Desmoplastic Small Round Cell Tumor

Description

Desmoplastic small round cell tumor (DSRCT) is 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.

Clinical Features

Top most frequent phenotypes and symptoms related to Desmoplastic Small Round Cell Tumor

  • Neoplasm
  • Pain
  • Anemia
  • Hepatomegaly
  • Constipation
  • Abdominal pain
  • Weight loss
  • Nausea and vomiting
  • Lymphadenopathy
  • Ascites

And another 13 symptoms. If you need more information about this disease we can help you.

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Incidence and onset information

— Currently we don't have prevalence information about this disease (Not enough data available about incidence and published cases.)
No data available about the known clinical features onset.

Alternative names

Desmoplastic Small Round Cell Tumor Is also known as dsrct.

Researches and researchers

Doctors, researchs, and experts related to Desmoplastic Small Round Cell Tumor extracted from public data.

Desmoplastic Small Round Cell Tumor Experts map



Current Researchs and researchers

  • WIEN — Pr Thomas BRODOWICZ

    Coordinator of expert centre - Clinical expert - Principal investigator of clinical trial - Investigator of research project

    • Institution/s:
      — Universitätsklinik für Innere Medizin I, Allgemeines Krankenhaus der Stadt Wien
    • Research area/topic::

      E-TRAB: Geriatric Assessmen of Elderly "Unsuited" Patients Receiving Trabectedin in First Line Treatment For Advanced Soft Tissue Sarcomas (STS)


  • HAMBURG — Dr Anja LEHNHARDT

    Investigator of research project - Manager of registry

    • Institution/s:
      — UKE - Universitätsklinikum Hamburg-Eppendorf
    • Research area/topic::

      Genotype-phenotype correlation of patients with WT1 gene mutation


Desmoplastic Small Round Cell Tumor Recommended genes panels

Panel Name, Specifity and genes Tested/covered
MitoMet®Plus aCGH Analysis.

By Baylor Miraca Genetics Laboratories (United States).

RGS9, RHO, GRK1, RLBP1, RNASEL, BCS1L, RP1, RP2, RP9, RPE65, RPGR, RPL35A, MRPL3, RPS14, RS1, SAG, SARDH, SCO2, SCP2, SDHB , (...)

View the complete list with 612 more genes
Specificity
1 %
Genes
50 %
Hereditary Renal Cancer Panel.

By Baylor Miraca Genetics Laboratories (United States).

SDHA, SDHB, SDHC, SDHD, VHL, WT1, CDKN1C, SDHAF2, FLCN, FH, GPC3, MET, PTEN
Specificity
8 %
Genes
50 %
Comprehensive Hereditary Cancer Panel.

By Baylor Miraca Genetics Laboratories (United States).

RUNX1, SDHA, SDHB, SDHC, SDHD, BMPR1A, BRCA1, BRCA2, STK11, EPCAM, TP53, VHL, WT1, CBL, SUFU, CDC73, CDH1, CDK4, CDKN1C, CDKN2A , (...)

View the complete list with 39 more genes
Specificity
2 %
Genes
50 %
Early Onset Nephrotic Syndrome Evaluation.

By Athena Diagnostics Inc (United States).

WT1, NPHS2, PLCE1, LAMB2, NPHS1
Specificity
20 %
Genes
50 %
WT1 DNA Sequencing Test.

By Athena Diagnostics Inc (United States).

WT1
Specificity
100 %
Genes
50 %
WT1-Related Disorders - Sequencing.

By Children's Hospital Colorado Molecular Genetics Laboratory Children's Hospital Colorado (United States).

WT1
Specificity
100 %
Genes
50 %
WT1-Related Disorders - Del/Dup Analysis.

By Children's Hospital Colorado Molecular Genetics Laboratory Children's Hospital Colorado (United States).

WT1
Specificity
100 %
Genes
50 %
MyeloidDx by NGS.

By Molecular Genetics Diagnostic Laboratory Detroit Medical Center University Laboratories (United States).

RUNX1, SF3B1, SRSF2, BRAF, SMC1A, STAG2, TP53, U2AF1, KDM6A, WT1, IKZF1, CALR, CBL, CBLB, SETBP1, FBXW7, CDKN2A, PHF6, ASXL1, CEBPA , (...)

View the complete list with 32 more genes
Specificity
2 %
Genes
50 %

You can get up to 166 more panels with our dedicated tool

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Sources and references

You can check the following sources for additional information.

MESH ORPHANET Rare Disease Search Engine

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