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Myxoid/Round Cell Liposarcoma
A malignant soft tissue sarcoma characterized by a mix of myxoid (mucoid) and round cell components, often presenting in the deep tissues of the limbs, particularly the thighs. It commonly affects adults and has a tendency to recur and metastasize, particularly to the lungs and bones
Prevalence
N/A
US Estimated
N/A
Europe Estimated
Age of Onset
ICD-10
C49.9
Inheritance
This condition does not appear to have a clear pattern of inheritance.
5 Facts you should know
FACT
Myxoid/Round Cell Liposarcoma is a subtype of liposarcoma, a rare type of soft tissue sarcoma that arises from fat cells
FACT
It is characterized by a myxoid matrix and the presence of round, uniform cells in the tumor tissue
FACT
Myxoid/Round Cell Liposarcoma has a spectrum that includes both myxoid and round cell components, with the latter associated with a more aggressive behavior
FACT
This subtype often occurs in the extremities and is more common in adults, particularly in the middle-aged and older population
FACT
Diagnosis involves imaging studies, biopsy, and molecular testing. Treatment typically involves surgical resection, and the prognosis can vary based on the extent of the disease and the proportion of round cell areas
Interest over time
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Common signs & symptoms
Palpable Mass
A painless lump or swelling in the affected area, commonly in the deep tissues of the limbs, particularly the thighs.
Local Pain or Discomfort
Pain or discomfort at the site of the tumor, especially if the tumor presses on nearby nerves or tissues.
Limited Range of Motion
Difficulty moving the affected limb or joint if the tumor restricts mobility.
Swelling or Edema
Swelling or fluid retention around the tumor site, causing visible swelling or feeling of fullness.
Other Systemic Symptoms
In rare cases, systemic symptoms such as fatigue, weight loss, or fever may occur, though these are less common.
Current treatments
Treatment options for Myxoid/Round Cell Liposarcoma typically include a combination of surgery, radiation therapy, and chemotherapy. Here are the current treatments commonly used:
Surgery
The primary treatment for Myxoid/Round Cell Liposarcoma involves surgical removal (resection) of the tumor whenever feasible. The goal is to achieve complete resection with negative margins (no cancer cells left behind).
Radiation Therapy
Radiation therapy may be used before surgery (neoadjuvant) to shrink the tumor and make it easier to remove, or after surgery (adjuvant) to destroy any remaining cancer cells and reduce the risk of recurrence.
Chemotherapy
Chemotherapy, particularly using agents like doxorubicin and ifosfamide, may be used in cases where the tumor is unresectable, has spread (metastasized), or has recurred. These drugs are typically administered in cycles to target and kill cancer cells throughout the body.
Targeted Therapy
Emerging therapies targeting specific genetic alterations or molecular pathways in Myxoid/Round Cell Liposarcoma are under investigation in clinical trials. This includes exploring potential targets related to the DDIT3-FUS fusion gene.
Clinical Trials
Participation in clinical trials may offer access to new treatments and therapies that are being tested for efficacy and safety in treating Myxoid/Round Cell Liposarcoma.
Top Clinical Trials
| Title | Description | Phases | Status | Interventions | More Information |
|---|---|---|---|---|---|
| Study to Evaluate Safety and Antitumor Activity of Lete-Cel (GSK3377794) in HLA-A2+ Participants With NY-ESO-1 Positive Previously Treated Advanced (Metastatic or Unresectable) Synovial Sarcoma or Myxoid/Round Cell Liposarcoma | This trial will evaluate safety and efficacy of human engineered T-cell therapies, in participants with advanced tumors. This trial is a sub study of the Master study NCT03967223. | Phase 2 | Active | Drug: Letetresgene autoleucel (Lete-Cel (GSK3377794)) Drug: Cyclophosphamide Drug: Fludarabine | More Info |
| EPITOME-1015-I: a Study to Investigate the Safety and Tolerability of MDG1015 in Patients with Epithelial Ovarian Cancer, Gastroesophageal Adenocarcinoma, Round Cell Liposarcoma And/or Synovial Sarcoma | MDG1015 is a third generation TCR-T therapy product targeting NY-ESO-1/LAGE-1a armored and enhanced by the PD1-41BB costimulatory switch protein (CSP). The study purpose is to establish the safety, tolerability and preliminary efficacy of MDG1015 in patients with epithelial ovarian cancer, gastroeso... | Phase 1 | Not yet recruiting | Drug: Lymphodepletion Biological: TCR-T cells (MDG1015) | More Info |
| Phase I/Ib Study of NK Expressing an Affinity-enhanced T-cell Receptor (TCR) Against the NY-ESO-1 | The goal of this clinical research study is to find a recommended dose of donated NK cells that can be given along with chemotherapy to patients with advanced cancers. The safety and effects of this therapy will also be studied. | Phase 1 | Recruiting | Drug: Fludarabine phosphate Drug: NY-ESO-1 TCR/IL-15 NK Drug: Cyclophosphamide | More Info |
| Evaluation of Chest CT Versus Chest X-Ray for Lung Surveillance After Curative-Intent Resection of High-Risk Truncal-Extremity Soft Tissue Sarcoma | This phase III trial compares chest computed tomography (CT) to chest x-ray (CXR) for lung surveillance after curative-intent resection of high-risk truncal-extremity soft tissue sarcoma. Currently, complete oncologic resection (with or without radiation therapy) is the standard of care for most hig... | Not yet recruiting | Procedure: Biopsy Procedure: Chest Computed Tomography Procedure: Chest Radiography Other: Quality-of-Life Assessment Other: Questionnaire Administration | More Info | |
| International Prospective Registry on Local Treatment Approaches in MLS | To prospectively study commonly used local treatment approaches in Myxoid Liposarcoma (MLS) | Recruiting | Procedure: Surgery Radiation: Preoperative RT 36Gy Radiation: Preoperative RT 50Gy Radiation: Postoperative RT 50-66Gy Radiation: RT 36Gy | More Info | |
| Study to Evaluate Safety and Antitumor Activity of Lete-Cel (GSK3377794) in HLA-A2+ Participants With NY-ESO-1 Positive Previously Untreated Advanced (Metastatic or Unresectable) Synovial Sarcoma and Myxoid/Round Cell Liposarcoma | This trial will evaluate safety and efficacy of human engineered T-cell therapies, in participants with advanced tumors. This trial is a sub study of the Master study NCT03967223. | Phase 2 | Active | Drug: Letetresgene autoleucel Drug: Cyclophosphamide Drug: Fludarabine | More Info |
| NRSTS2021, A Risk Adapted Study Evaluating Maintenance Pazopanib, Limited Margin, Dose-Escalated Radiation Therapy and Selinexor in Non-Rhabdomyosarcoma Soft Tissue Sarcoma (NRSTS) | The study participant has been diagnosed with non-rhabdomyosarcoma (NRSTS). Primary Objectives Intermediate-Risk * To estimate the 3-year event-free survival for intermediate-risk patients treated with ifosfamide, doxorubicin, pazopanib, surgery, and maintenance pazopanib, with or without RT. * T... | Phase 1, Phase 2 | Recruiting | Procedure: Surgical resection Radiation: Proton beam radiation therapy Drug: Pazopanib Drug: Ifosfamide Drug: Doxorubicin Drug: Selinexor | More Info |
| A Study of Mirdametinib in Combination With Palbociclib in People With Liposarcoma | The purpose of this study is to find out whether mirdametinib in combination with palbociclib is an effective and safe treatment for people with metastatic, recurrent, and unresectable liposarcoma. This study will test different doses of mirdametinib in combination with a fixed dose of palbociclib t... | Phase 1, Phase 2 | Recruiting | Drug: Mirdametinib Drug: Palbociclib | More Info |
| Spearhead 1 Study in Subjects With Advanced Synovial Sarcoma or Myxoid/Round Cell Liposarcoma | This is a study to investigate the efficacy and safety of ADP-A2M4 in HLA-A\*02 eligible and MAGE-A4 positive subjects with metastatic or inoperable (advanced) Synovial Sarcoma (Cohort 1, 2 and 3 ) or MRCLS (Cohort 1) . | Phase 2 | Active | Genetic: afamitresgene autoleucel (previously ADP-A2M4) | More Info |