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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

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
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
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

A Practical Approach to Small Round Cell Tumors Involving the Gastrointestinal Tract and Abdomen.
Thway K, Fisher C
Surg Pathol Clin 2023 Dec;16(4):765-778. Epub 2023 Jul 11 doi: 10.1016/j.path.2023.05.012. PMID: 37863565
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
Clinicopathological features of desmoplastic small round cell tumors: clinical series and literature review.
Wang LL, Ji ZH, Gao Y, Chang H, Sun PP, Li Y
World J Surg Oncol 2021 Jun 30;19(1):193. doi: 10.1186/s12957-021-02310-6. PMID: 34193155Free PMC Article
A nationwide analysis of desmoplastic small round cell tumor.
Xiang T, Zhang SY, Wang SS, Fei RS, Li H
Medicine (Baltimore) 2020 Jul 24;99(30):e21337. doi: 10.1097/MD.0000000000021337. PMID: 32791732Free PMC Article
Desmoplastic Small Round Cell Tumor: Long-Term Complications After Cytoreduction and Hyperthermic Intraperitoneal Chemotherapy.
Stiles ZE, Murphy AJ, Anghelescu DL, Brown CL, Davidoff AM, Dickson PV, Glazer ES, Bishop MW, Furman WL, Pappo AS, Lucas JT Jr, Deneve JL
Ann Surg Oncol 2020 Jan;27(1):171-178. Epub 2019 Apr 8 doi: 10.1245/s10434-019-07339-2. PMID: 30963398Free PMC Article

Diagnosis

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
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
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
Imaging of pediatric desmoplastic small-round-cell tumor with pathologic correlation.
Iyer RS, Schaunaman G, Pruthi S, Finn LS
Curr Probl Diagn Radiol 2013 Jan-Feb;42(1):26-32. doi: 10.1067/j.cpradiol.2012.05.004. PMID: 23146167
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

Therapy

Effectiveness of irinotecan plus trabectedin on a desmoplastic small round cell tumor patient-derived xenograft.
Zuco V, Pasquali S, Tortoreto M, Percio S, Doldi V, Barisella M, Collini P, Dagrada GP, Brich S, Gasparini P, Fiore M, Casanova M, Frezza AM, Gronchi A, Stacchiotti S, Ferrari A, Zaffaroni N
Dis Model Mech 2023 Jun 1;16(6) Epub 2023 Jun 14 doi: 10.1242/dmm.049649. PMID: 37158111Free 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: Retrospective Review of Institutional Data and Literature Review.
Jayakrishnan T, Moll R, Sandhu A, Sanguino A, Kaur G, Mao S
Anticancer Res 2021 Aug;41(8):3859-3866. doi: 10.21873/anticanres.15179. PMID: 34281846
Desmoplastic Small Round Cell Tumor: Long-Term Complications After Cytoreduction and Hyperthermic Intraperitoneal Chemotherapy.
Stiles ZE, Murphy AJ, Anghelescu DL, Brown CL, Davidoff AM, Dickson PV, Glazer ES, Bishop MW, Furman WL, Pappo AS, Lucas JT Jr, Deneve JL
Ann Surg Oncol 2020 Jan;27(1):171-178. Epub 2019 Apr 8 doi: 10.1245/s10434-019-07339-2. PMID: 30963398Free PMC Article
Imaging of pediatric desmoplastic small-round-cell tumor with pathologic correlation.
Iyer RS, Schaunaman G, Pruthi S, Finn LS
Curr Probl Diagn Radiol 2013 Jan-Feb;42(1):26-32. doi: 10.1067/j.cpradiol.2012.05.004. PMID: 23146167

Prognosis

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
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
Imaging of pediatric desmoplastic small-round-cell tumor with pathologic correlation.
Iyer RS, Schaunaman G, Pruthi S, Finn LS
Curr Probl Diagn Radiol 2013 Jan-Feb;42(1):26-32. doi: 10.1067/j.cpradiol.2012.05.004. PMID: 23146167

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

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