Perilipin 1 expression differentiates liposarcoma from other types of soft tissue sarcoma

Lipid droplets, a morphologic feature of adipocytic tumors, are strongly regulated by associated proteins of the perilipin/PAT (perilipin, adipophilin, and tail-interacting protein of 47 kD) family. So far, the use of perilipins as markers for differential diagnosis of soft tissue tumors has only be...

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Main Authors: Straub, Beate Katharina (Author) , Pawella, Lena Maria (Author) , Renner, Marcus (Author) , Eiteneuer, Eva (Author) , Hashani, Merita (Author) , Schirmacher, Peter (Author) , Mechtersheimer, Gunhild (Author)
Format: Article (Journal)
Language:English
Published: 22 May 2019
In: The American journal of pathology
Year: 2019, Volume: 189, Issue: 8, Pages: 1547-1558
ISSN:1525-2191
DOI:10.1016/j.ajpath.2019.04.017
Online Access:Verlag, Volltext: https://doi.org/10.1016/j.ajpath.2019.04.017
Verlag, Volltext: http://www.sciencedirect.com/science/article/pii/S0002944018307016
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Author Notes:Beate K. Straub, Hagen R. Witzel, Lena M. Pawella, Marcus Renner, Eva Eiteneuer, Merita Hashani, Peter Schirmacher, Wilfried Roth, and Gunhild Mechtersheimer

MARC

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520 |a Lipid droplets, a morphologic feature of adipocytic tumors, are strongly regulated by associated proteins of the perilipin/PAT (perilipin, adipophilin, and tail-interacting protein of 47 kD) family. So far, the use of perilipins as markers for differential diagnosis of soft tissue tumors has only been studied in a few cases. The aim of this study was to investigate the expression of perilipins in 478 human soft tissue tumors and 60 respective normal tissues. Perilipin 1 was immunohistochemically positive in all studied cases of well-differentiated liposarcomas, >90% of myxoid round cell liposarcomas, and >70% of pleomorphic liposarcomas, whereas only the differentiated components of dedifferentiated liposarcomas were immunohistochemically positive for perilipin 1. All other types of soft tissue sarcomas were negative for perilipin 1. Perilipin 2 was more prominent in dedifferentiated and pleomorphic liposarcomas and nearly all other high-grade sarcomas. In well-differentiated liposarcomas, lipomas, or normal adipose tissue, perilipin 2 was virtually absent. In addition, long-term stimulation of adipogenesis in the liposarcoma cell line LiSa-2 restored perilipin 1 expression, as exhibited in the source tumor. Furthermore, knockdown of perilipin 2 or perilipin 3 in LiSa-2 cells influenced lipid droplet number and size as well as cell vitality. In summary, perilipin 1 is a promising marker for the differential diagnosis of liposarcomas from other soft tissue sarcomas, whereas perilipin 2 correlates negatively with tumor grade and may be therapeutically useful. 
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