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    Childhood Soft Tissue Sarcoma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - General Information

    Table 2. Frequent Chromosomal Aberrations Seen in Nonrhabdomyosarcomatous STSa

    Histology Chromosomal Aberrations Genes Involved
    STS = soft tissue sarcoma.
    a Adapted from Sandberg,[25]Slater et al.,[26]Mertens et al.,[27]and Romeo.[28]
    Alveolar soft part sarcoma t(x;17)(p11.2;q25) ASPL/TFE3[29,30,31]
    Angiomatoid fibrous histiocytoma t(12;16)(q13;p11), t(2;22)(q33;q12), t(12;22)(q13;q12) FUS/ATF1,EWSR1/CREB1,[32]EWS/ATF1
    Clear cell sarcoma t(12;22)(q13;q12), t(2;22)(q33;q12) ATF1/EWS,EWSR1/CREB1
    Congenital (infantile) fibrosarcoma/mesoblastic nephroma t(12;15)(p13,q25) ETV-NTRK3
    Dermatofibrosarcoma protuberans t(17;22)(q22;q13) COL1A1/PDGFB
    Desmoid fibromatosis Trisomy 8 or 20, loss of 5q21 CTNNB1orAPCmutations
    Desmoplastic small round cell tumors t(11;22)(p13;q12) EWS/WT1[33]
    Epithelioid hemangioendothelioma t(1;3)(p36;q25)[34] WWTR1/CAMTA1
    Epithelioid sarcoma Inactivation SMARCB1 SMARCB1
    Extraskeletal myxoid chondrosarcoma t(9;22)(q22;q12), t(9:17)(q22;q11), t(9;15)(q22;q21), t(3;9)(q11;q22) EWSR1/NR4A3,TAF2N/NR4A3,TCF12/NR4A3,TGF/NR4A3
    Hemangiopericytoma t(12;19)(q13;q13.3) and t(13;22)(q22;q13.3)
    Inflammatory myofibroblastic tumor t(1;2)(q23;q23), t(2;19)(q23;q13), t(2;17)(q23;q23), t(2;2)(p23;q13), t(2;11)(p23;p15)[35] TPM3/ALK,TPM4/ALK,CLTC/ALK,RANBP2/ALK,CARS/ALK
    Low-grade fibromyxoid sarcoma t(7;16)(q33;p11), t(11;16)(p11;p11) FUS/CREB3L2,FUS/CREB3L1
    Malignant peripheral nerve sheath tumor 17q11.2, loss or rearrangement 10p, 11q, 17q, 22q NF1
    Myxoid/round cell liposarcoma t(12;16)(q13;p11), t(12;22)(q13;q12) FUS/DD1T3,EWSR/DD1T3
    Rhabdoid tumor Inactivation SMARCB1 SMARCB1
    Synovial sarcoma t(x;18)(p11.2;q11.2) SYT/SSX
    Tenosynovial giant cell tumor t(1;2)(p13;q35) CSF1

    Prognosis

    The prognosis of nonrhabdomyosarcomatous STS tumors varies greatly depending on the histologic grade, invasiveness, tumor size, resectability, use of radiation therapy, site of primary tumor, and presence of metastases.[36,37,38] Some pediatric nonrhabdomyosarcomatous STSs are associated with a better outcome. For instance, infantile fibrosarcoma, presenting in infants and children younger than 4 years, has an excellent prognosis given that the tumor is highly chemosensitive and surgery alone can cure a significant number of these patients.[5]

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