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 "growth factor" symptom

22q11.2 deletion syndrome

achondroplasia

adrenal incidentaloma

aniridia

cadasil

cholangiocarcinoma

coats disease

dedifferentiated liposarcoma

dentin dysplasia

dentinogenesis imperfecta

dystrophic epidermolysis bullosa

esophageal carcinoma

esophageal squamous cell carcinoma

hodgkin lymphoma, classical

holt-oram syndrome

inclusion body myositis

kallmann syndrome

krabbe disease

liposarcoma

lymphangioleiomyomatosis

oculocutaneous albinism

oral submucous fibrosis

pleomorphic liposarcoma

primary effusion lymphoma

primary hyperoxaluria type 1

severe combined immunodeficiency

systemic capillary leak syndrome

von hippel-lindau disease

wolf-hirschhorn syndrome

x-linked adrenoleukodystrophy

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