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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.
 
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

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
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
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

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
Domanski HA
Acta Cytol 2022;66(4):279-294. Epub 2022 Apr 13 doi: 10.1159/000524260. PMID: 35417916Free PMC Article
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
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
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

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
Domanski HA
Acta Cytol 2022;66(4):279-294. Epub 2022 Apr 13 doi: 10.1159/000524260. PMID: 35417916Free PMC Article
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
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
Hayes-Jordan A, Anderson PM
Curr Opin Oncol 2011 Jul;23(4):385-9. doi: 10.1097/CCO.0b013e3283477aab. PMID: 21577112

Therapy

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
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
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
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
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

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
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
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
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
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

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
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
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
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
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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