Abstract

BackgroundPleomorphic liposarcoma is the least common but most aggressive subtype of liposarcoma. Very few studies have presented data on pleomorphic liposarcoma specifically, often including a limited number of cases and short-term follow-up. As a result, the survivorship and prognostic characteristics of this tumor remain incompletely identified.Study design and settingCross-sectional analysis of the Surveillance Epidemiology and End Results database (1996–2015).ResultsOverall survival for the entire series was 54% (95% confidence interval [CI], 49–58%) and 40% (95% CI, 35–45%) at 5 and 10 years, respectively. Disease-specific survival for the entire series was 60% (95% CI, 56–65%) and 53% (95% CI, 48–58%) at 5 and 10 years, respectively. Patients who survived 10 years or more were more likely to die of events unrelated to pleomorphic liposarcoma. Univariate and multivariate analysis demonstrated that not receiving cancer-directed surgery was an independent poor prognostic factor. Older age (≥ 65 years old) was associated with worse overall survival but not disease-specific survival. Tumor stage and radiotherapy showed different impact on survival depending on tumor size. In comparison to localized staged tumors, regional stage only predicts poor survival in patients with tumor size less than 5 cm, while distant stage is an independent worse prognosis factor. Radiotherapy only benefits patients with tumor size larger than 10 cm. These results were confirmed in competing risk analysis.ConclusionSurvival rates of patients with pleomorphic liposarcoma has not changed over the past 20 years. Patients with distant stage have poor prognosis; regional stage indicates worse survival in patients with tumor size less than 5 cm. Receiving surgery could prolong the survival, while radiotherapy only benefits patients with large tumor size (> 10 cm). Older age is associated with poor overall survival but not disease-specific survival. Routine patient surveillance following initial diagnosis should at least be 10 years for pleomorphic liposarcoma.

Highlights

  • Pleomorphic liposarcoma is a rare malignancy that represents approximately 5–10% of all liposarcomas [1,2,3,4]

  • Older age is associated with poor overall survival but not disease-specific survival

  • Patient and tumor characteristics Between 1996 and 2015, a total of 11410 cases of liposarcoma were diagnosed with histology confirmation, of which pleomorphic liposarcoma accounted for 7.8% (891/11410)

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Summary

Introduction

Pleomorphic liposarcoma is a rare malignancy that represents approximately 5–10% of all liposarcomas [1,2,3,4]. Reported 5-year survivorship of pleomorphic liposarcoma was ranging from 29 to 63% [4,5,6,7,8,9], a figure that is significantly worse than other forms of liposarcoma [3, 5, 10] and which more parallel to other highgrade soft tissue sarcomas such as leiomyosarcoma and myxofibrosarcoma [7, 11]. Identifying survival and prognostic factors of a specific disease is valuable for modern clinical practice. A population-based study is more likely able to provide a comprehensive understanding of the survival and prognostic factors for this rare tumor. Very few studies have presented data on pleomorphic liposarcoma often including a limited number of cases and short-term follow-up. The survivorship and prognostic characteristics of this tumor remain incompletely identified

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