Sarcopedia

MalignantSoft tissue

Pleomorphic Liposarcoma

Synonyms: High-grade liposarcoma variant

Highest-grade liposarcoma variant

Quick Facts

Behaviour

Malignant

Category

Soft tissue

Grade

High

Synonyms

High-grade liposarcoma variant

Category

Soft tissue

Behaviour

Malignant

Grade

High

Gender

Both equally

Tissue of Origin

Adipose

Epidemiology

  • Rare High-grade liposarcoma variant
  • Peak incidence in middle-aged adults
  • Lower extremity predominance

Clinical Features

  • Large, rapidly growing mass
  • Often presents with advanced disease

Location

  • Lower extremity
  • Retroperitoneum

Imaging

  • MRI: heterogeneous High-grade appearance
  • Minimal lipid content - mostly solid

Pathology

  • Pleomorphic lipoblasts and undifferentiated sarcoma
  • High mitotic rate, extensive necrosis
  • Liposarcoma grade 3 (Highest)

Genetics

  • Complex karyotype
  • FUS, MDM2 may be present

Treatment

  • Wide surgical excision
  • Chemotherapy indicated

Prognosis

Poor - High-grade malignancy with metastatic potential

Key Points

  • Highest-grade liposarcoma variant
  • Aggressive behaviour - multimodal therapy required

Workup - Blood Tests

FBC, U&E, LFTs, LDH - baseline

Workup - Local Imaging

  • MRI primary site - local staging
  • CT chest/abdomen/pelvis - metastatic staging

Workup - Biopsy

Core needle biopsy - confirm diagnosis

Workup - Staging

CT chest/abdomen/pelvis - metastases

Follow-up Summary

  • Year 1: Post-operative at 6 weeks; 3–4 monthly clinical examination and CXR
  • Year 2: 3–4 monthly clinical examination and CXR
  • Years 3–4: 6-monthly clinical examination and CXR
  • Years 5–10: Annual clinical examination and CXR
  • Discharge at 10 years after surgery