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Desmoplastic small round cell tumor(DSRCT)

MedGen UID:
83833
•Concept ID:
C0281508
•
Neoplastic Process
Synonym: DSRCT
SNOMED CT: Desmoplastic small round cell tumor (128735004); DSRCT - desmoplastic small round cell tumor (1156420003); Desmoplastic small round cell tumor (1156420003)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0019373
Orphanet: ORPHA83469

Definition

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. [from ORDO]

Professional guidelines

PubMed

Anesthesia and pain management of pediatric cytoreductive surgery with hyperthermic intraperitoneal chemotherapy.
Suchar AM, Lane J, King AC, Hayes AA, Phelps JR
Paediatr Anaesth 2023 Mar;33(3):193-200. Epub 2022 Sep 8 doi: 10.1111/pan.14551. PMID: 36052662
Management of desmoplastic small round cell tumor.
Hayes-Jordan A, LaQuaglia MP, Modak S
Semin Pediatr Surg 2016 Oct;25(5):299-304. Epub 2016 Sep 14 doi: 10.1053/j.sempedsurg.2016.09.005. PMID: 27955733Free PMC Article
Review: desmoplastic small round cell tumor: current treatment approach and role of targeted therapy.
Sankhala KK, Chawla SP
Clin Adv Hematol Oncol 2009 Jul;7(7):476-8. PMID: 19701156

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

Recent clinical studies

Etiology

The Small Round Cell Sarcomas Complexities and Desmoplastic Presentation.
Domanski HA
Acta Cytol 2022;66(4):279-294. Epub 2022 Apr 13 doi: 10.1159/000524260. PMID: 35417916Free PMC Article
Treatment Concepts and Challenges in Nonrhabdomyosarcoma Soft Tissue Sarcomas.
Fuchs J, Schmidt A, Warmann SW, Rodeberg DA
Surg Oncol Clin N Am 2021 Apr;30(2):355-371. Epub 2021 Jan 27 doi: 10.1016/j.soc.2020.11.004. PMID: 33706905
Desmoplastic nested spindle cell tumours and nested stromal epithelial tumours of the liver.
Misra S, Bihari C
APMIS 2016 Apr;124(4):245-51. Epub 2016 Jan 14 doi: 10.1111/apm.12502. PMID: 26994733
Incidental finding of desmoplastic small round cell tumor during trauma laparotomy.
Egle JP, Berbiglia L, Seman S, David D
Am Surg 2014 Dec;80(12):E331-3. PMID: 25513899
Small blue round cell tumor: an unusual case presentation in the foot.
Devall JM, Frush KM, Steiner L
J Am Podiatr Med Assoc 2011 Jul-Aug;101(4):363-9. doi: 10.7547/1010363. PMID: 21817009

Diagnosis

HER2 Antibody-Drug Conjugates Are Active against Desmoplastic Small Round Cell Tumor.
Zhang T, Febres-Aldana CA, Liu Z, Dix JM, Cheng R, Dematteo RG, Lui AJW, Khodos I, Gili L, Mattar MS, Lisanti J, Kwong C, Linkov I, Tipping MJ, de Stanchina E, Odintsov I, Ladanyi M, Somwar R
Clin Cancer Res 2024 Oct 15;30(20):4701-4713. doi: 10.1158/1078-0432.CCR-24-1835. PMID: 39120576Free PMC Article
Ewing sarcoma and Ewing-like tumors.
Sbaraglia M, Righi A, Gambarotti M, Dei Tos AP
Virchows Arch 2020 Jan;476(1):109-119. Epub 2019 Dec 4 doi: 10.1007/s00428-019-02720-8. PMID: 31802230
Desmoplastic small round cell tumour with ovarian involvement: a case report.
Lee SY, Koo YJ, Lee DH
J Obstet Gynaecol 2020 Jan;40(1):141-142. Epub 2019 Jun 18 doi: 10.1080/01443615.2019.1590687. PMID: 31210087
Desmoplastic Small Round Cell Tumor: A Rare Case of Extraluminal Bowel Obstruction and Review of the Literature.
Ofori E, Ramai D, Nigar S, Xiao P, Shahzad G, Reddy M
J Gastrointest Cancer 2019 Sep;50(3):560-563. doi: 10.1007/s12029-017-0045-4. PMID: 29282625
Desmoplastic small round cell tumor.
Drugas GT, Boulanger B, Korones DN, Katzman PJ, Koniaris LG
Pediatr Blood Cancer 2004 May;42(5):489-92. doi: 10.1002/pbc.10424. PMID: 15049027

Therapy

HER2 Antibody-Drug Conjugates Are Active against Desmoplastic Small Round Cell Tumor.
Zhang T, Febres-Aldana CA, Liu Z, Dix JM, Cheng R, Dematteo RG, Lui AJW, Khodos I, Gili L, Mattar MS, Lisanti J, Kwong C, Linkov I, Tipping MJ, de Stanchina E, Odintsov I, Ladanyi M, Somwar R
Clin Cancer Res 2024 Oct 15;30(20):4701-4713. doi: 10.1158/1078-0432.CCR-24-1835. PMID: 39120576Free PMC Article
Desmoplastic small round cell tumor: from state of the art to future clinical prospects.
Hovsepyan S, Giani C, Pasquali S, Di Giannatale A, Chiaravalli S, Colombo C, Orbach D, Bergamaschi L, Vennarini S, Gatz SA, Gasparini P, Berlanga P, Casanova M, Ferrari A
Expert Rev Anticancer Ther 2023 May;23(5):471-484. Epub 2023 Apr 10 doi: 10.1080/14737140.2023.2200171. PMID: 37017324
Desmoplastic Small Round Cell Tumor of the Kidney: AIRP Best Cases in Radiologic-Pathologic Correlation.
Walton WJ, Flores RR
Radiographics 2016 Sep-Oct;36(5):1533-8. doi: 10.1148/rg.2016150245. PMID: 27618327
Human Epidermal Growth Factor Receptor 2 (HER2) -Specific Chimeric Antigen Receptor-Modified T Cells for the Immunotherapy of HER2-Positive Sarcoma.
Ahmed N, Brawley VS, Hegde M, Robertson C, Ghazi A, Gerken C, Liu E, Dakhova O, Ashoori A, Corder A, Gray T, Wu MF, Liu H, Hicks J, Rainusso N, Dotti G, Mei Z, Grilley B, Gee A, Rooney CM, Brenner MK, Heslop HE, Wels WS, Wang LL, Anderson P, Gottschalk S
J Clin Oncol 2015 May 20;33(15):1688-96. Epub 2015 Mar 23 doi: 10.1200/JCO.2014.58.0225. PMID: 25800760Free PMC Article
Review: desmoplastic small round cell tumor: current treatment approach and role of targeted therapy.
Sankhala KK, Chawla SP
Clin Adv Hematol Oncol 2009 Jul;7(7):476-8. PMID: 19701156

Prognosis

Desmoplastic small round cell tumor: from state of the art to future clinical prospects.
Hovsepyan S, Giani C, Pasquali S, Di Giannatale A, Chiaravalli S, Colombo C, Orbach D, Bergamaschi L, Vennarini S, Gatz SA, Gasparini P, Berlanga P, Casanova M, Ferrari A
Expert Rev Anticancer Ther 2023 May;23(5):471-484. Epub 2023 Apr 10 doi: 10.1080/14737140.2023.2200171. PMID: 37017324
Molecular profiling in desmoplastic small round cell tumours.
Tam YB, Jones RL, Huang PH
Int J Biochem Cell Biol 2023 Apr;157:106383. Epub 2023 Feb 1 doi: 10.1016/j.biocel.2023.106383. PMID: 36736718
Ewing sarcoma and Ewing-like tumors.
Sbaraglia M, Righi A, Gambarotti M, Dei Tos AP
Virchows Arch 2020 Jan;476(1):109-119. Epub 2019 Dec 4 doi: 10.1007/s00428-019-02720-8. PMID: 31802230
Desmoplastic Small Round Cell Tumor of the Kidney: AIRP Best Cases in Radiologic-Pathologic Correlation.
Walton WJ, Flores RR
Radiographics 2016 Sep-Oct;36(5):1533-8. doi: 10.1148/rg.2016150245. PMID: 27618327
Desmoplastic nested spindle cell tumours and nested stromal epithelial tumours of the liver.
Misra S, Bihari C
APMIS 2016 Apr;124(4):245-51. Epub 2016 Jan 14 doi: 10.1111/apm.12502. PMID: 26994733

Clinical prediction guides

CXCR4-Directed Imaging and Endoradiotherapy in Desmoplastic Small Round Cell Tumors.
Hartlapp I, Hartrampf PE, Serfling SE, Wild V, Weich A, Rasche L, Roth S, Rosenwald A, Mihatsch PW, Hendricks A, Wiegering A, Wiegering V, Hänscheid H, Schirbel A, Werner RA, Buck AK, Wester HJ, Einsele H, Kunzmann V, Lapa C, Kortüm KM
J Nucl Med 2023 Sep;64(9):1424-1430. Epub 2023 Jun 22 doi: 10.2967/jnumed.123.265464. PMID: 37348915
Mediastinal desmoplastic small round cell tumor.
Jin D, Chen M, Wang B, Gou Y
Medicine (Baltimore) 2020 Oct 30;99(44):e22921. doi: 10.1097/MD.0000000000022921. PMID: 33126354Free PMC Article
Recurrent secondary genomic alterations in desmoplastic small round cell tumors.
Chow WA, Yee JK, Tsark W, Wu X, Qin H, Guan M, Ross JS, Ali SM, Millis SZ
BMC Med Genet 2020 May 11;21(1):101. doi: 10.1186/s12881-020-01034-w. PMID: 32393201Free PMC Article
Ewing sarcoma and Ewing-like tumors.
Sbaraglia M, Righi A, Gambarotti M, Dei Tos AP
Virchows Arch 2020 Jan;476(1):109-119. Epub 2019 Dec 4 doi: 10.1007/s00428-019-02720-8. PMID: 31802230
Desmoplastic small round cell tumors: cytologic, histologic, and immunohistochemical features.
Chang F
Arch Pathol Lab Med 2006 May;130(5):728-32. doi: 10.5858/2006-130-728-DSRCTC. PMID: 16683894

Supplemental Content

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    Clinical resources

    Practice guidelines

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      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.

    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Soft Tissue Sarcoma, 2024

    Reviews

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