What is the primary treatment for liposarcoma?

Primary Treatment for Liposarcoma

Surgical resection with wide margins is the primary treatment for liposarcoma, with the goal of achieving complete tumor removal (R0 resection). 1

Diagnostic Workup

Before treatment initiation:

• MRI for primary tumors of limb, trunk wall, and pelvis
• CT scan for retroperitoneal or other sites
• Chest CT for staging
• Additional imaging for myxoid liposarcoma: spine and pelvic MRI 2
• Core needle biopsy with imaging guidance for lesions >3 cm
• Central pathological review by an expert sarcoma pathologist

Treatment Algorithm Based on Liposarcoma Subtype

1. Well-differentiated/Atypical Lipomatous Tumors (ALT/WDL)

• Complete en bloc surgical resection 1
• Marginal resection acceptable for extremity lesions 1
• Radiotherapy generally not needed for extremity lesions with clear margins 1

2. Myxoid/Round Cell Liposarcoma

• Wide surgical excision with negative margins 2
• Adjuvant radiotherapy recommended due to high radiosensitivity 2, 3
• More comprehensive staging needed due to risk of extrapulmonary metastases 2

3. Dedifferentiated Liposarcoma

• Wide surgical excision with negative margins 1
• Consider neoadjuvant therapy for large tumors or borderline resectable disease 1
• Adjuvant radiotherapy for high-grade tumors or positive margins 1

4. Pleomorphic Liposarcoma

• Wide surgical excision with negative margins 3
• Adjuvant radiotherapy strongly recommended due to high recurrence rate (37%) 3
• Consider adjuvant chemotherapy due to high metastatic rate (41%) 3

Treatment Based on Location

Extremity Liposarcoma

• Wide surgical excision (R0) 1
• Adjuvant radiation therapy for:
  • High-grade, deep, >5 cm tumors (standard) 1
  • High-grade, deep, <5 cm tumors (recommended) 2
  • Low-grade, deep, >5 cm tumors (consider) 2
  • Positive margins 1

Retroperitoneal Liposarcoma

• En bloc surgical resection with adherent organs 1
• Aim for macroscopically complete resection in one specimen bloc 1
• Consider neoadjuvant radiotherapy for low/intermediate-grade retroperitoneal liposarcoma 1
• 5-year survival rates: 85.7% with R0 resection vs 33.3% with R1 resection 4

Management of Unresectable/Metastatic Disease

• First-line: Doxorubicin with or without ifosfamide 1, 5
• Second-line options:
  • Ifosfamide
  • Gemcitabine-based combinations
  • Trabectedin (especially for myxoid liposarcoma)
  • Eribulin 5, 6

Important Considerations

• Treatment should be performed at high-volume sarcoma centers by a multidisciplinary team 1
• Histologic subtype is the most important prognostic factor 3
• Myxoid liposarcoma has atypical metastatic patterns with extrapulmonary sites 3
• Local recurrence is the main cause of death in retroperitoneal liposarcoma 4
• Surgical re-excision should be considered for local recurrences 4

Follow-up Recommendations

• High-grade tumors: Every 3-4 months in first 2-3 years, then twice yearly until year 5, then annually 1
• Low-grade tumors: Every 4-6 months for local relapse, with less frequent chest imaging 1
• Myxoid liposarcoma: Include spine and pelvic MRI in surveillance 2

The evidence strongly supports that complete surgical resection offers the best chance for cure in liposarcoma, with adjuvant treatments tailored based on histologic subtype, tumor location, size, and margin status.