Desmoplastic small round cell tumor

General Information (adopted from Orphanet):

Synonyms, Signs: DSRCT
Number of Symptoms 18
OrphanetNr: 83469
OMIM Id:
ICD-10: C48.2
UMLs: C0281508
MeSH: D058405
MedDRA: 10064581
Snomed: 128735004

Prevalence, inheritance and age of onset:

Prevalence: No data available.
Inheritance: Not applicable
[Orphanet]
Age of onset: Adolescent
Adult
[Orphanet]

Disease classification (adopted from Orphanet):

Parent Diseases: Primary malignant peritoneal tumor
 -Rare abdominal surgical disease
 -Rare oncologic disease
Soft tissue sarcoma
 -Rare oncologic disease

Symptom Information: Sort by abundance 

1
 (HPO:0100615) Ovarian neoplasm Occasional [Orphanet] 25 / 7739
2
 (HPO:0000035) Abnormality of the testis Occasional [Orphanet] 296 / 7739
3
 (HPO:0100006) Neoplasm of the central nervous system Occasional [Orphanet] 34 / 7739
4
 (HPO:0002585) Abnormality of the peritoneum Very frequent [Orphanet] 9 / 7739
5
 (HPO:0001541) Ascites Occasional [Orphanet] 94 / 7739
6
 (HPO:0007378) Neoplasm of the gastrointestinal tract Very frequent [Orphanet] 11 / 7739
7
 (HPO:0002027) Abdominal pain Very frequent [Orphanet] 184 / 7739
8
 (HPO:0002894) Neoplasm of the pancreas Occasional [Orphanet] 17 / 7739
9
 (HPO:0005214) Intestinal obstruction Frequent [Orphanet] 35 / 7739
10
 (HPO:0002240) Hepatomegaly Frequent [Orphanet] 467 / 7739
11
 (HPO:0002017) Nausea and vomiting Frequent [Orphanet] 134 / 7739
12
 (HPO:0004326) Cachexia Occasional [Orphanet] 71 / 7739
13
 (HPO:0004325) Decreased body weight Frequent [Orphanet] 492 / 7739
14
 (HPO:0001903) Anemia Occasional [Orphanet] 289 / 7739
15
 (HPO:0100526) Neoplasm of the lung Occasional [Orphanet] 26 / 7739
16
 (HPO:0100721) Mediastinal lymphadenopathy Frequent [Orphanet] 19 / 7739
17
 (HPO:0002716) Lymphadenopathy Very frequent [Orphanet] 129 / 7739
18
 (HPO:0030448) Soft tissue sarcoma Very frequent [Orphanet] 18 / 7739

Associated genes:

ClinVar (via SNiPA)

Gene symbol Variation Clinical significance Reference

Additional Information: