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Peritoneal Desmoplastic Small Round Cell Tumor

MedGen UID:
925936
•Concept ID:
C4288404
•
Neoplastic Process
Synonym: Peritoneal DSRCT

Definition

A desmoplastic small round cell tumor that occurs in the abdominal and/or pelvic peritoneum. [from NCI]

Term Hierarchy

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

Professional guidelines

PubMed

Peritoneal disease: key imaging findings that help in the differential diagnosis.
Campos NMF, Almeida V, Curvo Semedo L
Br J Radiol 2022 Feb 1;95(1130):20210346. Epub 2021 Nov 23 doi: 10.1259/bjr.20210346. PMID: 34767464Free PMC Article
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
Management of desmoplastic small round-cell tumors in children and young adults.
Jordan AH, Pappo A
J Pediatr Hematol Oncol 2012 May;34 Suppl 2:S73-5. doi: 10.1097/MPH.0b013e31824e38ad. PMID: 22525411

Recent clinical studies

Etiology

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
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
Desmoplastic Small Round Cell Tumor: Imaging Pattern of Disease at Presentation.
Morani AC, Bathala TK, Surabhi VR, Yedururi S, Jensen CT, Huh WW, Prasad S, Hayes-Jordan A
AJR Am J Roentgenol 2019 Mar;212(3):W45-W54. Epub 2019 Jan 23 doi: 10.2214/AJR.18.20179. PMID: 30673334
Desmoplastic small round cell tumor: A nationwide study of a rare sarcoma.
Stiles ZE, Dickson PV, Glazer ES, Murphy AJ, Davidoff AM, Behrman SW, Bishop MW, Martin MG, Deneve JL
J Surg Oncol 2018 Jun;117(8):1759-1767. Epub 2018 Jun 7 doi: 10.1002/jso.25071. PMID: 29878371
Desmoplastic small round cell tumor.
Leuschner I, Radig K, Harms D
Semin Diagn Pathol 1996 Aug;13(3):204-12. PMID: 8875710

Diagnosis

Omental desmoplastic small round cell tumor with metastasis.
Yelamanchi R, Yadav E, Gupta N, Ahuja A, Chauhan DS
Indian J Pathol Microbiol 2022 Jan-Mar;65(1):208-210. doi: 10.4103/IJPM.IJPM_548_20. PMID: 35075000
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
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
Management of desmoplastic small round-cell tumors in children and young adults.
Jordan AH, Pappo A
J Pediatr Hematol Oncol 2012 May;34 Suppl 2:S73-5. doi: 10.1097/MPH.0b013e31824e38ad. PMID: 22525411
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

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: 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
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
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

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
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
Desmoplastic small round-cell tumour of the peritoneal cavity: case report and literature review.
Tsoukalas N, Kiakou M, Nakos G, Tolia M, Galanopoulos M, Tsapakidis K, Kamposioras K, Christofyllakis C, Dimitrakopoulos G, Sambaziotis D
Ann R Coll Surg Engl 2020 Apr;102(4):e77-e81. Epub 2020 Jan 24 doi: 10.1308/rcsann.2019.0180. PMID: 31973562Free 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
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

Desmoplastic Small Round Cell Tumor With Ascending Intraspinal Metastasis at Recurrence: Case Report and Review of the Literature.
Cacciotti C, Samji N, Cox S, Yikilmaz A, Hann C, Marin JA, Fowler J, VanHouwelingen L, Athale U
J Pediatr Hematol Oncol 2022 Mar 1;44(2):e561-e566. doi: 10.1097/MPH.0000000000002138. PMID: 33710117
Abdominal desmoplastic small round cell tumor: multimodal treatment combining chemotherapy, surgery, and radiotherapy is the best option.
Honoré C, Amroun K, Vilcot L, Mir O, Domont J, Terrier P, Le Cesne A, Le Péchoux C, Bonvalot S
Ann Surg Oncol 2015 Apr;22(4):1073-9. Epub 2014 Oct 10 doi: 10.1245/s10434-014-4123-6. PMID: 25300608
Complete cytoreduction and HIPEC improves survival in desmoplastic small round cell tumor.
Hayes-Jordan A, Green HL, Lin H, Owusu-Agyemang P, Fitzgerald N, Arunkumar R, Mejia R, Okhuysen-Cawley R, Mauricio R, Fournier K, Ludwig J, Anderson P
Ann Surg Oncol 2014 Jan;21(1):220-4. Epub 2013 Sep 18 doi: 10.1245/s10434-013-3269-y. PMID: 24046124
Clinical perspective on desmoplastic small round-cell tumor.
Gil A, Gomez Portilla A, Brun EA, Sugarbaker PH
Oncology 2004;67(3-4):231-42. doi: 10.1159/000081323. PMID: 15557784
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

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