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1156420003: Desmoplastic small round cell tumor (disorder)


Status: current, Defined. Date: 31-Jul 2021. Module: SNOMED CT core

Descriptions:

Id Description Lang Type Status Case? Module
4574263015 DSRCT - desmoplastic small round cell tumour en Synonym Active Case sensitive SNOMED CT core
4574264014 DSRCT - desmoplastic small round cell tumor en Synonym Active Case sensitive SNOMED CT core
4574265010 Desmoplastic small round cell tumor (disorder) en Fully specified name Active Case insensitive SNOMED CT core
4574266011 Desmoplastic small round cell tumour en Synonym Active Case insensitive SNOMED CT core
4574267019 Desmoplastic small round cell tumor en Synonym Active Case insensitive SNOMED CT core
4574261018 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. en Definition Active Case sensitive SNOMED CT core
4574262013 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. en Definition Active Case sensitive SNOMED CT core


0 descendants.

Expanded Value Set


Outbound Relationships Type Target Active Characteristic Refinability Group Values
Desmoplastic small round cell tumour Associated morphology Desmoplastic small round cell tumour true Inferred relationship Some 1
Desmoplastic small round cell tumour Is a Primary malignant neoplasm of soft tissues false Inferred relationship Some
Desmoplastic small round cell tumour Finding site Soft tissue true Inferred relationship Some 1
Desmoplastic small round cell tumour Is a Sarcoma of soft tissue true Inferred relationship Some

Inbound Relationships Type Active Source Characteristic Refinability Group

Reference Sets

Clinical finding foundation reference set

Neoplasm and/or hamartoma reference set

Problem/Diagnosis reference set

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