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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)

Identity

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
Note liposarcomas are adipose tissue tumors, including low-malignant to highly malignant subtypes, constituting 10-15% of soft tissue sarcomas

Classification

   
  • 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

    Cytogenetics

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

    Bibliography

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    Characteristic sequence motifs at the breakpoints of the hybrid genes FUS/CHOP, EWS/CHOP and FUS/ERG in myxoid liposarcoma and acute myeloid leukemia.
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    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
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    Identification of genes differentially expressed in TLS-CHOP carrying myxoid liposarcomas.
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    Citation

    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 : http://AtlasGeneticsOncology.org/Tumors/liposarcID5029.html


    Other genes implicated (Data extracted from papers in the Atlas) [ 14 ]

    Genes CALR CDK4 CPM EWSR1 FRZB GPC5 HMGA2 HTRA3 KCNH1 MIR143
    PF4 PF4V1 PIM1 SRSF3

    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) FUS/DDIT3 [Case List]    t(12;16)(q13;p11) FUS/DDIT3 [Association List] Mitelman database (CGAP - NCBI)
    Mitelman database t(12;22)(q13;q12) EWSR1/DDIT3 [Case List]    t(12;22)(q13;q12) EWSR1/DDIT3 [Association List] Mitelman database (CGAP - NCBI)
    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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    indexed on : Mon Sep 18 17:21:21 CEST 2017


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