loading...| Preferred Name |
Desmoplastic small round cell tumor |
|
| Synonyms |
DSRCT - desmoplastic small round cell tumor Desmoplastic small round cell tumour |
|
| Definitions |
An aggressive soft tissue cancer that typically arises in serous lined surfaces of the abdominal or pelvic peritoneum, and spreads to the omentum, lymph nodes and hematogenously disseminates especially to the liver. Extraserous primary location has been reported in exceptional cases. The disease appears to originate from the mesothelium. In almost all cases, a specific translocation t(11;22)(p13;q12) is found that juxtaposes the EWSR1 gene to the WT1 tumor suppressor gene. However, the underlying molecular mechanism remains unknown. Several other associated chromosomal translocations have been described (t(5;19), t(X;16) and t(4;10)). Prognosis is poor. |
|
| ID |
http://purl.bioontology.org/ontology/SNOMEDCT/1156420003 |
|
| Active |
1 |
|
| altLabel |
DSRCT - desmoplastic small round cell tumor Desmoplastic small round cell tumor (disorder) DSRCT - desmoplastic small round cell tumour Desmoplastic small round cell tumour |
|
| CASE SIGNIFICANCE ID |
900000000000448009 900000000000017005 |
|
| CTV3ID |
XVG7k |
|
| cui |
C0281508 |
|
| definition |
An aggressive soft tissue cancer that typically arises in serous lined surfaces of the abdominal or pelvic peritoneum, and spreads to the omentum, lymph nodes and hematogenously disseminates especially to the liver. Extraserous primary location has been reported in exceptional cases. The disease appears to originate from the mesothelium. In almost all cases, a specific translocation t(11;22)(p13;q12) is found that juxtaposes the EWSR1 gene to the WT1 tumor suppressor gene. However, the underlying molecular mechanism remains unknown. Several other associated chromosomal translocations have been described (t(5;19), t(X;16) and t(4;10)). Prognosis is poor. An aggressive soft tissue cancer that typically arises in serous lined surfaces of the abdominal or pelvic peritoneum, and spreads to the omentum, lymph nodes and haematogenously disseminates especially to the liver. Extraserous primary location has been reported in exceptional cases. The disease appears to originate from the mesothelium. In almost all cases, a specific translocation t(11;22)(p13;q12) is found that juxtaposes the EWSR1 gene to the WT1 tumour suppressor gene. However, the underlying molecular mechanism remains unknown. Several other associated chromosomal translocations have been described (t(5;19), t(X;16) and t(4;10)). Prognosis is poor. |
|
| DEFINITION STATUS ID |
900000000000073002 |
|
| Effective time |
20210731 |
|
| Has associated morphology | ||
| Has finding site | ||
| notation |
1156420003 |
|
| prefLabel |
Desmoplastic small round cell tumor |
|
| Subset member |
447562003~MAPRULE~TRUE 447562003~MAPADVICE~ALWAYS C49.9 | POSSIBLE REQUIREMENT FOR MORPHOLOGY CODE 6011000124106~MAPGROUP~1 447562003~MAPTARGET~C49.9 900000000000508004~ACCEPTABILITYID~900000000000549004 447562003~MAPGROUP~1 447562003~CORRELATIONID~447561005 6011000124106~MAPCATEGORYID~447637006 900000000000509007~ACCEPTABILITYID~900000000000548007 900000000000508004~ACCEPTABILITYID~900000000000548007 447562003~MAPPRIORITY~1 447562003~MAPCATEGORYID~447637006 6011000124106~MAPTARGET~C49.9 6011000124106~MAPPRIORITY~1 900000000000497000~MAPTARGET~XVG7k 6011000124106~CORRELATIONID~447561005 6011000124106~MAPRULE~TRUE 6011000124106~MAPADVICE~ALWAYS C49.9 900000000000509007~ACCEPTABILITYID~900000000000549004 |
|
| tui |
T191 |
|
| Type ID |
900000000000003001 900000000000013009 |
|
| subClassOf |