Mediastinal Myxoid Liposarcoma with Intrapericardial Involvement and Large Pericardial Effusion

2015 ◽  
Vol 18 (5) ◽  
pp. 192
Author(s):  
Santiago Adolfo Endara ◽  
Gerardo Augusto Davalos ◽  
Ana Lucia Vinueza ◽  
Nelson Montalvo ◽  
Patricia Graciela Duran ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Pericardial Effusion ◽  
English Language ◽  
Mediastinal Mass ◽  
Soft Tissue Sarcomas ◽  
Myxoid Liposarcoma ◽  
Large Pericardial Effusion ◽  
Adipocytic Differentiation ◽  
Mediastinal Liposarcoma ◽  
Uncommon Neoplasm

Liposarcoma is the name given to a group of soft tissue sarcomas (STSs) with adipocytic differentiation. As a group, liposarcomas are the second most common STSs in adults. In 1951 Kozonis et al published that in the English language only four cases of liposarcomas originating in the mediastinum had been described. Primary mediastinal liposarcoma is an uncommon neoplasm of intrathoracic origin. We present the case of a 47-year-old woman diagnosed with a large mediastinal mass with intrapericardial invasion and massive pericardial effusion; biopsies showed a mediastinal liposarcoma.

scholarly journals Carbon ion radiotherapy for recurrent calf myxoid liposarcoma: a case report

2021 ◽  
Vol 49 (4) ◽  
pp. 030006052110097
Author(s):  
Xiaojun Li ◽  
Yanshan Zhang ◽  
Yancheng Ye ◽  
Ying Qi ◽  
Chunlan Feng ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcomas ◽  
Myxoid Liposarcoma ◽  
Ion Beams ◽  
Carbon Ion Radiotherapy ◽  
Irradiation Damage ◽  
Radiation Dermatitis ◽  
Complete Disappearance ◽  
Carbon Ion ◽  
The Right

Liposarcoma (LPS) is the most common soft tissue sarcoma. Myxoid LPS (MLPS) is the second most common subtype of LPS and accounts for 25% to 50% of all LPSs. Like most other soft tissue sarcomas, the mainstay of treatment for LPS is inevitably surgery. Multidisciplinary approaches, including surgery, chemotherapy, and radiotherapy, have been successful in the treatment of LPS during the last three decades. Even so, recurrence of LPS remains challenging. Carbon ion beams produce increased energy deposition at the end of their range to form a Bragg peak while minimizing irradiation damage to surrounding tissues, which facilitates more precise dosage and localization than that achieved with photon beams. Furthermore, carbon ion beams have high relative biologic effectiveness. We herein describe a patient who developed recurrent MLPS in the right calf after two surgeries and underwent carbon ion radiotherapy (CIRT), achieving complete disappearance of the tumor. The patient developed Grade 1 radiation dermatitis 30 days after CIRT, but no other acute toxicities were observed. The tumor had completely disappeared by 120 days after CIRT, and the patient remained disease-free for 27 months after CIRT. The CARE guidelines were followed in the reporting of this case.

Rechallenge with trabectedin in patients with locally advanced or metastatic soft tissue sarcoma following drug holiday: The experience of the French Sarcoma Group (FSG).

2012 ◽  
Vol 30 (15_suppl) ◽  
pp. 10062-10062 ◽  
Author(s):  
Esma Saada ◽  
Chahineze Rahal ◽  
Isabelle Ray Coquard ◽  
Antoine Italiano ◽  
Christine Chevreau ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Objective Response ◽  
Locally Advanced ◽  
Soft Tissue Sarcomas ◽  
Myxoid Liposarcoma ◽  
Median Number ◽  
Drug Holiday ◽  
Who Grade ◽  
Well Differentiated ◽  
Number Of Cycles

10062 Background: Trabectedin (T) is a marine-derived alkaloid used to treat advanced soft tissue sarcomas (STS) after ifosfamide and/or anthracyclins failure. Since then, the FSG evaluated the clinical benefit in re-administrating T after an initial hold, either medically indicated or upon patient’s request. Methods: Following an online request, clinical and histopathological data were collected from six centers of the FSG who declared to have rechallenged patients. Baseline data were collected and analyze will be used. Results: From 1999 to 2011, 49 pts with T drug holiday have been identified (26 male/ 23 female), with a median age of 50 y [23-75]. Most frequent histotypes were: myxoid liposarcoma (18, 36.7%), leiomyosarcomas (13, 26.5 %) and well-differentiated/dedifferentiated liposarcoma (9, 18%). WHO grade were 1 in 14 (29%), 2 in 19 (39%) and 3 in 5 (10%) pts respectively. Patients who had a maximum of 2, 3 or 4 therapeutic sequences (TS) with T (drug-holiday and rechallenge) were 41/49 ,7/49 and 1/49 respectively. Median number of cycles for 1, 2, 3 and 4 TS were 7 [3-21], 6 [2-30], 6 [2-9] and 6. Median total number of cycles was 15 [6-43]. Median duration of drug-holiday for 1, 2 and 3 TS were 11 [3-91], 7 [2-29] and 4 months [1-5]. Grade 3-4 toxicities incidence decreased with the number of TS (occurred in 36%, 29%, 14% and 0% of pts with 1, 2, 3 and 4 TS) as well as mean T dose per cycle (1.3 mg/m², 1.2 mg/m², 1.1 mg/m² and 1.1 mg/m² for TS 1, 2, 3, 4). Efficacy decreased with number of TS (Number of CR/PR/SD/PD were 1 (2%)/15 (31%)/33 (67%)/0 for TS1; 0/4 (8%)/29 (59%)/16 (3%) for TS2; 0/1 (14%)/2 (29%)/4 (57%) for TS3 and 0/0/0/1(100%) for TS4). Median overall survival was 5.0 y [2.7-7.3] since T introduction, and 1.5 y [0.1-4.8], 0.8 y [0.5-1.3] and 0.6 y following 2nd, 3rd and 4th T reintroduction respectively. Objective response after TS2 were seen in 4 cases of grade 1 sarcomas. Conclusions: Due to the lack of cumulative toxicities over time with T, its rechallenging in responding patients to T (no progression under T) have to be considered in advanced STS.

Radiomic features for patients with primary soft tissue sarcomas: A prognostic study.

2020 ◽  
Vol 38 (15_suppl) ◽  
pp. e23532-e23532
Author(s):  
Sandro Pasquali ◽  
Marco Bologna ◽  
Gabriele Infante ◽  
Raffaella Vigorito ◽  
Antonella Messina ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Ct Scan ◽  
Soft Tissue Sarcomas ◽  
Disease Free Survival ◽  
Distant Metastases ◽  
Myxoid Liposarcoma ◽  
Added Value ◽  
Discriminative Ability ◽  
End Point

e23532 Background: Prognosis of extremity soft tissue sarcomas (ESTS) and retroperitoneal sarcomas (RPS) is currently estimated on clinical-pathological features, as those incorporated in the Sarculator nomogram. This study investigated the added value of end-point specific radiomic signatures (RS) in patients with primary ESTS and RPS. Methods: We retrospectively analysed data from patients with a ESTS (N = 100) or a RPS (N = 116) who underwent surgery (Jan2011-Dec2015) at one sarcoma reference institute and had magnetic resonance imaging (MRI) or computed tomography (CT) scan, respectively. Radiomic features were extracted from DICOM files of MRI and CT scan for ESTS and RPS, respectively. The study end-points were incidence of distant metastases (DM) and overall survival (OS) for ESTS and disease-free survival (DFS) and OS for RPS. A RS was established for each end-point. Models discriminative ability was measured using the Harrell c-index (C). Results: ESTS histology was undifferentiated pleomorphic sarcoma, myxofibrosarcoma, myxoid liposarcoma, dedifferentiated/pleomorphic liposarcoma, and others in 28, 21, 18, 9, and 24 patients, respectively. At a median follow-up of 61 months, the 5-yr DM-free rate and OS were 73.6% (95%CI 64.2-82.2%) and 73.4% (95%CI 64.5-83.6), respectively. A prognostic model based on the Sarculator and a DM-RS achieved higher C (0.86, 95%CI 0.77-0.93) than the Sarculator alone (0.83, 95%CI 0.71-0.94). For OS, the RS was not able to increase the Sarculator discriminative ability (Sarculator and OS-RS: C = 0.73, 95%CI 0.62-0.82; Sarculator alone: C = 0.72, 95%CI 0.62-0.81). RPS histology was well differentiated liposarcoma, dedifferentiated liposarcoma, leiomyosarcoma, and other in 22 (19%), 60 (52%), 22 (19%), and 12 (10%) patients, respectively. At a median follow-up of 58 months, 5-yr DFS and OS were 46.7% (95%CI, 37.8-57.6%) and 68% (95% CI 59.9-79.0%), respectively. RS extracted from CT scan without contrast enhancement slightly improve the discriminative ability of the Sarculator for DFS (Sarculator and DFS-RS: C = 0.65, 95%CI 0.57-0-72; Sarculator alone: C = 0.63, 95%CI 0.55-0.70) but not for OS (0.70, 95%CI 0.60-0.80 and 0.69, 95%CI 0.59-0.79, respectively). Conclusions: RS did not improve the discriminative ability of the Sarculator for OS. The added value for RS in relapse prediction (DM incidence for ESTS and DFS for RPS) deserves further investigations.

scholarly journals Efficacy and Safety of Trabectedin in Patients with Soft Tissue Sarcoma: A Bicentric Retrospective Analysis.

2021 ◽  
Author(s):  
Chaigneau Loïc ◽  
Jary Marine ◽  
Nerich Virginie ◽  
Hervieu Alice ◽  
Aubry Sébastien ◽  
...  
Keyword(s):  
Overall Survival ◽  
Soft Tissue ◽  
Soft Tissue Sarcomas ◽  
Progression Free Survival ◽  
Myxoid Liposarcoma ◽  
Free Survival ◽  
Median Progression Free Survival ◽  
Heterogenous Group ◽  
End Of Treatment ◽  
Low Toxicity

Abstract Background: Soft tissue sarcomas (STS) are a rare and heterogenous group of tumors, with poor prognostic, judging from their frequency to relapse. Few drugs are available. Since 2007, trabectedin got approval after failure of anthracyclines and ifosfamide, for advanced or metastatic STS.Patients and Methods: This retrospective study describes effects of trabectedin on survival, response, and toxicity, in STS patients. One hundred twenty-nine patients treated between 2002 and 2019 were analysed, from two French centers. All patients were tested for toxicities, and efficacy was assessed in patients exposed to at least 2 cycles of trabectedin.Results: Three median cycles were administered per patient (1-79). Among the 115 patients analysed for efficacy, the median progression free survival was 3.0 months [CI95%: 2.3 – 4.7], with an overall survival of 11.9 months [CI95%: 10.2 – 16.6]. The rate of disease control was 45.2% at the end of treatment. Myxoid liposarcoma (n = 11) was the histology subtype that benefited most from this chemotherapy with median progression free survival and an overall survival of 13.3 months [CI95%: 2.3 – 18.7] and 27.8 months [CI95%: 3.2 – 64.7], respectively. Adverse events were manageable. Conclusion: Efficacy of trabectedin is confirmed in terms of clinical benefit and low toxicity, especially for myxoid liposarcoma.

The Past, Present, and Future of Cytotoxic Chemotherapy and Pathway-Directed Targeted Agents for Soft Tissue Sarcoma

2013 ◽  
pp. e386-e393 ◽  
Author(s):  
Christopher W. Ryan ◽  
Jayesh Desai
Keyword(s):  
Soft Tissue ◽  
Systemic Therapy ◽  
Soft Tissue Sarcomas ◽  
Progression Free Survival ◽  
Myxoid Liposarcoma ◽  
The United States ◽  
Cytotoxic Agents ◽  
Phase Iii ◽  
Current Phase ◽  
Anaplastic Lymphoma

The individual rarity of the many subtypes of soft tissue sarcomas has historically mandated an empiric approach to systemic therapy. Doxorubicin, first reported to have activity in sarcomas 40 years ago, remains the generalizable first-line treatment of choice for many subtypes, with no other drug or combination having shown an overall-survival advantage. Other cytotoxic agents, such as paclitaxel for angiosarcoma or gemcitabine with docetaxel for leiomyosarcoma, are commonly used for certain histologic subtypes based on relatively small studies. Trabectedin, particularly active against leiomyosarcoma and myxoid liposarcoma, is approved in many countries worldwide but not yet in the United States or Australia. Newer cytotoxic agents, including ifosfamide derivatives, are in current phase III testing. Although advances is systemic therapy of soft-tissue sarcomas have been hampered by their biologic heterogeneity, this diversity also serves as fertile ground for discovery and validation of targetable molecular drivers. The most notable success in this regard has been the development of small molecule therapies for gastrointestinal stromal tumors. Other targets of recent interest include mouse double minute 2 homolog (MDM2) in dedifferentiated liposarcoma and anaplastic lymphoma kinase (ALK) in inflammatory myofibroblastic tumor. Molecular therapies that have shown activity in diverse sarcoma populations include mammalian target of rapamycin (mTOR) inhibitors and vascular endothelial growth factor (VEGF-R) inhibitors. Among the latter, pazopanib demonstrated a progression-free survival over placebo in prior-treated patients with advanced sarcoma, and is now approved for use in the sarcomas in many countries. Efforts to understand the key molecular aberrations in any particular tumor continue towards a goal of individualized sarcoma therapy.

scholarly journals Molecular profiling of soft-tissue sarcomas with FoundationOne® Heme identifies potential targets for sarcoma therapy: a single-centre experience

2021 ◽  
Vol 13 ◽  
pp. 175883592110291
Author(s):  
Susanne Scheipl ◽  
Iva Brcic ◽  
Tina Moser ◽  
Stefan Fischerauer ◽  
Jakob Riedl ◽  
...  
Keyword(s):  
Soft Tissue ◽  
Soft Tissue Sarcomas ◽  
Myxoid Liposarcoma ◽  
Genetic Alterations ◽  
Tumour Suppressor Genes ◽  
Dna And Rna ◽  
Single Centre Experience ◽  
Somatic Gene ◽  
Fusion Detection ◽  
Gene Alterations

Background: Molecular diagnosis has become an established tool in the characterisation of adult soft-tissue sarcomas (STS). FoundationOne® Heme analyses somatic gene alterations in sarcomas via DNA and RNA-hotspot sequencing of tumour-associated genes. Methods: We evaluated FoundationOne® Heme testing in 81 localised STS including 35 translocation-associated and 46 complex-karyotyped cases from a single institution. Results: Although FoundationOne® Heme achieved broad patient coverage and identified at least five genetic alterations in each sample, the sensitivity for fusion detection was rather low, at 42.4%. Nevertheless, potential targets for STS treatment were detected using the FoundationOne® Heme assay: complex-karyotyped sarcomas frequently displayed copy-number alterations of common tumour-suppressor genes, particularly deletions in TP53, NF1, ATRX, and CDKN2A. A subset of myxofibrosarcomas (MFS) was amplified for HGF ( n = 3) and MET ( n = 1). PIK3CA was mutated in 7/15 cases of myxoid liposarcoma (MLS; 46.7%). Epigenetic regulators (e.g. MLL2 and MLL3) were frequently mutated. Conclusions: In summary, FoundationOne® Heme detected a broad range of genetic alterations and potential therapeutic targets in STS (e.g. HGF/MET in a subset of MFS, or PIK3CA in MLS). The assay’s sensitivity for fusion detection was low in our sample and needs to be re-evaluated in a larger cohort.

Compressive Mediastinal Mass and Large Pericardial Effusion in a Child

2015 ◽  
Vol 123 (4) ◽  
pp. 928-928 ◽  
Author(s):  
Adam C. Adler ◽  
Alan Jay Schwartz ◽  
Aruna T. Nathan
Keyword(s):  
Pericardial Effusion ◽  
Mediastinal Mass ◽  
Large Pericardial Effusion

scholarly journals Cytogenetics and Molecular Genetics of Myxoid Soft-Tissue Sarcomas

2011 ◽  
Vol 2011 ◽  
pp. 1-13 ◽  
Author(s):  
Jun Nishio ◽  
Hiroshi Iwasaki ◽  
Kazuki Nabeshima ◽  
Masatoshi Naito
Keyword(s):  
Soft Tissue ◽  
Molecular Genetic ◽  
Soft Tissue Sarcomas ◽  
Dermatofibrosarcoma Protuberans ◽  
Myxoid Liposarcoma ◽  
Genetic Alterations ◽  
Clinicopathological Characteristics ◽  
Low Grade ◽  
Mesenchymal Tumors ◽  
Fibromyxoid Sarcoma

Myxoid soft-tissue sarcomas represent a heterogeneous group of mesenchymal tumors characterized by a predominantly myxoid matrix, including myxoid liposarcoma (MLS), low-grade fibromyxoid sarcoma (LGFMS), extraskeletal myxoid chondrosarcoma (EMC), myxofibrosarcoma, myxoinflammatory fibroblastic sarcoma (MIFS), and myxoid dermatofibrosarcoma protuberans (DFSP). Cytogenetic and molecular genetic analyses have shown that many of these sarcomas are characterized by recurrent chromosomal translocations resulting in highly specific fusion genes (e.g., FUS-DDIT3 in MLS, FUS-CREB3L2 in LGFMS, EWSR1-NR4A3 in EMC, and COL1A1-PDGFB in myxoid DFSP). Moreover, recent molecular analysis has demonstrated a translocation t(1; 10)(p22; q24) resulting in transcriptional upregulation of FGF8 and NPM3 in MIFS. Most recently, the presence of TGFBR3 and MGEA5 rearrangements has been identified in a subset of MIFS. These genetic alterations can be utilized as an adjunct in diagnostically challenging cases. In contrast, most myxofibrosarcomas have complex karyotypes lacking specific genetic alterations. This paper focuses on the cytogenetic and molecular genetic findings of myxoid soft-tissue sarcomas as well as their clinicopathological characteristics.

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