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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
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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.