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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]

Soft tissue sarcomas in older children and adolescents often behave similarly to those in adult patients.[5,21]

Pediatric patients with unresected localized nonrhabdomyosarcomatous STSs have a poor outcome. Only about one-third of patients treated with multimodality therapy remain disease free.[36,39]; [40,41][Level of evidence: 3iiiA]

In a pooled analysis from U.S. and European pediatric centers, outcome was better for patients who received radiation therapy than for patients who did not, and outcome was better for patients whose tumor-removal procedure was deemed complete than for patients whose tumor removal was incomplete.[40][Level of evidence: 3iiiA]


WebMD Public Information from the National Cancer Institute

Last Updated: February 25, 2014
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