What is liposarcoma?

Updated: Oct 07, 2019
  • Author: Guy J Petruzzelli, MD, PhD, MBA, FACS; Chief Editor: Gregory Gary Caputy, MD, PhD, FICS  more...
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Answer

Answer

Clinical presentation

Liposarcomas usually manifest in the fifth to seventh decades of life. However, case reports have been published that describe liposarcomas in the pediatric population. [102]

These tumors manifest clinically on the proximal upper and lower extremities or within the retroperitoneum. Liposarcomas often grow very large and can exhibit a variety of histologic variants, including well-differentiated, myxoid, round cell, and pleomorphic. The well-differentiated subtype contains lipocytes and appears histologically similar to lipomas. The other subtypes contain cells (lipoblasts) that have a more primitive differentiation, resembling fetal fat cells with characteristic lipid-containing vacuoles that abut the nucleus. The most common variant is the myxoid subtype, which is histologically characterized by the presence of stellate mesenchymal cells with a sparse amount of lipoblasts. Clinically, the round cell and pleomorphic variants are more aggressive than the well-differentiated and myxoid subtypes; therefore, they are associated with a worse prognosis and have a tendency to recur after surgical excision.

Pathogenesis

Of all the subtypes, the best-characterized is the myxoid variant. Crozat and colleagues identified a characteristic balanced chromosomal translocation creating the TLS-CHOP fusion protein. [103] The exact function and role of this fusion protein in actual tumorigenesis remains a mystery. 

Treatment

The surgical treatment for liposarcoma entails excision and, if possible, resection with wide margins, depending on anatomic constraints. [104, 105]

Radiation therapy and chemotherapy as adjuvant treatments have been proposed and attempted on individual patients, but they have not been studied rigorously in clinical trials. [106, 107]  Radiotherapy for liposarcoma of the vulva was reported effective by Yokouchi in 2000. [108] Randomized trials need to be performed to define the specific role of radiation.


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