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Liposarcoma, Well-Differentiated, Not Otherwise Specified (NOS)
Source: Childhood Soft Tissue Sarcoma Treatment (PDQ®)–Health Professional Version, National Cancer Institute.
Source updated: April 21, 2025 · Captured 2026-09-09.
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Context: Treatment of Adipocytic Tumors
Liposarcoma is rare in the pediatric population and accounts for 3% of soft tissue sarcoma in patients younger than 20 years (see Table 1).
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In a review of 182 pediatric patients with adult-type sarcomas, only 14 had a diagnosis of liposarcoma.[7] One retrospective study identified 34 patients younger than 22 years from 1960 to 2011.[8] There were roughly equal numbers of male and female patients, and the median age was 18 years. In an international clinico-pathological review, the characteristics of 82 cases of pediatric liposarcoma were reported.[9] The median age was 15.5 years, and females were more commonly affected. In both reports, most patients had myxoid liposarcoma.[8,9]
A literature review of 275 cases of pediatric liposarcoma showed that:[10]
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Myxoid liposarcoma was the most common histology (68%), followed by well-differentiated liposarcoma (10.5%).
Twelve percent of patients died of disease, and most of the deaths occurred in patients with the pleomorphic and myxoid pleomorphic subtypes.
About 70% of patients with myxoid and well-differentiated liposarcoma were treated with surgery only. The overall clinical outcomes for these groups of patients were excellent, with no evidence of disease in 114 of 127 patients.
In contrast, more than 50% of patients with pleomorphic liposarcoma received radiation therapy and chemotherapy in addition to surgery, and their clinical outcome was suboptimal, with no evidence of disease in only 5 of 10 patients.
Germline TP53 pathogenic variants were seen in two patients with myxoid pleomorphic liposarcoma and two patients with well-differentiated liposarcoma who had a family history compatible with Li-Fraumeni syndrome.
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