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Cancer Health Center

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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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