult genitourinary sarcoma in Japan.MethodsA hospital-based cancer registry data in Japan was used to identify and enroll patients diagnosed with genitourinary sarcoma in 2013. The datasets were registered from 121 institutions.ResultsA total of 116 men and 39 women were included, with a median age of 66 years. The most common primary site was the kidney in 47 patients, followed by the paratestis in 36 patients. The most common histological type was liposarcoma in 54 patients, followed by leiomyosarcoma in 25 patients. The 5-year overall survival rates were 57.6%. On univariate analysis, male gender, paratestis as primary organ, and histological subtype of liposarcoma were predictive of favorable survival while primary kidney, bladder, or prostate gland location were predictive of unfavorable survival. On multivariate analysis, primary paratestis was an independent predictor of favorable survival while primary kidney, bladder, or prostate gland were independent predictors of unfavorable survival.ConclusionsThis is the first report showing the clinical characteristics and survival outcomes of adult genitourinary sarcoma in Japan using a real-world large cohort database.

Genetic, Epigenetic and Transcriptome Alterations in Liposarcoma for Target Therapy Selection.

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.

An Uncommon Entity: Myxoid Liposarcoma Occurring in the Male Breast

Abstract Sarcoma of the breast represents a rare entity accounting for <1% of all breast tumors. Liposarcoma (LPS) of the breast is an exceedingly rare malignancy with an incidence of 0.3% of all breast sarcomas and myxoid LPS (MLPS), the second most common subtype of LPS, is even rarer in breast tissue. We present a notable case of MLPS affecting the breast of a 35-year-old male patient. The patient presented with a painless swelling in the left breast, which was subsequently diagnosed with MLPS through histopathological analysis following wide local excision. The tumor exhibited low mitotic count, clear resected margins, and positivity for S-100 on immunohistochemistry. Postoperative positron emission computerized tomography revealed no residual disease, and the patient received postoperative radiation therapy to minimize the risk of local recurrence. MLPS management requires a tailored approach, considering tumor size, location, and histological characteristics. Achieving negative margins through surgical resection remains crucial, with standardized definitions aiding in treatment consistency. The present case highlights the challenges in managing rare breast sarcomas and emphasizes the importance of a multidisciplinary approach to optimize patient outcomes.

Pleomorphic liposarcoma of the extremity with solitary huge liver metastasis at initial diagnosis treated with conversion surgery combined with adjuvant chemotherapy: a case report

BackgroundPleomorphic liposarcoma is the rarest subtype of liposarcoma. Pleomorphic liposarcomas are generally unresponsive to chemotherapy and radiotherapy. Moreover, metastasis in the liver, as the first and sole site, from a primary extremity soft tissue sarcoma, including pleomorphic liposarcoma, is extremely rare. Information regarding the appropriate management of these lesions is limited.Case presentationA 50-year-old Japanese woman presented with a mass in the left thigh. Imaging examination revealed a soft tissue sarcoma on the left posterior thigh. The tumor was histologically diagnosed as pleomorphic liposarcoma. Computed tomography examination for assessment of metastases incidentally detected a huge liver mass. Wide excision of sarcoma was performed prior to chemotherapy. Right trisectionectomy was necessary to achieve hepatic clearance; however, the future liver remnant volume was insufficient. Therefore, we decided to administer anthracycline-based chemotheraphy to shrink the tumor. After seven courses of adriamycin-based chemotherapy, the liver tumor size was reduced from 211 mm × 106 mm × 180 mm to 105 mm × 66 mm × 90 mm. Finally, a right hemihepatectomy was performed. The patient was continuously monitored and was metastasis or local recurrence free within 5 months after liver surgery.ConclusionChemotherapy is effective in some cases for the treatment of unresectable liver metastases of pleomorphic liposarcoma, and complete resection is possible with conversion surgery. If the patient’s general condition permits, anthracycline-based chemotherapy can be used for the treatment of stage 4 pleomorphic liposarcoma.

Giant myxoid liposarcoma of the gluteal region: Resection and buttock reconstruction

Introduction: Soft tissue sarcoma in adult is a rare disease and it needs an interdisciplinary team management. Liposarcoma has mesenchymal origin and arises in the fatty tissue. Multiple histologic subtypes of liposarcoma are recognized, including myxoid liposarcoma. In two-third of the cases, this tumor occurs in the muscle while often demonstrating a misleading benign appearance. Myxoid liposarcoma occurs predominantly in the deep soft tissues of the extremities and has the ability of metastasis to a wide range of soft tissue. Surgery is the main therapeutic modality in treatment of soft tissue sarcoma. Case Report: Presenting an exceptional case of a 50-year-old woman with history of painless large swelling of size 28×21×13 cm in left gluteal region since one year with rapid progression over three months. It was diagnosed as low grade myxoid liposarcoma of the left buttock. This is the largest reported case of myxoid liposarcoma of extremities in Asia found in literature. The patient was treated with complete excision of tumor with gluteus maximum muscle. Gluteus minimus flap was elevated to cover the exposed sciatic nerve and buttock recontouring was done as single procedure for aesthetically favorable results followed by post-operative radiotherapy and necessary follow-ups. Conclusion: Buttock contouring done post-resection as single procedure is more acceptable by the patient and has got superior aesthetic results.

Discoidin Domain Receptor Tyrosine Kinase 1 (DDR1) Is a Novel Therapeutic Target in Liposarcoma: A Tissue Microarray Study.

Liposarcoma is the most commonly diagnosed subtype of soft tissue sarcoma. As these tumors often arise near vital organs and neurovascular structures, complete resection can be challenging; consequently, recurrence rates are high. Additionally, available chemotherapeutic agents have shown limited benefit and substantial toxicities. There is, therefore, a clear and unmet need for novel therapeutics for liposarcoma. Discoidin domain receptor tyrosine kinase 1 (DDR1) is involved in adhesion, proliferation, differentiation, migration, and metastasis in several cancers. However, the expression and clinical importance of DDR1 in liposarcoma are unknown. The purposes of this study were to assess (1) the expression, (2) the association between DDR1 and survival, and (3) the functional roles of DDR1 in liposarcoma. The correlation between DDR1 expression in tumor tissues and clinicopathological features and survival was assessed via immunohistochemical staining of a liposarcoma tissue microarray. It contained 53 samples from 42 patients with liposarcoma and 11 patients with lipoma. The association between DDR1 and survival in liposarcoma was analyzed by Kaplan-Meier plots and log-rank tests. The DDR1 knockout liposarcoma cell lines were generated by CRISPR-Cas9 technology. The DDR1-specific and highly selective DDR1 inhibitor 7RH was applied to determine the impact of DDR1 expression on liposarcoma cell growth and proliferation. In addition, the effect of DDR1 inhibition on liposarcoma growth was further accessed in a three-dimensional cell culture model to mimic DDR1 effects in vivo. The results demonstrate elevated expression of DDR1 in all liposarcoma subtypes relative to benign lipomas. Specifically, high DDR1 expression was seen in 55% (23 of 42) of liposarcomas and no benign lipomas. However, DDR1 expression was not found to be associated with poor survival in patients with liposarcoma. DDR1 knockout or treatment of 7RH showed decreased liposarcoma cell growth and proliferation. DDR1 is aberrantly expressed in liposarcoma, and it contributes to several markers of oncogenesis in these tumors. This work supports DDR1 as a promising therapeutic target in liposarcoma.

Leiomyosarcoma and liposarcoma in young patients: The national netsarc+ network experience

BackgroundLeiomyosarcoma (LMS) and liposarcoma (LPS) are ultra-rare sarcomas in pediatric (0–18 years) and young adult (19−30) populations. We aimed to analyze their clinical characteristics at these young ages and to determine whether they should be considered with the same therapeutic strategy in both populations. MethodsNational retrospective multicenter study of all young patients (0–30 years) included in the sarcoma database “ConticaBase”, treated for LMS or LPS between 2010 and 2019 via the national NETSARC+ network, with available pathology/biology review. FindingsA total of 218 patients were identified, 34 children (nine LMS, 25 LPS) and 184 young adults (58 LMS, 126 LPS). Myxoid/Round Cell LPS (M/RC-LPS) was the most frequent LPS subtype (72 %). All children had localized LMS and LPS, versus 52/58 and 116/126 respectively in adults. Clinical presentation of LMS and all LPS subtypes was comparable in both populations, except for a preferential limb location of LMS in children. The therapeutic strategy was mainly based on primary surgery in LMS (9/9 children, 52/58 adults) and for LPS (respectively 25/25 and 122/126), exclusively or with adjuvant radiotherapy and systemic treatment. With a median follow-up of 62.4 months (range, 2.5–146), 5-year overall survival was respectively 83 % [95 % CI, 58–100] in children and 73 % [61–88] in young adults for LMS, 100 % [100] vs 92 % [87–99] for M/RC-LPS and 25 % [5–100] vs 60 % [29–100] for pleomorphic LPS. InterpretationLMS and all LPS subtypes appear to display comparable behavior in children and young adults. The authors propose that the same therapeutic strategy should be considered for both groups.

Real-world experience with liposomal doxorubicin in metastatic and locally advanced sarcoma.

e18795 Background: Sarcomas are a type of cancer that have been historically difficult to treat given their rarity, numerous subtypes, and variable response to treatment. Doxorubicin is the most used first-line chemotherapy in metastatic sarcoma; pegylated liposomal doxorubicin (PLD) has been explored as a potentially better tolerated alternative to free doxorubicin, with less myelosuppression, alopecia, and potentially less cardiotoxicity. In this work, we examine the efficacy and real-world challenges of using PLD as treatment for metastatic and locally advanced sarcoma. Methods: Medical records of metastatic and locally advanced sarcoma patients treated with single agent PDL as a therapy were obtained from a single tertiary medical center. Retrospective analysis was conducted to measure overall survival, progression free survival, best overall RECIST response, dosage, and complications. Results: 58 subjects were identified with 52% being male and 48% female. Sarcoma subtypes included liposarcoma (28%), leiomyosarcoma (21%), undifferentiated sarcoma (16%), synovial sarcoma (5%), and other subtypes (30%). Mean age at time of initiating therapy was 64 years. 22% of patients had previously been treated with doxorubicin, in the neo-adjuvant or adjuvant setting. Patients received an average of 5 cycles of PLD. Starting dosage was 50 mg/m² every 28 days, however, changes to lower the starting dose were made in 38% of cases to adjust for anticipated tolerance, and in 21% subsequently due to side effects such as cutaneous toxicity and fatigue. There were no complete responses, 12% partial response, 31% stable disease, and 57% progressive disease. Mean overall survival and progression free survival was 452 and 180 days, respectively. Sixty-seven percent of participants had at least one follow-up echocardiogram, and 15% of those had some evidence of cardiotoxicity, with an ejection fraction of < 50% or 10% decrease from baseline. Of the participants with changes in ejection fraction, 67% had received prior anthracycline therapy and 33% had received a total lifetime dose of doxorubicin exceeding the recommended 500 mg/m² limit. Conclusions: PLD is an option for first-line therapy for metastatic sarcoma in general, however, its efficacy remains limited in a real-world setting with most patients showing a best RECIST response of progressive disease and a mean progression free survival of 180 days. While PLD is generally well-tolerated, there are potential toxicities that require attention, including cutaneous toxicity, such as hand-foot syndrome, and infusion reactions. Furthermore, a significant portion of our sample had echocardiographic changes, with decreased ejection fraction, pointing out that, while PLD may be less cardiotoxic than free doxorubicin, there is potential risk. This highlights the need to continue the search for novel therapeutic strategies in metastatic and locally advanced sarcoma.

Epidemiology, survival, and treatment of liposarcoma subtypes: An analysis of the national cancer database.

e23535 Background: Liposarcomas are a soft tissue sarcoma which grow in adipose tissue, mainly in the subcutaneous fat of the limbs, and are the most common type of soft tissue sarcoma. Relatively little is known about the epidemiology, prevalence, and survival of various histological subtypes of liposarcoma, therefore, in the largest study to date, we analyzed this patient population using the NCDB. Methods: The National Cancer Database (NCDB) was used to identify patients diagnosed with Liposarcoma from 2004 to 2019 using the histology codes 8850, 8851, 8852, 8853, 8855, and 8858 as assigned by the Commission on Cancer Accreditation program. Kaplan-Meier, Chi-Square, and Cox Proportional Hazards tests were performed. Data was analyzed using SPSS version 27 and statistical significance was set at α = 0.05. Results: We identified 19,351 patients with liposarcoma. Mean age at diagnosis was 59 years for all subtypes and 83.3% of patients were white. Well-differentiated tumors were the most common histological subtype (34.8%), conferred the longest overall survival, and were the largest tumors on average. Round-cell tumors were the rarest (2.4%) and had smaller average tumor size than other subtypes. Patients with the de-differentiated subtype (20.5%) experienced the lowest median survival (70.5 months; p < 0.05) and an increased hazard ratio (HR = 2.8; p < 0.05) after controlling for other subtypes, age, and stage at diagnosis. Surgery was the mainstay of therapy with 90% of patients receiving surgery. 11.2% received additional adjuvant chemotherapy and 2.8% received adjuvant radiotherapy. Round cell and Myxoid tumors were most amenable to resection (89.8% and 88.9% of patients having no residual tumor) compared to only 64.4% of dedifferentiated tumors with complete surgical resection. Conclusions: Liposarcomas are a heterogeneous group of tumors which carry unique patterns of presentation, treatment, and prognosis. Well differentiated tumors are common and amenable to treatment; de-differentiated tumors are more aggressive and remain difficult to fully treat.

Malignant adipocytic tumours: A 20‑year single‑centre retrospective study.

Adipocytic tumours are the most common soft tissue neoplasms. Among them, liposarcoma is the most frequent malignant neoplasm. However, to the best of our knowledge, no previously published study has assessed the evolution and oncological prognosis of the different subtypes of liposarcoma at the retroperitoneal level compared with at other locations. The present study is a retrospective observational study in which all patients were operated on between October 2000 and January 2020 with a histological diagnosis of liposarcoma. Variables, such as age, sex, location, histological type, recurrence, type of treatment and mortality, among others, were analysed. The patients were divided into two groups: Group A (retroperitoneal location) and group B (non-retroperitoneal location). A total of 52 patients with a diagnosis of liposarcoma (17 women and 35 men) and a mean age of 57.2±15.9 years were assessed. A total of 16 patients were classified into group A and 36 into group B. The OR of recurrence was 1.5 (P=0.02) for R1 vs. R0 resection in group A. The OR of recurrence in group B for R1 vs. R0 resection was 1.8 (P=0.77), whereas for R2 vs. R0 resection, the OR was 69 (P=0.011). In conclusion, 52 cases of malignant adipocytic tumours collected during 2000-2020 were analysed with the new World Health Organization classification (updated 2020). Although its recurrence potential and capacity for distant metastasis depended on each histological type, surgical treatment with unaffected margins was the main prognostic factor for survival. The present study identified differences in relation to the survival of each histological subtype and its location, finding greater survival in dedifferentiated liposarcoma, myxoid liposarcoma and pleomorphic liposarcoma located at the extraperitoneal level than in the retroperitoneal location. Resectability was not influenced by liposarcoma location.

Primary pulmonary pleomorphic liposarcoma found with a massive hemothorax

BackgroundPleomorphic liposarcoma (PLPS) is a rare and aggressive subtype of liposarcoma. Here we report the case of a 45-year-old man with PLPS in the lung, which was accompanied by a massive hemothorax.Case presentationThe patient presented to hospital with chest pain. A chest x-ray showed massive right pleural effusion. We drained the right chest cavity of 1 L of blood and enhanced computed tomography (CT) revealed a massive right hemothorax and a 78 × 70 mm mass in the right lower lung lobe. The tumor was ruptured and bleeding, so we performed right lower lobe resection. Pathological investigations revealed a PLPS. One month after surgery, positron emission tomography-CT showed considerable pleural dissemination in the right lung field. The patient died 3 months after surgery.ConclusionsPLPS of the lung is rare, but requires attention because it can cause tumor rupture and hemothorax due to rapid tumor growth, and which may make complete resection of tumor difficult or necessitate emergency surgery.

The usefulness of immunohistochemistry for phosphohistone H3 as a prognostic factor in myxoid liposarcoma

Myxoid liposarcoma (MLS) is a common subtype of liposarcoma. Although the prognosis is generally good, there are factors known to be associated with poor prognosis. Accurate indices are important to predict prognosis. We aimed to assess the usefulness of immunohistochemistry for phosphohistone H3 (PHH3) as a potential biomarker in comparison with Ki-67 antigen and other prognostic factors. Twenty-five patients with MLS were evaluated. Age, sex, depth of tumor, tumor size, surgical margin, oncological outcome, histological grade, presence of necrosis, proportion of round cell component (RC%), PHH3 index, and Ki-67 index were examined. Prognostic factors of the examination criteria were statistically analyzed, survival rate analyses were performed using the Kaplan–Meier method, and multivariate analysis was performed using Cox proportional-hazard regression analysis. The number of PHH3-positive tumor cells and the PHH3 and Ki-67 indices demonstrated a statistical correlation with the prognosis of MLS in univariate analysis (P < 0.001, P < 0.001, P = 0.01, respectively). PHH3 index and RC% were significant factors in multivariate analysis (P = 0.03, P = 0.02). The immunohistochemistry of PHH3 may be associated with prognosis and could serve as a valid criterion of histological grade in MLS.