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Soft Tissue Tumors
Myxoid/round cell liposarcoma - general
Author: Nat Pernick, M.D., PathologyOutlines.com, Inc.
Reviewer: David Lucas, M.D., University of Michigan Health Systems (January 2009)
Revised: 17 June 2009, last major update June 2009
See also subtypes myxoid-well differentiated liposarcoma, myxoid-round cell liposarcoma
Epidemiology
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● Common liposarcoma subtype (33% to 50%)
● Adults in 20’s to 40’s
● Rare in children under 10 years
Sites
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● Usually limb or thigh in muscle or deep tissue
● Almost never retroperitoneal, only rarely in subcutis
● Either well differentiated/low grade (well differentiated) or poorly differentiated/high grade (round cell) - click on links for more details and images
Treatment and prognosis
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● Wide local excision, possibly radiotherapy (Cancer 2009 May 26 [Epub ahead of print]) or chemotherapy
● Poor prognostic factors include poorly differentiated (round cell) component, large size, necrosis, high Ki-67 (J Clin Pathol 2003;56:579), p53 overexpression (Clin Cancer Res 2001;7:3977)
Molecular / cytogenetics
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● Associated with t(12;16)(q13;p11) - FUS-DDIT3 also called TLS-CHOP fusion gene in 90% (J Mol Diagn 2000;2:132)
● Less common is t(12;22)(q13;p11) - EWSR1-DDIT3 (Cancer Res 1995;55:24) or ins(12;16)(q13;p11.2p13) (AJCP 1997;108:35)
● Multifocal tumors may originate from same clone (Clin Cancer Res 2000;6:2788)
● Have immature adipogenic status (Hum Pathol 2009 Apr 13 [Epub ahead of print])
Molecular images
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Fusion gene
Additional references
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● Atlas of Genetics and Cytogenetics, Stanford University, OMIM 126337 (DDIT3/CHOP)
End of Soft Tissue Tumors > Myxoid/round cell liposarcoma
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