Review Article

Structure-Function Based Molecular Relationships in Ewing’s Sarcoma

Table 1

Cancer-associated gene fusions in ESFTs and EWS Fusion Proteins (EFPs) related tumours. Presented are the Fusion genes (providing DNA-binding) of EAD (providing Trans-activation) in human cancer. EFPs and the tumors that they cause: Ewing’s sarcoma, Desmoplastic Small Round Cell Tumor, Myxoid Liposarcoma, Extraskeletal Myxoid Chondrosarcoma, Malignant Melanoma of Soft Parts or Clear Cell Sarcoma, Acute leukaemia. The EAD contains a similar region of EWS in each case, including minimally the first seven exons of EWS. The EWS homologue translocated in Liposarcoma (TLS) provides the EAD. EWS fusion partners provide the DNA-binding domain of the EFP.

Trans-activationDNA-bindingTumorKaryotype

EADFLI-1Ewing’s sarcoma family of tumourst(11;22)(q24;q12)
EADERGEwing’s sarcoma family of tumourst(21;22)(q22;q12)
EADETV1Ewing’s sarcoma family of tumourst(7;22)(p22;q12)
EADETV4/E1AFEwing’s sarcoma family of tumourst(17;22)(q12;q12)
EADFEVEwing’s sarcoma family of tumourst(2;22)(q33;q12)
EAD (TLS)CHOPMyxoid liposarcomat(12;22)(q13;q12)
EADATF-1Malignant melanoma of soft parts/soft tissue clear cell sarcomat(12;22)(q13;q12)
EADWT1Desmoplastic small round cell tumourt(11;22)(p13;q12)
EADZSGSmall round cell sarcoma
Ewing’s sarcoma/PNET (rare)
t(1;22)(p36.1;q12)
EADPOU5F1 (OCT3/4)Undifferentiated bone sarcomat(6;22)(p21;q12)
EADNR4A3 (CHN/TEC or NOR1)Extraskeletal myxoid chondrosarcomat(9;22)(q22;q12)
EADCIZ/NMP4
ZNF384
Acute leukaemiat(12;22)(p13;q12)
FUS (TLS)CHOPmyxoid liposarcoma and round cell liposarcomat(12;16)(q13;p11)
EWSR1ATF1/CREB1Angiomatoid fibrous histiocytomat(2;22)(q33;q12)
EWSR1ERGThe Ewing sarcoma (ES) family of tumorst(1;21;7)(q25;q22.3;q22)
EWSR1CREB1Angiomatoid fibrous histiocytoma
clear cell sarcoma
t(2;22)(q34;q12)
RBP56/hTAFII6CHN orphan nuclear receptorExtraskeletal myxoid chondrosarcomast(9;17)(q22;q11.2)
EWSR1hSNF5/INI1 (SMARCB1)Extrarenal rhabdoid tumors of soft tissue/cell soft tissue sarcomast(1;11)(q12;q25)
EWSR1 or Not identifiedNot identified or
Fli-1
EWS/PNET
Soft tissue sarcoma
t(18;19)(q23;q13.2)
EWSUQCRHsmall round cell sarcomat(1;22)(p34;q12)
EWSR1NFATC2(ES/PNET)t(20;22)(q13;q12)