Desmoplastic Small Round Cell Tumor

Common Name(s)

Desmoplastic Small Round Cell Tumor

Desmoplastic small round cell tumors (DSRCT), a rare malignant cancer, is a soft tissue sarcoma that usually affects young boys and men and is found most often in the abdomen. Its name means that it is formed by small, round cancer cells surrounded by scarlike tissue. The most common symptoms include abdominal pain, abdominal mass and symptoms of gastrointestinal obstruction.  DSRCTs are treated first with chemotherapy, then with surgery to remove the tumor, if possible. Radiation therapy is sometimes given, depending on the tumor. In addition, some people with DSRCT are candidates for a bone marrow transplant.
 

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Advocacy and Support Organizations

 

Condition Specific Organizations

Following organizations serve the condition "Desmoplastic Small Round Cell Tumor" for support, advocacy or research.

There are currently no organizations listed in Disease InfoSearch that support this condition. Create a listing.

 

 

General Support Organizations

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Scientific Literature

Articles from the PubMed Database

Research articles describe the outcome of a single study. They are the published results of original research.
The terms "Desmoplastic Small Round Cell Tumor" returned 45 free, full-text research articles on human participants. First 3 results:

Abdominal desmoplastic small round cell tumor without extraperitoneal metastases: Is there a benefit for HIPEC after macroscopically complete cytoreductive surgery?
 

Author(s): C Honoré, V Atallah, O Mir, D Orbach, G Ferron, C LePéchoux, J B Delhorme, P Philippe-Chomette, S Sarnacki, S Msika, P Terrier, O Glehen, H Martelli, V Minard-Colin, F Bertucci, J Y Blay, S Bonvalot, D Elias, A LeCesne, P Sargos,

Journal:

 

Desmoplastic Small Round Cell Tumor (DSRCT) is a rare disease affecting predominantly children and young adults and for which the benefit of hyperthermic intraperitoneal chemotherapy (HIPEC) after complete cytoreductive surgery (CCRS) remains unknown.

Last Updated: 24 Feb 2017

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Desmoplastic Small Round Cell Tumor of the Pleura Successfully Treated with a Lower Dose of Pazopanib.
 

Author(s): Tatsuyoshi Ikeue, Issei Ohi, Susumu Noguchi, Akari Fukao, Satoshi Terashita, Sadao Horikawa, Takakazu Sugita

Journal: Intern. Med.. 2016 ;55(17):2463-7.

 

Desmoplastic small round cell tumor (DSRCT) is an aggressive mesenchymal tumor which primarily affects the abdomen. Even a multimodal approach rarely achieves durable remission and the optimal therapy for extended disease is unknown. We herein describe a rare case of DSRCT arising ...

Last Updated: 1 Sep 2016

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Desmoplastic small round cell tumor: Diagnostic dilemma and uncertain prognosis: Report of few cases.
 

Author(s): Palash Kumar Mandal, Anindya Adhikari, Anuradha De, Santosh Kumar Mondal

Journal: J Cancer Res Ther. ;11(4):1028.

 

Desmoplastic small round cell tumor (DSRCT) is rare and highly malignant neoplasm. DSRCT affects usually young males but can occur in adults also. Intra-abdominal pelvic region is the preferred site. Though confirmed by histology, immunohistochemistry (IHC) plays a key role in diagnosis. ...

Last Updated: 17 Feb 2016

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Reviews from the PubMed Database

Review articles summarize what is currently known about a disease. They discuss research previously published by others.
The terms "Desmoplastic Small Round Cell Tumor" returned 7 free, full-text review articles on human participants. First 3 results:

Management of desmoplastic small round cell tumor.
 

Author(s): Andrea Hayes-Jordan, Michael P LaQuaglia, Shakeel Modak

Journal: Semin. Pediatr. Surg.. 2016 Oct;25(5):299-304.

 

Desmoplastic small round cell tumor (DSRCT) is a soft tissue sarcoma of mesenchymal cell origin that typically presents with multiple intra-abdominal tumors and exhibits a multi-phenotypic pattern of immunohistochemical staining. The specific organ or tissue type of origin has yet ...

Last Updated: 13 Dec 2016

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Testicular desmoplastic small round cell tumor: a case report and review of literature.
 

Author(s): Gui-Ming Zhang, Yao Zhu, Hua-Lei Gan, Ding-Wei Ye

Journal:

 

Desmoplastic small round cell tumor (DSRCT) is an uncommon and highly aggressive malignancy with undetermined histogenesis and poor prognosis. To date, no case of testicular DSRCT has been reported in the literature.

Last Updated: 23 Jul 2014

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Fine-needle aspiration in desmoplastic small round cell tumor: a report of 10 new tumors in 8 patients with clinicopathological and molecular correlations with review of the literature.
 

Author(s): Jerzy Klijanienko, Pierre Colin, Jérôme Couturier, Réal Lagacé, Paul Fréneaux, Gaëlle Pierron, Marick Laé, Alice Klijanienko, Hervé Brisse, Daniel Orbach, Stamatios Theocharis

Journal: Cancer Cytopathol. 2014 May;122(5):386-93.

 

Desmoplastic small round cell tumor (DSRCT) is a rare round cell sarcoma entity characterized by a specific t(11;22)(p13;q12) translocation, usually intra-abdominal localization and an aggressive clinical outcome. To date, only 35 DSRCT cases diagnosed by fine-needle aspiration have been described.

Last Updated: 14 May 2014

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Trial of Nab-paclitaxel in Patients With Desmoid Tumors and Multiply Relapsed/Refractory Desmoplastic Small Round Cell Tumors and Ewing Sarcoma
 

Status: Recruiting

Condition Summary: Tumor, Desmoplastic Small Round Cell, Adult; Tumor, Desmoplastic Small Round Cell, Childhood; Sarcoma, Ewing; Sarcoma; Desmoid

 

Last Updated: 6 Sep 2017

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Activated T Cells Armed With GD2 Bispecific Antibody in Children and Young Adults With Neuroblastoma and Osteosarcoma
 

Status: Recruiting

Condition Summary: Desmoplastic Small Round Cell Tumor; Disseminated Neuroblastoma; Metastatic Osteosarcoma; Recurrent Neuroblastoma; Recurrent Osteosarcoma

 

Last Updated: 17 Aug 2017

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