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Childhood Soft Tissue Sarcoma Treatment (PDQ®)
Patient Version   Health Professional Version   En español   Last Modified: 12/05/2008
Table 1. Frequent Aberrations Seen In Nonrhabdomyosarcomatous Soft Tissue Tumorsa

Histology  Chromosomal Aberrations   Genes Involved 
Alveolar soft part sarcoma t(x;17)(p11.2;q25) ASPL/TFE3 [4-6]
Clear cell sarcoma (malignant melanoma of soft parts) t(12;22)(q13;q12) ATF1/EWS
Congenital fibrosarcoma/ mesoblastic nephroma t(12;15)(p13,q25) [4] ETV-NTRK3 [4]
Dermatofibrosarcoma t(17;22)(q22;q13) COL1A1/PDGFB
Desmoplastic small round cell tumors t(11;22)(p13;q12) WT1/EWS [7]
Extraskeletal myxoid chondrosarcoma t(9;22)(q22;q12) EWS-CHN
Hemangiopericytoma t(12;19)(q13;q13.3) and t(13;22)(q22;q13.3)
Infantile fibrosarcoma t(12;15);+11; also +8,+17,+20 ETVG(TEL)/NTRK3
Leiomyosarcoma t(12;14)
Low-grade fibromyxoid sarcoma t(7;16)(q33;p11) FUS/BBF2H7
Malignant fibrous histiocytoma 19p+, ring chromosome
Myxoid liposarcoma t(12;16)(q13;p11) FUS/CHOP
Neurofibrosarcoma Deletion 17q11.2
Rhabdoid tumor t(1;22)(p36:q11.2) [4] SNFS/INI1 [4]
Synovial sarcoma t(x;18)(p11.2;q11.2) SYT/SSX

aAdapted from Sandberg [3] and Slater et al.[4]

References

  1. Sandberg AA: Translocations in malignant tumors. Am J Pathol 159 (6): 1979-80, 2001.  [PUBMED Abstract]

  2. Slater O, Shipley J: Clinical relevance of molecular genetics to paediatric sarcomas. J Clin Pathol 60 (11): 1187-94, 2007.  [PUBMED Abstract]

  3. Ladanyi M: The emerging molecular genetics of sarcoma translocations. Diagn Mol Pathol 4 (3): 162-73, 1995.  [PUBMED Abstract]

  4. Barnoud R, Sabourin JC, Pasquier D, et al.: Immunohistochemical expression of WT1 by desmoplastic small round cell tumor: a comparative study with other small round cell tumors. Am J Surg Pathol 24 (6): 830-6, 2000.  [PUBMED Abstract]


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