Preferred Name |
Dermatofibrosarcoma protuberans |
|
Synonyms |
DFSP |
|
Definitions |
Dermatofibrosarcoma protuberans (DFSP) is a rare infiltrating soft tissue sarcoma, generally of low grade malignancy, arising from the dermis of the skin and characteristically associated with a specific chromosomal translocation t(17;22). |
|
ID |
http://www.orpha.net/ORDO/Orphanet_31112 |
|
alternative_term |
DFSP |
|
definition |
Dermatofibrosarcoma protuberans (DFSP) is a rare infiltrating soft tissue sarcoma, generally of low grade malignancy, arising from the dermis of the skin and characteristically associated with a specific chromosomal translocation t(17;22). |
|
definition_citation |
Orphanet |
|
expertlink |
https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=31112 |
|
has_age_of_onset |
All ages |
|
has_inheritance |
Not applicable |
|
hasDbXref |
MedDRA:10057070 UMLS:C0392784 ICD-10:C49.9 MeSH:C538219 ICD-11:2B53.Y OMIM:607907 |
|
label |
Dermatofibrosarcoma protuberans |
|
notation |
ORPHA:31112 |
|
part_of |
http://www.orpha.net/ORDO/Orphanet_271832 http://www.orpha.net/ORDO/Orphanet_79386 |
|
prefixIRI |
ORDO:Orphanet_31112 |
|
prefLabel |
Dermatofibrosarcoma protuberans |
|
present_in |
Europe AND has_point_prevalence_average_value : 10.0 AND has_point_prevalence_range : 1-5 / 10 000 |
|
treeView |
http://www.orpha.net/ORDO/Orphanet_271832 http://www.orpha.net/ORDO/Orphanet_79386 |
|
subClassOf |