U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Unresectable Desmoplastic Small Round Cell Tumor

MedGen UID:
1739996
•Concept ID:
C5418823
•
Neoplastic Process

Definition

A desmoplastic small round cell tumor that is not amenable to surgical resection. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVUnresectable Desmoplastic Small Round Cell Tumor

Professional guidelines

PubMed

The diagnosis and management of desmoplastic small round cell tumor: a review.
Hayes-Jordan A, Anderson PM
Curr Opin Oncol 2011 Jul;23(4):385-9. doi: 10.1097/CCO.0b013e3283477aab. PMID: 21577112

Recent clinical studies

Etiology

Trabectedin in advanced desmoplastic round cell tumors: a retrospective single-center series.
Verret B, Honore C, Dumont S, Terrier P, Adam J, Cavalcanti A, Sourrouille I, Klausner G, Ahlenc-Gelas M, Kiavue N, Domitrescu G, El Amarti L, Mir O, Le Cesne A
Anticancer Drugs 2017 Jan;28(1):116-119. doi: 10.1097/CAD.0000000000000435. PMID: 27782906
Clinical and computed tomography features of adult abdominopelvic desmoplastic small round cell tumor.
Shen XZ, Zhao JG, Wu JJ, Liu F
World J Gastroenterol 2014 May 7;20(17):5157-64. doi: 10.3748/wjg.v20.i17.5157. PMID: 24803835Free PMC Article
Use of radiofrequency for ablation of unresectable hepatic metastasis in desmoplastic small round cell tumor.
de Oliveira-Filho AG, Miranda ML, Diz FL, Guimarães RM, Aguiar SS, Bustorff-Silva JM
Med Pediatr Oncol 2003 Nov;41(5):476-7. doi: 10.1002/mpo.10386. PMID: 14515395

Diagnosis

Response to chemotherapy combined with anlotinib plus anlotinib maintenance in intra-abdominal desmoplastic small round cell tumors (IADSRCT): a case report and literature review.
Cheng K, Liu X, Chen Y, Zhou K, Li Z
BMC Gastroenterol 2022 Aug 17;22(1):388. doi: 10.1186/s12876-022-02463-y. PMID: 35978277Free PMC Article
Clinical and computed tomography features of adult abdominopelvic desmoplastic small round cell tumor.
Shen XZ, Zhao JG, Wu JJ, Liu F
World J Gastroenterol 2014 May 7;20(17):5157-64. doi: 10.3748/wjg.v20.i17.5157. PMID: 24803835Free PMC Article
Metastatic desmoplastic small round cell tumor controlled by an anthracycline-based regimen: review of the role of chemotherapy.
Mrabti H, Kaikani W, Ahbeddou N, Abahssain H, El Khannoussi B, Amrani M, Errihani H
J Gastrointest Cancer 2012 Mar;43(1):103-9. doi: 10.1007/s12029-011-9260-6. PMID: 21301996
The diagnosis and management of desmoplastic small round cell tumor: a review.
Hayes-Jordan A, Anderson PM
Curr Opin Oncol 2011 Jul;23(4):385-9. doi: 10.1097/CCO.0b013e3283477aab. PMID: 21577112
FNA biopsy of small round cell tumors of childhood: cytomorphologic features and the role of ancillary studies.
Silverman JF, Joshi VV
Diagn Cytopathol 1994;10(3):245-55. doi: 10.1002/dc.2840100312. PMID: 8050333

Therapy

Response to chemotherapy combined with anlotinib plus anlotinib maintenance in intra-abdominal desmoplastic small round cell tumors (IADSRCT): a case report and literature review.
Cheng K, Liu X, Chen Y, Zhou K, Li Z
BMC Gastroenterol 2022 Aug 17;22(1):388. doi: 10.1186/s12876-022-02463-y. PMID: 35978277Free PMC Article
Trabectedin in advanced desmoplastic round cell tumors: a retrospective single-center series.
Verret B, Honore C, Dumont S, Terrier P, Adam J, Cavalcanti A, Sourrouille I, Klausner G, Ahlenc-Gelas M, Kiavue N, Domitrescu G, El Amarti L, Mir O, Le Cesne A
Anticancer Drugs 2017 Jan;28(1):116-119. doi: 10.1097/CAD.0000000000000435. PMID: 27782906
A phase II study of tasisulam sodium (LY573636 sodium) as second-line or third-line treatment for patients with unresectable or metastatic soft tissue sarcoma.
Ryan CW, Matias C, Agulnik M, Lopez-Pousa A, Williams C, de Alwis DP, Kaiser C, Miller MA, Ermisch S, Ilaria R Jr, Keohan ML
Invest New Drugs 2013 Feb;31(1):145-51. Epub 2012 Apr 27 doi: 10.1007/s10637-012-9819-5. PMID: 22539091
Metastatic desmoplastic small round cell tumor controlled by an anthracycline-based regimen: review of the role of chemotherapy.
Mrabti H, Kaikani W, Ahbeddou N, Abahssain H, El Khannoussi B, Amrani M, Errihani H
J Gastrointest Cancer 2012 Mar;43(1):103-9. doi: 10.1007/s12029-011-9260-6. PMID: 21301996
The diagnosis and management of desmoplastic small round cell tumor: a review.
Hayes-Jordan A, Anderson PM
Curr Opin Oncol 2011 Jul;23(4):385-9. doi: 10.1097/CCO.0b013e3283477aab. PMID: 21577112

Prognosis

Trabectedin in advanced desmoplastic round cell tumors: a retrospective single-center series.
Verret B, Honore C, Dumont S, Terrier P, Adam J, Cavalcanti A, Sourrouille I, Klausner G, Ahlenc-Gelas M, Kiavue N, Domitrescu G, El Amarti L, Mir O, Le Cesne A
Anticancer Drugs 2017 Jan;28(1):116-119. doi: 10.1097/CAD.0000000000000435. PMID: 27782906
Clinical and computed tomography features of adult abdominopelvic desmoplastic small round cell tumor.
Shen XZ, Zhao JG, Wu JJ, Liu F
World J Gastroenterol 2014 May 7;20(17):5157-64. doi: 10.3748/wjg.v20.i17.5157. PMID: 24803835Free PMC Article
Use of radiofrequency for ablation of unresectable hepatic metastasis in desmoplastic small round cell tumor.
de Oliveira-Filho AG, Miranda ML, Diz FL, Guimarães RM, Aguiar SS, Bustorff-Silva JM
Med Pediatr Oncol 2003 Nov;41(5):476-7. doi: 10.1002/mpo.10386. PMID: 14515395
Urogenital involvement by desmoplastic small round-cell tumor.
Furman J, Murphy WM, Wajsman Z, Berry AD 3rd
J Urol 1997 Oct;158(4):1506-9. PMID: 9302152
FNA biopsy of small round cell tumors of childhood: cytomorphologic features and the role of ancillary studies.
Silverman JF, Joshi VV
Diagn Cytopathol 1994;10(3):245-55. doi: 10.1002/dc.2840100312. PMID: 8050333

Clinical prediction guides

Response to chemotherapy combined with anlotinib plus anlotinib maintenance in intra-abdominal desmoplastic small round cell tumors (IADSRCT): a case report and literature review.
Cheng K, Liu X, Chen Y, Zhou K, Li Z
BMC Gastroenterol 2022 Aug 17;22(1):388. doi: 10.1186/s12876-022-02463-y. PMID: 35978277Free PMC Article
Clinical and computed tomography features of adult abdominopelvic desmoplastic small round cell tumor.
Shen XZ, Zhao JG, Wu JJ, Liu F
World J Gastroenterol 2014 May 7;20(17):5157-64. doi: 10.3748/wjg.v20.i17.5157. PMID: 24803835Free PMC Article
FNA biopsy of small round cell tumors of childhood: cytomorphologic features and the role of ancillary studies.
Silverman JF, Joshi VV
Diagn Cytopathol 1994;10(3):245-55. doi: 10.1002/dc.2840100312. PMID: 8050333

Supplemental Content

Table of contents

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...