Desmoplastic Small Round Cell Tumor

Preferred Label : Desmoplastic Small Round Cell Tumor;

NCIt synonyms : Desmoplastic Small Round-Cell Tumor; Desmoplastic Small Round-Cell Neoplasm; DSRCT; Polyphenotypic Small Round Cell Tumor;

NCIt related terms : Desmoplas. small round cell tumor;

NCIt definition : An aggressive malignant soft tissue neoplasm of uncertain differentiation. It is characterized by a recurrent chromosomal translocation t(11;22)(p13;q12) and the presence of small round cells in a desmoplastic stroma. It usually affects children and young adults. The most common site of involvement is the abdomen. Patients usually present with abdominal distention, pain, ascites, and a palpable abdominal mass. The prognosis is usually poor.;

Alternative definition : NCI-GLOSS: A rare, aggressive cancer that usually affects young males and usually is located in the abdomen.;

Neoplastic status : Malignant;

ICD-O code : 8806/3;

Codes from synonyms : 10064587; CDR0000044495;

Details

Origin ID

C8300;

UMLS CUI

C0281508;

Currated CISMeF NLP mapping
- Desmoplastic small round cell tumor [ICD-O code]
- Desmoplastic small round cell tumor [ORDO Disease]
- Desmoplastic small round cell tumor [MedDRA LLT]
- Desmoplastic small round cell tumor (disorder) [SNOMED CT concept]
- Desmoplastic small round cell tumor (morphologic abnormality) [SNOMED CT concept]
- Desmoplastic small round cell tumour [ICD-11 Extension code]
- Desmoplastic small round cell tumour [MedDRA Preferred Term]
- Small round cell desmoplastic tumor [PASCAL descriptor]
- desmoplastic small round cell tumor [Disease (Nov.)]
- desmoplastic small round cell tumor [DO Concept]
- desmoplastic small round cell tumor [MeSH Descriptor]
- desmoplastic small round cell tumor [MeSH concept]
DO Cross reference
- desmoplastic small round cell tumor [DO Concept]
Disease excludes normal cell origin
- Epithelial Cell [NCIt concept]
Disease may have findings
- Abdominal Mass [NCIt concept]
- High Mitotic Activity [NCIt concept]
- Pain [NCIt concept]
- Peritoneal Effusion [NCIt concept]
- Tumor Cell Necrosis [NCIt concept]
Excludes anatomical site(s)
- Bone [NCIt concept]
Has associated anatomic sites
- Connective and Soft Tissue [NCIt concept]
Semantic type(s)
- Neoplastic Process [UMLS semantic type]
UMLS correspondences (same concept)
- Desmoplastic small round cell tumor [MedDRA LLT]
- Desmoplastic small round cell tumor [ORDO Disease]
- Desmoplastic small round cell tumor [ICD-O code]
- Desmoplastic small round cell tumor (disorder) [SNOMED CT concept]
- Desmoplastic small round cell tumor (morphologic abnormality) [SNOMED CT concept]
- Desmoplastic small round cell tumour [MedDRA Preferred Term]
- Small round cell desmoplastic tumor [PASCAL descriptor]
- desmoplastic small round cell tumor [MeSH concept]
- desmoplastic small round cell tumor [MeSH Descriptor]
- desmoplastic small round cell tumor [Disease (Nov.)]
concept_is_in_subset
- CTRP Disease Terminology [NCIt concept]
- CTRP Terminology [NCIt concept]
- GDC Terminology [NCIt concept]
- GDC Value Terminology [NCIt concept]
- Mapped ICDO3.1 Morphology PT Terminology [NCIt concept]
- Mapped ICDO3.1 Morphology Terminology [NCIt concept]
- Mapped ICDO3.1 Terminology [NCIt concept]
- Mapped ICDO3.2 Morphology PT Terminology [NCIt concept]
- Mapped ICDO3.2 Morphology Terminology [NCIt concept]
- Mapped ICDO3.2 Terminology [NCIt concept]
- NCI CTEP SDC Non-Rhabdomyosarcoma Soft Tissue Sarcoma Sub-Category Terminology [NCIt concept]
- NCI CTEP Simplified Disease Classification Terminology [NCIt concept]
disease_excludes_finding
- Benign Cellular Infiltrate [NCIt concept]
- Indolent Clinical Course [NCIt concept]
- Intermediate Filaments Present [NCIt concept]
- Intracytoplasmic Eosinophilic Inclusion [NCIt concept]
disease_excludes_normal_tissue_origin
- Epithelial Tissue [NCIt concept]
disease_has_abnormal_cell
- Malignant Cell [NCIt concept]
- Malignant Small Cell [NCIt concept]
- Malignant Small Round Cell [NCIt concept]
- Neoplastic Cell [NCIt concept]
disease_has_finding
- Desmoplastic Stroma Formation [NCIt concept]
- Malignant Cellular Infiltrate [NCIt concept]
- Metastatic/Recurrent Disease [NCIt concept]
- Tissue Differentiation Uncertain [NCIt concept]
disease_has_normal_tissue_origin
- Connective and Soft Tissue [NCIt concept]
- Soft Tissue [NCIt concept]
disease_mapped_to_gene
- EWSR1/WT1 Fusion Gene [NCIt concept]
pathogenesis_of_disease_involves_gene
- EWSR1/WT1 Fusion Gene [NCIt concept]

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