Gene Translocations In Soft Tissue Tumors

 

 

Uploaded: 2007-12-07, Updated: 2007-12-07

 

Tumor

Translocation

 Fused Gene

Alveolar rhabdomyosarcoma

t(2;13)(q35;q14)

65%

PAX3-FKHR

t(1;13)(p36;q14)

15%

PAX7-FKHR

Alveolar soft part sarcoma

t(X;17)(p11;q25)

>95%

ASPL-TFE3

Clear cell sarcoma

t(12;22)(q13;q12)

>90%

EWS-ATF1

t(2;22)(q33;q12)

 

EWS-CREB1

Dermatofibrosarcoma protuberans

t(17;22)(q21;q13)

>90%

COL1A1-PDGFB

Desmoplastic small round cell tumor

t(11;22)(p13;q12)

>95%

EWS-WT1

Extraskeletal myxoid chondrosarcoma

t(9;22)(q22-q3;q12)

75%

EWS-NR4A3

t(9;17)(q22:q11)

25%

TAF15-NR4A3

Ewing sarcoma/PNET

t(11;22)(q24;q12)

90%

EWS-FLI1

t(21;22)(q22;q12)

5%

EWS-ERG

Fibromyxoid sarcoma (low-grade)

t(7;16)(q33;p11.2)

>95%

FUS-CREB3L2

t(11;16)(p13;p11.2)

<5%

FUS-CREB3L1

Infantile fibrosarcoma  

t(12;15)(p13;q26)

>95%

ETV6-NTRK3

Inflammatory myofibroblastic tumor

t with 2p23

>50%

ALK fusions

Myxoid/round cell liposarcoma

t(12;16)(q13;p11)

>95%

FUS-CHOP

t(12;22)(q13;q11)

<5%

EWS-CHOP

Synovial sarcoma

t(X;18)(p11.2;q11.2)

65%

 SYT-SSX1

35%

SYT-SSX2

<1%

SYT-SSX4

     
List of Genes Involved in Translocation

Gene

Aliases

Location

Function

ALK

 

 2p23

Membrane associated tyrosine kinase receptor; probable role in the nervous system development.

ASPL

ASPSCR1

17q25

Contains an UBX domain, may be related to the ubiquitylation pathway

ATF1

TREB36

 12q13

DNA binding protein, members of the CREB protein family

CHOP

DDIT3

 12q13.1-q13.2

Belongs to the C/EBP family of transcription factors

COL1A1

 

 17q21.31-q22

Member of the collagen family

CREB3L2

BBF2H7

 7q34

B-ZIP transcription factor

ETV6

TEL

 12p13.1

Transcriptional regulator; important in vitelline angiogenesis and in bone marrow hematopoiesis

EWSR1

 

 22q12

RNA binding

FKHR

FOXO1

13q14.1 

N/D

FLI1

EWSR2

11q24.1-q24.3

N/D

FUS

TLS

16p11.2

RNA-binding protein, may play role in the BCR/ABL-mediated leukemogenesis.

NR4A3

TEC

 9q22

Orphan nuclear receptor, roles in cell proliferation, steroid/thyroid receptor super family.

NTRK3

TrkC

 15q25

Tyrosine kinase cell surface receptor, proliferation and differentiation of neural derived cells

PAX3

CDHS

 2q35-2q36.1

Transcription factor, role in the development of the neuroskeletal structures

PAX7

HUP1

1p36.2-p36.12

N/D

PDGFB

 

 22q12.3-q13.1

Member of the PDGF/VEGF family, potent growth factor.

SSX1

SSRC

 Xp11.23-p11.22

 N/D

SSX2

SSX

 Xp11.23-p11.22

 N/D

SYT

SS18

 18q11.2

Transcriptional coactivator.

TFE3

ITF

21q22.3

Promotes migration of epithelial cells and enhance mucosal healing and epithelial restitutions.

WT1

 

11p13 

Zinc finger transcription factor.

     
REFERENCES
  • Enzinger and Weiss's Soft Tissue Tumors, 5th Edition. By Drs. Sharon Weiss and John Goldblum. 2007
  • http://atlasgeneticsoncology.org/