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Non-Rhabdomyosarcoma (Non-RMS)






Liposarcoma is the most common soft tissue sarcoma in adults (20% of all adult STS). This is a rare tumor in children and usually low grade in this setting.

Liposarcoma is a lipogenic tumor which is subclassified into four main histological groups:

  • Well differentiated liposarcoma (lipoma like and sclerosing types)
  • Myxoid round cell liposarcoma
  • Pleomorphic liposarcoma
  • De-differentiated liposarcoma

The histological group predicts the clinical course of the disease and the prognosis.


1. Well differentiated liposarcoma (lipoma like and sclerosing types)

  • Low grade malignancy
  • May recur locally but do not metastasize

2. Myxoid round cell liposarcoma

  • Characterized in 90% of tumors by the reciprocal chromosomal translocation t(12;16)(q13;p11)
  • This translocation creates the FUS-DDIT3 chimeric gene
  • Round cell liposarcoma is a variant of this tumor which behaves aggressively and may be associated with intra-abdominal and pelvic metastases.  Staging investigations should include a CT scan of the abdomen and pelvis as well as the chest.
  • Many tumors contain both components of myxoid and round cell liposarcoma
  • In the absence of a round cell component, myxoid sarcoma has a good prognosis

3. Pleomorphic liposarcoma

  • Highly malignant with a disorderly growth pattern and extensive cellular pleomorphism



  • Surgery is the most important therapy for liposarcoma
  • The role of adjuvant chemotherapy for liposarcoma is poorly defined





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