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Metastatic Desmoplastic Small Round Cell Tumor

MedGen UID:
1753135
•Concept ID:
C5418824
•
Neoplastic Process

Definition

A desmoplastic small round cell tumor that has spread from the original site of growth to another anatomic site. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMetastatic 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
Desmoplastic Small Round Cell Tumors: A review with focus on clinical management and therapeutic options.
Hendricks A, Boerner K, Germer CT, Wiegering A
Cancer Treat Rev 2021 Feb;93:102140. Epub 2020 Dec 24 doi: 10.1016/j.ctrv.2020.102140. PMID: 33388539
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

Diagnosis

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
Disseminated intrathoracic desmoplastic small round-cell tumor: a case report.
Venkateswaran L, Jenkins JJ, Kaste SC, Shurtleff SA, Downing JR, Pappo AS
J Pediatr Hematol Oncol 1997 Mar-Apr;19(2):172-5. doi: 10.1097/00043426-199703000-00017. PMID: 9149753

Therapy

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
Temsirolimus for metastatic desmoplastic small round cell tumor.
Thijs AM, van der Graaf WT, van Herpen CM
Pediatr Blood Cancer 2010 Dec 15;55(7):1431-2. doi: 10.1002/pbc.22755. PMID: 20730888

Prognosis

Pulmonary tumor thrombotic microangiopathy with cor pulmonale due to desmoplastic small round cell tumor.
Sadimin ET, Collier AG, Gaffney JW, Fyfe B
J Pediatr 2012 Apr;160(4):697-9. Epub 2012 Jan 10 doi: 10.1016/j.jpeds.2011.12.010. PMID: 22240106
Disseminated intrathoracic desmoplastic small round-cell tumor: a case report.
Venkateswaran L, Jenkins JJ, Kaste SC, Shurtleff SA, Downing JR, Pappo AS
J Pediatr Hematol Oncol 1997 Mar-Apr;19(2):172-5. doi: 10.1097/00043426-199703000-00017. PMID: 9149753

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