Pleomorphic liposarcoma: clinical observations and molecular variables.

[pleomorphic liposarcoma]

Pleomorphic liposarcoma (PLS ) is a rare high -grade sarcoma that has lipoblastic differentiation . In this study , the authors evaluated PLS natural history , patient outcomes , and commonly deregulated protein biomarkers .Medical records from patients (n = 155 ) who had PLS from 1993 to 2010 were reviewed . Univariate and multivariate analyses were conducted to identify independent prognosticators . A PLS tissue microarray (TMA ) (n = 56 patient specimens ) was constructed for immunohistochemical analysis of molecular markers , and p 53 gene sequencing (exons 5 -9 ) was conducted .The average patient age was 57 years , and the patients presented with primary disease (n = 102 ), recurrent disease (n = 16 ), and metastatic disease (n = 37 ). Lower extremity was the most common disease site (40 %), and the average tumor size was 11 cm . Complete follow-up data were available for 83 patients , and their median follow-up was 22 .6 months . The 5 -year disease-specific survival rate was 53 %; and recurrent disease , unresectability , and microscopic positive margins were identified as predictors of a poor prognosis . Systemic relapse (the strongest poor prognostic determinant ) developed in 35 % of patients with localized PLS . Immunohistochemical analysis revealed increased expression of peroxisome proliferator-activated receptor gamma (an adipogenic marker ), B-cell leukemia 2 and survivin (survival factors ), vascular endothelial growth factor (an angiogenic factor ), matrix metalloproteinase 2 , and other biomarkers . Frequent loss of retinoblastoma protein expression and high p 53 mutation rates (approximately 60 %) were observed .PLS is an aggressive , metastasizing sarcoma . Identifying ubiquitous molecular events underlying PLS progression is crucial for progress in patient management and outcomes .

Diseases
Validation

Diseases presenting "rare high-grade sarcoma" symptom

pleomorphic liposarcoma

You can validate or delete this automatically detected symptom