Transmediastinal primary pulmonary liposarcoma: Case report and review of management strategies.

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.

TRAbectedin in adVanced rEtroperitoneal well differentiated/dedifferentiated Liposarcoma and Leiomyosarcoma (TRAVELL): results of a phase II study from the Italian Sarcoma Group.

BACKGROUND: This is a multicentre, single-arm, phase II study aimed at further exploring the activity of trabectedin as second-/further-line treatment in retroperitoneal leiomyosarcoma (LMS) and well-differentiated/dedifferentiated liposarcoma (LPS). MATERIALS AND METHODS: The primary endpoint was the growth modulation index (GMI) defined as the ratio between PFS under trabectedin (PFS) and during previous chemotherapy treatment: time to progression (TTP-1). Secondary endpoints were objective response rate (ORR) and PFS. As per protocol, patients were considered responders if the GMI was >1.33, non-responders if <0.75 and neither if 0.76-1.32. RESULTS: Overall 91 patients were assessable for the primary endpoint (32 patients with LMS and 59 patients with LPS): the median number of cycles received was 6.0 (Q1-Q3 3.0-12.0), and the main reason for treatment discontinuation was disease progression in 72% of patients. The median PFS was 6.0 months, while the median TTP1 was 7.5 months (8.1 and 6.4 months for LMS and LPS, respectively). Thirty-three patients [52%, 95% confidence interval (CI) 36% to 58%, P = 0.674, odds of response 1.1] had a GMI >1.33 (LMS 46%, 95% CI 26% to 67%, odds of response 0.85; LPS 56%, 95% CI 40% to 72%, odds of response 1.3). Overall, in LPS we observed 15/47 patients with a GMI <0.5 and 15/47 patients with a GMI >2. Among LMS patients, 9/26 had a GMI <0.5 and 10/26 had a GMI >2. Overall, ORR (complete response + partial response) was 16% (24% for LMS and 12% for LPS). CONCLUSIONS: While the primary endpoint of the study was not met, we noticed a subgroup of patients with a markedly discrepant TTP with trabectedin in comparison to previous therapy (GMI <0.5 or >2, the latter including some patients with a long TTP with trabectedin). A mismatch between PFS and overall survival was observed, possibly due to the natural history of the two different histologies and the availability of further lines in LMS.

Exploring the biological behavior differences between retroperitoneal and non-retroperitoneal liposarcomas.

Background: Liposarcoma is a malignant tumor that originates from adipose tissue and can occur in any part of the body. There is currently no clear conclusion on whether there are significant differences in prognosis between liposarcoma at different anatomical locations, especially retroperitoneal liposarcoma (RLPS) and non retroperitoneal liposarcoma (NRLPS). The aim of this study is to reveal whether there are differences in prognosis between these two locations of liposarcoma, and further explore the fundamental reasons behind these differences. Methods: We conducted an in-depth investigation into the factors affecting the prognosis of patients with liposarcoma by analyzing the data from the Surveillance, Epidemiology, and End Results Program (SEER) database. Then, we used propensity score matching (PSM) to balance these prognostic factors for comparative analysis of survival between RLPS and NRLPS. In addition, by analyzing transcriptome and whole exome data from TCGA and the Japan Genotypic Phenotype Archive (JGA), we identified genes with significant expression differences and explored changes in the immune microenvironment. Result: Through analysis of RLPS and NRLPS patients in the SEER database, we observed significant prognostic differences between the two groups, with RLPS exhibiting worse prognosis (p < 0.001). Even after adjusting for confounding factors through PSM, these survival rate differences remained significant, with RLPS still showing worse prognosis (p = 0.017). Furthermore, our analysis of transcriptomic data led to the identification of 467 differentially expressed genes. Additionally, we noted significant differences in the immune microenvironment and whole exome sequencing data between the two groups. Conclusion: There are significant differences between patients with RLPS and NRLPS. Therefore, from clinical research to treatment strategies, RLPS and NRLPS should be considered as two distinct types of tumors, necessitating differentiated approaches for their study and treatment.

Prognostic Nutritional Index (PNI): A More Promising Nutritional Predictor for Patients Undergoing Surgery for Retroperitoneal Liposarcoma.

Background: Extended surgery with multi-visceral resection is the standard treatment for retroperitoneal liposarcoma (RLPS). Malnutrition tends to result in increased surgical complications and reduced survival. The aim of this study was to identify the prognostic role of nutritional status in patients with RLPS. Patients and methods: Data from 189 consecutive patients with RLPS who underwent surgical treatment at the Peking University Cancer Hospital Sarcoma Center between April 2011 and August 2022 were retrospectively reviewed. The following nutritional parameters were calculated: nutritional risk index, prognostic nutritional index (PNI) and Nutrition Risk Screening 2002. Time-dependent receiver operating characteristic (time-ROC) curve analysis was conducted to compare the prognostic utility of nutritional indicators. The associations between nutritional indicators and major complications, local recurrence-free survival (LRFS) and overall survival (OS) were investigated. Results: Based on the time-ROC curve analysis, the PNI was superior to other nutritional indices at predicting OS. The optimal cut-off value of PNI was 41.2. The PNI was significantly inversely associated with tumor size, tumor grade, and histological subtype. Patients in the low PNI group (< 41.2) had significantly shorter LRFS and OS than those in the high PNI (≥ 41.2) group, with higher major morbidity and mortality rates. The PNI was found to be a unique nutritional predictor that independently predicted LRFS and OS in the multivariate analysis. Conclusion: The PNI is an effective tool for nutritional assessment in patients with RLPS. A low PNI value in patients with RLPS predicts worse survival outcomes.

Well-differentiated liposarcoma of spermatic cord presenting as recurrent inguinal hernia: a case report.

Background: Well-differentiated liposarcoma arising from the paratesticular region is rare, with only a few hundred cases reported in the literature. Due to their unexpected location, these tumors are often confused for common pathologies found in the groin, including inguinal hernia, seroma, or lymphoma. Standardized diagnostic and treatment pathways have not been established for patients with paratesticular liposarcoma, thereby elevating the importance of sharing our experience. Case Description: This case describes the presentation of a 65-year-old man with a well-differentiated liposarcoma of the spermatic cord. Diagnosis was made after he underwent open herniorrhaphy to repair what was presumed to be a recurrent left inguinal hernia. Although a recommendation for formal oncologic resection and orchiectomy was made, the patient elected to proceed with watchful waiting and remains well up to last known contact. Conclusions: Paratesticular liposarcoma remains a rare clinical entity. While a few hundred cases have been reported in the literature, only a handful describe its presentation masked as an early recurrence of a groin hernia. Wide local resection along with orchiectomy and potential radiation therapy have been the mainstay of treatment. Clinicians should maintain a healthy level of suspicion for this uncommon pathology, especially in cases where patients present with early recurrence of an inguinal hernia.

Lipomatous pseudohypertrophy of the pancreas mimicking liposarcoma: A case report.

Lipomatous pseudohypertrophy of the pancreas (LPH) is a rare disease in which the pancreatic parenchyma is replaced with mature adipose tissue. It is an idiopathic condition whose diagnosis is made based on histopathological analyses. Herein, we report the case of a 50-year-old male patient with a lipomatous mass in the head of the pancreas on computed tomography for close examination of a renal tumor. We suspected liposarcoma, and laparotomy was performed. However, histological analyses revealed LPH. Several imaging findings of LPH can enable a noninvasive diagnosis and help its clinical approach.

Massive, Retroperitoneal, Well-Differentiated Liposarcoma in Second-Trimester Pregnancy: A Report of a Rare Case.

Retroperitoneal liposarcoma during pregnancy is rare and poses significant diagnostic challenges, even for experienced specialists. We present a case report of a 27-year-old female patient, 15 weeks pregnant, who was admitted to the hospital due to a massive retroperitoneal liposarcoma. The patient underwent surgical resection of the tumor. Postoperative pathology confirmed a diagnosis of well-differentiated liposarcoma. Although liposarcoma during pregnancy is rare and challenging to diagnose, CT or MRI plays a crucial role in its detection. The recurrence rate depends on the pathological stage, histological grade, and ability to resect the tumor.

Dedifferentiated Liposarcoma of the Rectum: A Case Report.

Liposarcoma is a malignant soft tissue tumor that rarely involves the gastrointestinal tract. The dedifferentiated type typically carries the worst prognosis. Here, we describe a rare case of dedifferentiated liposarcoma of the rectum in a young male patient who was treated with surgical excision. Treatment is often difficult and there is no clear consensus on the benefits of radiotherapy or chemotherapy.

Spermatic cord dedifferentiated liposarcoma: Review of literature and case report.

Spermatic cord malignancies are a scarce modality and liposarcoma of spermatic cord is even a rarer condition encountered. Liposarcoma is usually a slowly progressive, non-tender, well circumscribed mass of variable shapes owing to conformity to fascial compartments. We are reporting a case of 65-year-old male, with a two-month history of initially tender and later non-tender mass in the scrotum, above the testis. Ultrasonography showed a right mid inguinal mass measuring 6x3x3 cm at the superior pole of the right testis and small fluid around the right testis. Fine needle aspiration cytology (FNAC) of the right inguinal mass revealed a spindle cell neoplasm. The patient underwent right inguinal radical orchiectomy with local wide excision of the sarcoma of the spermatic cord origin. Final histopathology confirmed dedifferentiated liposarcoma. No adjuvant treatment was offered and the patient was put on surveillance. Follow-up of more than 10 months has not revealed any local recurrence, regional or non-regional lymph nodes, or systemic metastasis.

A Case of Dedifferentiated Liposarcoma That Contributes to Accompanying Vessels of Various Size.

Dedifferentiated liposarcoma (DDLPS) is a non-lipogenic sarcoma, generally arising from well-differentiated liposarcoma (WDLPS), although it can develop de novo. DDLPS tumors rarely trans-differentiate into non-adipose mesenchymal tissues; however, the latter lack notable variety and mostly show striated muscle or osteogenic/chondrogenic differentiation. Here, we report a case of DDLPS that contained numerous atypical vessels. A man in his sixties presented with a large tumor in his right thigh, and the tumor was surgically resected. Microscopically, most of the tumor was WDLPS, but a minor portion showed DDLPS, consisting of high-grade spindle cells. Remarkably, the DDLPS contained vessels of various sizes with atypical cytoarchitecture, including vessels with seemingly muscular layers. Immunohistochemically, the atypical cells within the vascular wall expressed aSMA, consistent with smooth muscle cells or pericytes, whereas surrounding high-grade spindle cells only focally expressed it, and these aSMA-positive cells within the vessels exhibited MDM2 amplification by immuno-fluorescence in situ hybridization. Our results demonstrate that DDLPS can trans-differentiate into smooth muscle cells of various-sized accompanying vessels, which may support their survival and proliferation.

Surgical outcomes of major vascular resection for retroperitoneal liposarcoma from a high­volume sarcoma center: a propensity score matching analysis.

PURPOSE: Radical resection of retroperitoneal liposarcoma (RLPS) may necessitate vascular resection and reconstruction. The study was conducted to assess surgical outcomes of surgery for RLPS with major vascular involvement. METHODS: Patients with RLPS who underwent surgical resection at the Sarcoma Center of Peking University Cancer Hospital between April 2011 and December 2022 were identified from a prospectively maintained database. Patients were classified into two groups: vascular resection and non-vascular resection groups. A propensity score matching analysis was performed to eliminate baseline differences between the groups. Surgical details and postoperative outcomes were analyzed. Furthermore, prognostic factors for local recurrence-free survival (LRFS) and overall survival (OS) were assessed. RESULTS: Overall, 199 patients were identified and the median follow-up period was 48 (interquartile range [IQR] 45-69) months. Vascular resection was performed in 42 (21%) patients, 25 of whom had vascular infiltration. A total of 39 patients had vascular replacement and 3 patients underwent partial resection (side-wall resection). Vascular resection was burdened by higher rates of major morbidity (38% vs. 14%, p < 0.001) and 30-day mortality (7.1% vs. 1.3%, p = 0.005). After propensity-matched analysis, patients who underwent vascular resection had 5-year LRFS and OS rates comparable to those without vascular involvement. Major vascular resection was not an independent risk factor for LRFS or OS. CONCLUSIONS: Although accompanied by increased risks of major morbidity and mortality, the major vascular resection enabled radical resection in patients with advanced RLPS, affording comparable 5-year LRFS and OS rates compared to those who did not.

Dedifferentiated Mediastinal Liposarcoma: A Case Report.

Liposarcoma is the most common soft tissue sarcoma type in adults, originating mainly from the retroperitoneum and lower extremities. Mediastinal liposarcomas constitute an extremely rare clinical entity of mesenchymal origin. Among subtypes, dedifferentiated liposarcoma is characterized by poor survival, but little is known about its biological behavior. We present the case of a 78-year-old male patient who presented with vague symptoms, predominantly dyspnea and chest pain. Imaging revealed a large mediastinal mass and surgical resection was performed in a piecemeal manner due to the inability to achieve a microscopically negative surgical margin (R0 resection) for the residual tumor. Histological examination confirmed the diagnosis of dedifferentiated liposarcoma. The patient's postoperative course was uneventful, with discharge from the hospital on the 10th postoperative day. However, local recurrence was detected after two months and the patient died four months after the operation. The present case report highlights the importance of radical excision for the prevention of local recurrence and the presentation of histological characteristics of this tumor. Radical surgical resection remains the fundamental treatment, while chemo and radiotherapy may have an adjuvant role. In cases of inability to obtain negative margins, surgical debulking can offer symptomatic relief.

Optimizing surgical strategies for retroperitoneal liposarcoma: a comprehensive evaluation of standardized aggressive surgical policies.

BACKGROUND: Retroperitoneal liposarcoma (RLPS) constitutes the majority of retroperitoneal sarcomas. While surgical resection remains the sole curative approach, determining the optimal surgical strategy for RLPS remains elusive. This study addresses the ongoing debate surrounding the optimal surgical strategy for RLPS. METHODS: We recruited 77 patients with RLPS who underwent aggressive surgical policies. Patients were categorized into three surgical subtypes: suprapancreatic RLPS, pancreatic RLPS, and subpancreatic RLPS. Our standardized surgical strategy involved resecting macroscopically uninvolved adjacent organs according to surgical subtypes. We collected clinical, pathological and prognostic data for analyses. RESULTS: The median follow-up was 45.5 months. Overall survival (OS) and recurrence-free survival (RFS) were significantly correlated with multifocal RLPS, pathological subtype, recurrent RLPS and histological grade (P for OS = 0.011, 0.004, 0.010, and < 0.001, P for RFS = 0.004, 0.001, < 0.001, and < 0.001, respectively). The 5-Year Estimate OS of well-differentiated liposarcoma (WDLPS), G1 RLPS, de novo RLPS and unifocal RLPS were 100%, 89.4%, 75.3% and 69.1%, respectively. The distant metastasis rate was 1.4%. The morbidity rates (≥ grade III) for suprapancreatic, pancreatic, and subpancreatic RLPS were 26.7%, 15.6%, and 13.3%, respectively. The perioperative mortality rate is 2.6%. CONCLUSIONS: Standardized aggressive surgical policies demonstrated prognostic benefits for RLPS, particularly for G1 RLPS, WDLPS, unifocal RLPS, and de novo RLPS. This approach effectively balanced considerations of adequate exposure, surgical safety, and thorough removal of all fat tissue. G1 RLPS, WDLPS, unifocal RLPS, and de novo RLPS could be potential indications for aggressive surgical policies.

Fluorescence in situ hybridization-negative intra-articular myxoid liposarcoma with complex rearrangements involving EWSR1::DDIT3 detected using nanopore sequencing.

Myxoid liposarcoma (MLPS) is a rare sarcoma, typically arising in deep soft tissues during the fourth to fifth decades of life. Histologically, MLPS is composed of uniform oval cells within a background of myxoid stroma and chicken-wire capillaries. Genetically, MLPS is characterized by the FUS/EWSR1::DDIT3 fusion gene, which generally results from balanced interchromosomal translocation and is detectable via DDIT3 break-apart fluorescence in situ hybridization (FISH). Here, we report an unusual intra-articular MLPS case, negative for DDIT3 break-apart FISH but positive for EWSR1::DDIT3. An 18-year-old female was referred to our hospital complaining of an intra-articular mass in the right knee joint. Histologically, the tumor was mainly composed of mature adipocytes, brown fat-like cells, and lipoblasts. Nanopore sequencing detected DNA rearrangements between EWSR1 and DDIT3 and clustered complex rearrangements involving multiple chromosomes, suggesting chromoplexy. Methylation classification using random forest, t-distributed stochastic neighbor embedding, and unsupervised hierarchical clustering correctly classified the tumor as MLPS. The copy number was almost flat. The TERT promoter C-124T was also detected. This report highlights, for the first time, the potential value of a fast and low-cost nanopore sequencer for diagnosing sarcomas.

Management of Liposarcoma With Posterior Intercostal Artery Propeller Flap Reconstruction: A Case Report.

This case shows the administration of a 57-year-old male with liposarcoma within the right flank region. Surgical treatment of the case included wide local excision (WLE), taken after reconstruction utilizing a posterior intercostal artery propeller flap. Postoperative care included regular checking for signs of repeat. Comparison with similar cases highlights the changeability in clinical introduction and surgical approaches for liposarcomas. This case emphasizes the significance of convenient diagnosis, fastidious surgical procedures, and successful reconstruction in overseeing liposarcomas. This case report points to highlights the clinical administration, surgical intercession, and postoperative care included in treating a giant liposarcoma and compares this case with similar instances to emphasize the challenges and procedures in treating liposarcomas.

Paratesticular Liposarcoma Presenting as an Inguinal Swelling: A Diagnostic Conundrum.

Paratesticular tumours are rare malignancies that are frequently misdiagnosed on presentation. We present a case of an elderly male with a six-month history of painless, progressively increasing left inguinal swelling. On preliminary examination and investigation, the swelling was misdiagnosed as a lymph nodal mass. Subsequently, a magnetic resonance imaging study detected a lesion that was not distinct from the spermatic cord. Biopsy testing of the said lesion was suggestive of poorly differentiated spindle cell neoplasm. The patient then underwent a high inguinal orchidectomy. Histopathological examination confirmed the diagnosis of a high-grade paratesticular dedifferentiated liposarcoma with rhabdomyoblastic differentiation. Due to the rarity of such tumours, the need for adjuvant chemotherapy and radiotherapy is debated.

A case of dedifferentiated liposarcoma discovered due to an intrascrotal calcified ossification.

Dedifferentiated liposarcoma is a rare cancer with a poor prognosis. A 52-year-old man presented with a chief complaint of a mass in his left scrotum. He came with suspected testicular tumor, but all the measured tumor markers were negative. Imaging test showed approximately 2 cm diameter mass accompanied by calcification with some substantial components between the testis and epididymis. Left high testicular resection was performed. The tumor had no continuity between the testis and epididymis, and the spermatic cord transection was negative. Pathological findings showed well differentiated fatty component and a dedifferentiated component around the trabecular bone-like tissue. We observed dedifferentiated dysmorphic cells mixed with fatty droplets of unequal size. Immunostaining led to the diagnosis of dedifferentiated liposarcoma. No additional postoperative therapy was performed. The possibility of dedifferentiated liposarcoma should be kept in mind even if mass is confined to the scrotum and consisted of calcification. In the case of an intrascrotal calcified mass with malignant perspective, radical surgery is highly recommended.

Mechanical property estimation of sarcoma-relevant extracellular vesicles using transmission electron microscopy.

Analysis of single extracellular vesicles (EVs) has the potential to yield valuable label-free information on their morphological structure, biomarkers and therapeutic targets, though such analysis is hindered by the lack of reliable and quantitative measurements of the mechanical properties of these compliant nanoscale particles. The technical challenge in mechanical property measurements arises from the existing tools and methods that offer limited throughput, and the reported elastic moduli range over several orders of magnitude. Here, we report on a flow-based method complemented by transmission electron microscopy (TEM) imaging to provide a high throughput, whole EV deformation analysis for estimating the mechanical properties of liposarcoma-derived EVs as a function of their size. Our study includes extracting morphological data of EVs from a large dataset of 432 TEM images, with images containing single to multiple EVs, and implementing the thin-shell deformation theory. We estimated the elastic modulus, E = 0.16 ± 0.02 MPa (mean±SE) for small EVs (sEVs; 30-150 nm) and E = 0.17 ± 0.03 MPa (mean±SE) for large EVs (lEVs; >150 nm). To our knowledge, this is the first report on the mechanical property estimation of LPS-derived EVs and has the potential to establish a relationship between EV size and EV mechanical properties.

A Case of a Giant Liposarcoma at Binh Dan Hospital.

Retroperitoneal liposarcomas (RLSs) are uncommon mesenchymal tumors that might present a diagnostic challenge due to their rarity and anatomical location. Despite grossly complete resections, they are commonly linked to a high recurrence rate, necessitating long-term or indefinite follow-up. This report discusses a 59-year-old male patient referred to the Gastrointestinal Department due to chronic abdominal distention, right-sided back pain, and a sizable abdominal mass. The diagnosis was an RLS, and the patient underwent en bloc resection of the mass.

Prevalence, Anatomical Distribution, and Risk Factors of Adipocytic Tumors and Xanthomas in Psittaciformes: 1096 Cases (1998-2018).

Adipocytic tumors are mesenchymal tumors that are commonly reported in psittacine birds; however, large-scale studies evaluating their prevalence and associated risk factors are lacking. A retrospective study of adipocytic tumors in psittacine birds was performed by reviewing pathology submissions from the University of California, Davis-Drury Reavill Pathology Database, containing 26 013 submissions from psittacine birds (1998-2018). Age, sex, genus, anatomic distribution, and pathological diagnosis were collected for each case when available. The prevalence, risk factors, and association with other lipid-accumulation disorders were reported. A total of 450 cases of lipoma, 129 cases of myelolipoma, 35 cases of hemangiolipoma, 31 cases of liposarcoma, and 451 cases of xanthoma were identified. The prevalence of adipocytic tumors and xanthomas on necropsy was 1.3% (158/11 737, 95% confidence interval [CI]: 1.1-1.6). Adipocytic tumors were identified in 27 genera. Amazona (odds ratio [OR] = 1.93, 95% CI: 1.24-2.99, p = 0.004), Myiopsitta (OR = 2.3, 95% CI: 1.0-5.2, p = 0.041), Melopsittacus (OR = 3.4, 95% CI: 2.1-5.5, p < 0.001), and Agapornis (OR = 3.5, 95% CI: 2.0-6.1, p < 0.001) had significantly higher odds of developing adipocytic tumors compared with other genera, whereas Ara had significantly lower odds (OR = 0.5, 95% CI: 0.3-0.9, p = 0.030). Age was also a significant risk factor for many types of adipocytic tumors. There was no significant association between general adipocytic tumor formation and atherosclerosis or hepatic lipidosis. Xanthomas were associated with atherosclerosis (OR = 1.88, 95% CI: 1.01-3.51, p = 0.048), but not hepatic lipidosis (p = 0.503). On necropsy, the trunk and air sacs were the most common sites of xanthoma formation, whereas the trunk and liver were the most common sites of lipoma and myelolipoma formation, respectively.