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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

HistologyChromosomal AberrationsGenes Involved
STS = soft tissue sarcoma.
a Adapted from Sandberg,[25]Slater et al.,[26]Mertens et al.,[27]and Romeo.[28]
Alveolar soft part sarcomat(x;17)(p11.2;q25)ASPL/TFE3[29,30,31]
Angiomatoid fibrous histiocytomat(12;16)(q13;p11), t(2;22)(q33;q12), t(12;22)(q13;q12)FUS/ATF1,EWSR1/CREB1,[32]EWS/ATF1
Clear cell sarcomat(12;22)(q13;q12), t(2;22)(q33;q12)ATF1/EWS,EWSR1/CREB1
Congenital (infantile) fibrosarcoma/mesoblastic nephromat(12;15)(p13,q25)ETV-NTRK3
Dermatofibrosarcoma protuberanst(17;22)(q22;q13)COL1A1/PDGFB
Desmoid fibromatosisTrisomy 8 or 20, loss of 5q21CTNNB1orAPCmutations
Desmoplastic small round cell tumorst(11;22)(p13;q12)EWS/WT1[33]
Epithelioid hemangioendotheliomat(1;3)(p36;q25)[34]WWTR1/CAMTA1
Epithelioid sarcomaInactivation SMARCB1SMARCB1
Extraskeletal myxoid chondrosarcomat(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
Hemangiopericytomat(12;19)(q13;q13.3) and t(13;22)(q22;q13.3)
Inflammatory myofibroblastic tumort(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 sarcomat(7;16)(q33;p11), t(11;16)(p11;p11)FUS/CREB3L2,FUS/CREB3L1
Malignant peripheral nerve sheath tumor17q11.2, loss or rearrangement 10p, 11q, 17q, 22qNF1
Myxoid/round cell liposarcomat(12;16)(q13;p11), t(12;22)(q13;q12)FUS/DD1T3,EWSR/DD1T3
Rhabdoid tumorInactivation SMARCB1SMARCB1
Synovial sarcomat(x;18)(p11.2;q11.2)SYT/SSX
Tenosynovial giant cell tumort(1;2)(p13;q35)CSF1


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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