| Entry |
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| Name |
Myxoid liposarcoma |
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| Supergrp |
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| Description |
Liposarcoma(LS) represents the most common soft-tissue sarcoma of adults and occurs most often in the thigh and retroperitoneum. LSs are subclassified into well-differentiated, myxoid, round cell, and pleomorphic types. The myxoid type, the most common subtype, accounts for approximately 40% to 54% of all liposarcomas. The karyotypic hallmark of myxoid LS is the t(12;16)(q13;p11), present cytogenetically in >90% of the cases. The translocation leads to the fusion of the DDIT3(CHOP) and FUS(TLS) genes at 12q13 and 16p11, respectively, and the generation of a FUS-DDIT3 hybrid protein. In 4 cases of myxoid LS, a variant chromosomal translocation has been described, t(12;22), in which DDIT3 fuses instead with EWSR1(EWS), a gene highly related to TLS.
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| Category |
Cancer
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| Brite |
Human diseases in ICD-11 classification [BR:br08403]
02 Neoplasms
Malignant neoplasms, except primary neoplasms of lymphoid, haematopoietic, central nervous system or related tissues
Malignant neoplasms, stated or presumed to be primary, of specified sites, except of lymphoid, haematopoietic, central nervous system or related tissues
Malignant mesenchymal neoplasms
2B59 Liposarcoma, primary site
H00049 Myxoid liposarcoma
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| Gene |
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| Other DBs |
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| Reference |
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| Authors |
Antonescu CR, Elahi A, Humphrey M, Lui MY, Healey JH, Brennan MF, Woodruff JM, Jhanwar SC, Ladanyi M |
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| Title |
Specificity of TLS-CHOP rearrangement for classic myxoid/round cell liposarcoma: absence in predominantly myxoid well-differentiated liposarcomas. |
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| Journal |
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| Reference |
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| Authors |
Letson GD, Muro-Cacho CA. |
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| Title |
Genetic and molecular abnormalities in tumors of the bone and soft tissues. |
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| Journal |
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| Reference |
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| Authors |
Helman LJ, Meltzer P. |
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| Title |
Mechanisms of sarcoma development. |
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| Journal |
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| Reference |
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| Authors |
Tateishi U, Hasegawa T, Beppu Y, Kawai A, Moriyama N. |
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| Title |
Prognostic significance of grading (MIB-1 system) in patients with myxoid liposarcoma. |
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| Journal |
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| Reference |
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| Authors |
Gibas Z, Miettinen M, Limon J, Nedoszytko B, Mrozek K, Roszkiewicz A, Rys J, Niezabitowski A, Debiec-Rychter M. |
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| Title |
Cytogenetic and immunohistochemical profile of myxoid liposarcoma. |
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| Journal |
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| Reference |
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| Authors |
Knight JC, Renwick PJ, Cin PD, Van den Berghe H, Fletcher CD. |
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| Title |
Translocation t(12;16)(q13;p11) in myxoid liposarcoma and round cell liposarcoma: molecular and cytogenetic analysis. |
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| Journal |
Cancer Res 55:24-7 (1995) |
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