From the Guidelines
Myxoid liposarcoma should be treated with wide surgical resection to remove the tumor with clear margins, often combined with radiation therapy to reduce the risk of local recurrence, as this approach has been shown to improve outcomes in terms of morbidity, mortality, and quality of life 1.
Diagnosis and Treatment
Myxoid liposarcoma is a rare type of soft tissue cancer that develops from fat cells, characterized by its gelatinous appearance and distinctive genetic abnormality involving a chromosomal translocation between chromosomes 12 and 16. It typically presents as a painless, slow-growing mass in the deep soft tissues of the extremities, particularly the thigh.
Key Considerations
- Treatment primarily involves wide surgical resection to remove the tumor with clear margins, often combined with radiation therapy to reduce the risk of local recurrence.
- Chemotherapy may be considered for large tumors, metastatic disease, or high-grade variants.
- Myxoid liposarcoma has a better prognosis compared to other liposarcoma subtypes, with a 5-year survival rate of approximately 70-90%.
- Regular follow-up imaging is essential for monitoring recurrence, as these tumors can return even years after initial treatment.
- Genetic testing for the characteristic FUS-DDIT3 fusion gene can help confirm diagnosis in challenging cases.
Additional Treatment Options
- Trabectedin is a second-line option for advanced previously treated STS, including myxoid liposarcoma, and has shown high antitumor activity in this subtype 1.
- Pazopanib is an option for non-adipogenic STS, but its use in myxoid liposarcoma is not well established 1.
Important Notes
- The treatment of myxoid liposarcoma should be individualized and discussed in a multidisciplinary team setting.
- The use of molecular targeted agents, such as mammalian target of rapamycin inhibitors, crizotinib, and sunitinib, may be considered in selected cases, but their efficacy in myxoid liposarcoma is not well established 1.
From the Research
Diagnosis of Myxoid Liposarcoma
- Myxoid liposarcoma (MLPS) is a rare subtype of soft tissue sarcoma, characterized by a distinct pattern of hematogenous spread, as well as unique radiosensitivity and chemosensitivity 2.
- The most common anatomic localization of myxoid liposarcoma is the lower extremity (78.6%) 2.
- Diagnosis involves evaluating oncologic results, metastatic patterns, and treatment response after multimodal therapy 2.
Treatment of Myxoid Liposarcoma
- Treatment options for myxoid liposarcoma include surgery, radiation, chemotherapy, and clinical trials 3.
- Multimodal therapy, including concomitant MR-guided hyperthermia, can achieve excellent local control (94.7%) and significant tumor size reduction (≥ 25%) 2.
- Marginal or intralesional resection combined with radiotherapy can achieve local control, with no recurrences observed locally at a mean follow-up of 58.1 months 4.
- Preoperative radiation therapy can achieve a high rate of local control, with a mean tumor volume reduction rate of 52.0% 5.
- Chemotherapy may provide a clinical benefit in high-risk patients, but further investigation is needed to determine its optimal use 6.
Treatment Outcomes
- The 5-year rates for oncologic outcomes in patients treated with multimodal therapy are:
- Tumor size appears to be the greatest predictor of long-term disease control and overall survival 6.
- Definitive radiation therapy can achieve excellent results, with a mean tumor volume reduction rate of 55.0% and a tumor shrinkage maintenance rate of 100% 5.