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Soft Tissues: Liposarcoma :: malignant lipomatous tumors

Written2000-05Nils Mandahl
Department of Clinical Genetics, University Hospital, Lund, Sweden

(Note : for Links provided by Atlas : click)


ICD-Topo C470-C476,C478-C479,C490-C496,C498-C499 CONNECTIVE & SOFT TISSUE
ICD-Morpho 8850/3 Liposarcoma, NOS
Atlas_Id 5029
Phylum Soft Tissues::Liposarcoma
WHO/OMS Classification Soft Tissues
Note liposarcomas are adipose tissue tumors, including low-malignant to highly malignant subtypes, constituting 10-15% of soft tissue sarcomas


  • Well-differentiated liposarcoma: the well-differentiated liposarcomas are tumors of low grade malignancy that may recur locally but not metastasize; the terminolgy of subtypes is not straightforward; three related sybtypes have been distinguished: lipoma-like (the most common form), inflammatory, and sclerosing; other terms that have been suggested to describe at least subsets of these tumors are atypical lipoma and atypical lipomatous tumor
  • Myxoid liposarcoma / round cell liposarcoma: myxoid liposarcoma is the most common form of liposarcomas, constituting about half of the cases, with a relatively favorable prognosis; the much less common, and more aggressive round cell liposarcoma is regarded as a poorly differentiated variant of myxoid liposarcoma; pure round cell liposarcomas are very rare, and more often the tumors represent mixed liposarcomas with both myxoid and round cell components at different proportions; in recurrences the round cell component may increase
  • Pleomorphic liposarcoma: the pleomorphic liposarcomas are highly malignant tumors showing a disorderly growth pattern and extensive cellular pleomorphism
  • Clinics and Pathology

    Epidemiology the reported annual incidence of liposarcoma is in the range of 2.5 per million; liposarcomas are tumors of adult life with a median age of 55-60 years; patients younger than 15 years are rare; men are slightly more often affected than women
    Clinics the major sites are the lower extremities and the retroperitoneum; most tumors range from 5 to 10 cm in diameter, but much larger tumors are not rarely seen.
    Evolution the risk of distant metastases relate to the type and degree of histological differentiation; well-differentiated liposarcomas may occasionally dedifferentiate to highly malignant tumors that may metastasize
    Prognosis the survival rates are primarily dependent on the histological type, and patients with well-differentiated and myxoid liposarcomas, on the one hand, fare much better than round cell and pleomorphic liposarcomas on the other hand


    Pleomorphic liposarcoma
    few cases have been cytogenetically characterized; they invariably show complex karyotypic changes, with no characteristic changes identified, and an extensive intratumor heterogeneity


    Fusion genes in solid tumors.
    Aman P
    Seminars in cancer biology. 1999 ; 9 (4) : 303-318.
    PMID 10448117
    Expression patterns of the human sarcoma-associated genes FUS and EWS and the genomic structure of FUS.
    Aman P, Panagopoulos I, Lassen C, Fioretos T, Mencinger M, Toresson H, Höglund M, Forster A, Rabbitts TH, Ron D, Mandahl N, Mitelman F
    Genomics. 1996 ; 37 (1) : 1-8.
    PMID 8921363
    Rearrangement of the transcription factor gene CHOP in myxoid liposarcomas with t(12;16)(q13;p11).
    Aman P, Ron D, Mandahl N, Fioretos T, Heim S, Arheden K, Willén H, Rydholm A, Mitelman F
    Genes, chromosomes & cancer. 1992 ; 5 (4) : 278-285.
    PMID 1283316
    Interphase cytogenetic analysis of myxoid soft tissue tumors by fluorescence in situ hybridization and DNA flow cytometry using paraffin-embedded tissue.
    Aoki T, Hisaoka M, Kouho H, Hashimoto H, Nakata H
    Cancer. 1997 ; 79 (2) : 284-293.
    PMID 9010102
    CHOP (GADD153) and its oncogenic variant, TLS-CHOP, have opposing effects on the induction of G1/S arrest.
    Barone MV, Crozat A, Tabaee A, Philipson L, Ron D
    Genes & development. 1994 ; 8 (4) : 453-464.
    PMID 8125258
    HMGIC, the gene for an architectural transcription factor, is amplified and rearranged in a subset of human sarcomas.
    Berner JM, Meza-Zepeda LA, Kools PF, Forus A, Schoenmakers EF, Van de Ven WJ, Fodstad O, Myklebost O
    Oncogene. 1997 ; 14 (24) : 2935-2941.
    PMID 9205100
    Molecular abnormalities of MDM-2 in human sarcomas.
    BuesoRamos CE, Yang Y, Manshouri T, Feltz L, Ayala A, Glassman AB, Albitar M
    Int J Oncol. 1995 ; 7 : 1043-1048.
    Fusion of CHOP to a novel RNA-binding protein in human myxoid liposarcoma.
    Crozat A, Aman P, Mandahl N, Ron D
    Nature. 1993 ; 363 (6430) : 640-644.
    PMID 8510758
    Cytogenetic and fluorescence in situ hybridization investigation of ring chromosomes characterizing a specific pathologic subgroup of adipose tissue tumors.
    Dal Cin P, Kools P, Sciot R, De Wever I, Van Damme B, Van de Ven W, Van den Berghe H
    Cancer genetics and cytogenetics. 1993 ; 68 (2) : 85-90.
    PMID 8353809
    Correlation between clinicopathological features and karyotype in lipomatous tumors. A report of 178 cases from the Chromosomes and Morphology (CHAMP) Collaborative Study Group.
    Fletcher CD, Akerman M, Dal Cin P, de Wever I, Mandahl N, Mertens F, Mitelman F, Rosai J, Rydholm A, Sciot R, Tallini G, van den Berghe H, van de Ven W, Vanni R, Willen H
    The American journal of pathology. 1996 ; 148 (2) : 623-630.
    PMID 8579124
    Comparative genomic hybridization analysis of human sarcomas: I. Occurrence of genomic imbalances and identification of a novel major amplicon at 1q21-q22 in soft tissue sarcomas.
    Forus A, Weghuis DO, Smeets D, Fodstad O, Myklebost O, van Kessel AG
    Genes, chromosomes & cancer. 1995 ; 14 (1) : 8-14.
    PMID 8527390
    The structure and dynamics of ring chromosomes in human neoplastic and non-neoplastic cells.
    Gisselsson D, Höglund M, Mertens F, Johansson B, Dal Cin P, Van den Berghe H, Earnshaw WC, Mitelman F, Mandahl N
    Human genetics. 1999 ; 104 (4) : 315-325.
    PMID 10369161
    Characteristics of genomic breakpoints in TLS-CHOP translocations in liposarcomas suggest the involvement of Translin and topoisomerase II in the process of translocation.
    Kanoe H, Nakayama T, Hosaka T, Murakami H, Yamamoto H, Nakashima Y, Tsuboyama T, Nakamura T, Ron D, Sasaki MS, Toguchida J
    Oncogene. 1999 ; 18 (3) : 721-729.
    PMID 9989822
    Translocation t(12;16)(q13;p11) in myxoid liposarcoma and round cell liposarcoma: molecular and cytogenetic analysis.
    Knight JC, Renwick PJ, Cin PD, Van den Berghe H, Fletcher CD
    Cancer research. 1995 ; 55 (1) : 24-27.
    PMID 7805034
    p53 Mutation and MDM2 amplification in human soft tissue sarcomas.
    Leach FS, Tokino T, Meltzer P, Burrell M, Oliner JD, Smith S, Hill DE, Sidransky D, Kinzler KW, Vogelstein B
    Cancer research. 1993 ; 53 (10 Suppl) : 2231-2234.
    PMID 8387391
    Cytogenetic aberrations in 188 benign and borderline adipose tissue tumors.
    Mandahl N, Höglund M, Mertens F, Rydholm A, Willén H, Brosjö O, Mitelman F
    Genes, chromosomes & cancer. 1994 ; 9 (3) : 207-215.
    PMID 7515663
    Nonrandom pattern of telomeric associations in atypical lipomatous tumors with ring and giant marker chromosomes.
    Mandahl N, Mertens F, Willén H, Rydholm A, Kreicbergs A, Mitelman F
    Cancer genetics and cytogenetics. 1998 ; 103 (1) : 25-34.
    PMID 9595041
    Cytogenetic analysis of 46 pleomorphic soft tissue sarcomas and correlation with morphologic and clinical features: a report of the CHAMP Study Group. Chromosomes and MorPhology.
    Mertens F, Fletcher CD, Dal Cin P, De Wever I, Mandahl N, Mitelman F, Rosai J, Rydholm A, Sciot R, Tallini G, Van den Berghe H, Vanni R, Willén H
    Genes, chromosomes & cancer. 1998 ; 22 (1) : 16-25.
    PMID 9591630
    MDM2 gene amplification in bone and soft-tissue tumors: Association with tumor progression in differentiated adipose-tissue tumors.
    Nakayama T, Toguchida J, Wadayama B, Kanoe H, Kotoura Y, Sasaki MS
    Int J Cancer (Pred Oncol). 1995 ; 64 : 342-346.
    Characteristic sequence motifs at the breakpoints of the hybrid genes FUS/CHOP, EWS/CHOP and FUS/ERG in myxoid liposarcoma and acute myeloid leukemia.
    Panagopoulos I, Lassen C, Isaksson M, Mitelman F, Mandahl N, Aman P
    Oncogene. 1997 ; 15 (11) : 1357-1362.
    PMID 9315104
    Structure of the supernumerary ring and giant rod chromosomes in adipose tissue tumors.
    Pedeutour F, Forus A, Coindre JM, Berner JM, Nicolo G, Michiels JF, Terrier P, Ranchere-Vince D, Collin F, Myklebost O, Turc-Carel C
    Genes, chromosomes & cancer. 1999 ; 24 (1) : 30-41.
    PMID 9892106
    Distinct mdm2/p53 expression patterns in liposarcoma subgroups: implications for different pathogenetic mechanisms.
    Pilotti S, Della Torre G, Lavarino C, Di Palma S, Sozzi G, Minoletti F, Rao S, Pasquini G, Azzarelli A, Rilke F, Pierotti MA
    The Journal of pathology. 1997 ; 181 (1) : 14-24.
    PMID 9071998
    Fusion of the dominant negative transcription regulator CHOP with a novel gene FUS by translocation t(12;16) in malignant liposarcoma.
    Rabbitts TH, Forster A, Larson R, Nathan P
    Nature genetics. 1993 ; 4 (2) : 175-180.
    PMID 7503811
    Combined morphologic and karyotypic study of 59 atypical lipomatous tumors. Evaluation of their relationship and differential diagnosis with other adipose tissue tumors (a report of the CHAMP Study Group).
    Rosai J, Akerman M, Dal Cin P, DeWever I, Fletcher CD, Mandahl N, Mertens F, Mitelman F, Rydholm A, Sciot R, Tallini G, Van den Berghe H, Van de Ven W, Vanni R, Willen H
    The American journal of surgical pathology. 1996 ; 20 (10) : 1182-1189.
    PMID 8827023
    Cytogenetic findings in liposarcoma correlate with histopathologic subtypes.
    Sreekantaiah C, Karakousis CP, Leong SP, Sandberg AA
    Cancer. 1992 ; 69 (10) : 2484-2495.
    PMID 1568170
    GLI gene expression in bone and soft tissue sarcomas of adult patients correlates with tumor grade.
    Stein U, Eder C, Karsten U, Haensch W, Walther W, Schlag PM
    Cancer research. 1999 ; 59 (8) : 1890-1895.
    PMID 10213497
    Gains and losses of DNA sequences in liposarcomas evaluated by comparative genomic hybridization.
    Szymanska J, Tarkkanen M, Wiklund T, Virolainen M, Blomqvist C, Asko-Seljavaara S, Tukiainen E, Elomaa I, Knuutila S
    Genes, chromosomes & cancer. 1996 ; 15 (2) : 89-94.
    PMID 8834171
    Combined morphologic and karyotypic study of 28 myxoid liposarcomas. Implications for a revised morphologic typing, (a report from the CHAMP Group).
    Tallini G, Akerman M, Dal Cin P, De Wever I, Fletcher CD, Mandahl N, Mertens F, Mitelman F, Rosai J, Rydholm A, Sciot R, Van den Berghe H, Van den Ven W, Vanni R, Willen H
    The American journal of surgical pathology. 1996 ; 20 (9) : 1047-1055.
    PMID 8764741
    Identification of genes differentially expressed in TLS-CHOP carrying myxoid liposarcomas.
    Thelin-Jä S, Lassen C, Panagopoulos I, Mandahl N, Aman P
    International journal of cancer. Journal international du cancer. 1999 ; 83 (1) : 30-33.
    PMID 10449603
    Complexity of 12q13-22 amplicon in liposarcoma: microsatellite repeat analysis.
    Wolf M, Aaltonen LA, Szymanska J, Tarkkanen M, Blomqvist C, Berner JM, Myklebost O, Knuutila S
    Genes, chromosomes & cancer. 1997 ; 18 (1) : 66-70.
    PMID 8993982
    Inflammatory Bowel Disease Questionnaire: cross-cultural adaptation and further validation.
    de Boer AG, Wijker W, Bartelsman JF, de Haes HC
    European journal of gastroenterology & hepatology. 1995 ; 7 (11) : 1043-1050.
    PMID 8680903


    This paper should be referenced as such :
    Mandahl, N
    Soft tissue tumors: Liposarcoma / malignant lipomatous tumors
    Atlas Genet Cytogenet Oncol Haematol. 2000;4(3):138-141.
    Free journal version : [ pdf ]   [ DOI ]
    On line version :

    Other genes implicated (Data extracted from papers in the Atlas) [ 17 ]


    Translocations implicated (Data extracted from papers in the Atlas)

     t(12;16)(q13;p11) FUS/DDIT3
     t(12;22)(q13;q12) EWSR1/DDIT3

    External links

    Mitelman database t(12;16)(q13;p11) [CaseList]     t(12;16)(q13;p11) [Transloc - MCList]   FUS/DDIT3 Fusion - MCList]
    COSMIC[ FUS ]   [ DDIT3 ]
    Mitelman database t(12;22)(q13;q12) [CaseList]     t(12;22)(q13;q12) [Transloc - MCList]   EWSR1/DDIT3 Fusion - MCList]
    COSMIC[ EWSR1 ]   [ DDIT3 ]
    arrayMap Topo ( C47,C49) arrayMap ((UZH-SIB Zurich)   [auto + random 100 samples .. if exist ]   [tabulated segments]
    Mitelman databaseEWSR1/DDIT3[MCList]    EWSR1 (22q12.2) DDIT3 (12q13.3)   ins(22;12)(q12;q13q14)
    TICdbEWSR1/DDIT3    EWSR1 (22q12.2) DDIT3 (12q13.3)
    COSMIC_fusionFUS (16p11.2) DDIT3 (12q13.3)    [fusion1018] [fusion1019] [fusion291] [fusion292] [fusion293] [fusion294] [fusion297] [fusion298] [fusion300] [fusion301] [fusion302] [fusion303] [fusion304] [fusion342] [fusion832] [fusion833] [fusion834] [fusion835] [fusion836] [fusion867] [fusion868]
    Mitelman databaseFUS/DDIT3[MCList]    FUS (16p11.2) DDIT3 (12q13.3)   t(12;16)(q13;p11)
    TICdbFUS/DDIT3    FUS (16p11.2) DDIT3 (12q13.3)
    Disease databaseSoft Tissues: Liposarcoma :: malignant lipomatous tumors
    REVIEW articlesautomatic search in PubMed
    Last year articlesautomatic search in PubMed

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