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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

ageofonset adult https://raremedicalnews.com/wp-content/uploads/2024/06/Depositphotos_245709784_L-e1719523120444.jpg

ICD-10

C49.9

Inheritance

This condition does not appear to have a clear pattern of inheritance.

5 Facts you should know

FACT

1

Myxoid/Round Cell Liposarcoma is a subtype of liposarcoma, a rare type of soft tissue sarcoma that arises from fat cells

FACT

2

It is characterized by a myxoid matrix and the presence of round, uniform cells in the tumor tissue

FACT

3

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

4

This subtype often occurs in the extremities and is more common in adults, particularly in the middle-aged and older population

FACT

5

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

Myxoid/Round Cell Liposarcoma is also known as...

Myxoid/Round Cell Liposarcoma is also known as:

  • MRCLS
  • Myxoid cell liposarcoma
  • Round cell liposarcoma
  • Myxoliposarcoma

What’s your Rare IQ?

Which genetic alteration is commonly associated with Myxoid/Round Cell Liposarcoma?

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

TitleDescriptionPhasesStatusInterventionsMore 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 LiposarcomaThis 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 2ActiveDrug: 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 SarcomaMDG1015 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 1Not yet recruitingDrug: 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-1The 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 1RecruitingDrug: 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 SarcomaThis 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 recruitingProcedure: 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 MLSTo prospectively study commonly used local treatment approaches in Myxoid Liposarcoma (MLS)RecruitingProcedure: 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 LiposarcomaThis 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 2ActiveDrug: 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 2RecruitingProcedure: 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 LiposarcomaThe 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 2RecruitingDrug: Mirdametinib
Drug: Palbociclib
More Info
Spearhead 1 Study in Subjects With Advanced Synovial Sarcoma or Myxoid/Round Cell LiposarcomaThis 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 2ActiveGenetic: afamitresgene autoleucel (previously ADP-A2M4)More Info