, Volume 18, Issue 13, pp 3762-3770
Date: 26 May 2011

Diagnosis, Management, and Outcome of Patients with Dedifferentiated Liposarcoma Systemic Metastasis

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Abstract

Background

Dedifferentiated liposarcomas (DDLPSs) result in worse patient outcomes than well-differentiated tumors despite shared molecular derangements. Prevalence and pattern of DDLPS systemic metastases have not been extensively reported; information regarding diagnosis, treatment, and outcomes of metastatic DDLPS patients is limited. Our study seeks to address this knowledge gap.

Methods

Metastatic patients were identified from a clinical database consisting of 277 DDLPS patients treated at the University of Texas M D Anderson Cancer Center (UTMDACC) (1993–2010). Only patients with radiologically demonstrable distant metastases were included. Patient, tumor, treatment, and outcome variables were recorded. Available imaging studies and tumor FFPE blocks were assessed.

Results

A total of 40 patients were identified, translating into a DDLPS metastatic rate of 14% (17% for de novo DDLPS and 9% for secondary dedifferentiation). The average patient age was 61 years with a male predilection. The retroperitoneum and lungs were the most common primary and metastatic tumor sites. Any of the 4 described DDLPS morphological subtypes harbored metastatic potential; MFH/UPS-like morphology was the most common. The median time from primary diagnosis to metastasis was 25 months; more than 50% of metastases developed without local failure. The median survival of metastatic patients was 11.5 months; the 5-year DSS was 5.2%. Patients amenable to complete surgical extirpation (n = 14) faired significantly better (P = .001; log rank).

Conclusions

Metastatic spread is an ominous consequence of DDLPS, especially regarding de novo tumors. Occurring relatively early in the course of disease and exhibiting a pulmonary predilection, these lesions are highly aggressive and commonly fatal. Further studies to identify metastatic biomarkers are needed.

M.P. Ghadimi and T. Al-Zaid contributed equally to the manuscript.