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 44 free, full-text research articles on human participants. First 3 results:

Beware of immunohistochemistry--report of a cytokeratin-, desmin- and INI-1-negative pelvic desmoplastic small round cell tumor in a 51 year old woman.
 

Author(s): Gwyneth St Soon, Fredrik Petersson

Journal:

 

We present a 51 year old female patient with a pelvic desmoplastic small round cell tumor with an unusual immunohistochemical profile, including absence of significant cytokeratin expression, complete negativity for desmin and widespread loss of nuclear INI-1 expression (>90% of tumor ...

Last Updated: 10 Mar 2015

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Primary abdominopelvic desmoplastic small round cell tumor: CT and correlated clinicopathologic features.
 

Author(s): G Li, H-T Wang, Y Gao, X-J Cui, G-Z Zhang

Journal: Eur Rev Med Pharmacol Sci. 2014 ;18(18):2670-7.

 

To evaluate the CT characteristics of primary abdominopelvic desmoplastic small round cell tumor (DSCRT) and investigate the relation between radiologic features and corresponding clinicopathologic features.

Last Updated: 16 Oct 2014

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Primary desmoplastic small round cell tumor of the duodenum.
 

Author(s): Qi Liu, Nan Liu, DeXing Chen

Journal:

 

The desmoplastic small round cell tumor (DSRCT) is an extremely rare tumor that mainly affects adolescents and mostly involves the abdominal and pelvic peritoneum. A 14-year-old girl presented with intermittent epigastric pain; abdominal computed tomography and upper gastrointestinal ...

Last Updated: 15 Jul 2014

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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 5 free, full-text review articles on human participants. First 3 results:

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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Misdiagnosed desmoplastic small round cell tumor of the pleura: case report and literature review.
 

Author(s): Zhang Jian, Huang Shaohong, Zhong Wenzhao, Gu Lijia

Journal: J. Formos. Med. Assoc.. 2014 Jan;113(1):60-1.

 

Last Updated: 21 Jan 2014

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Symptoms, Diagnosis, and Treatment

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Understanding Communication in Healthcare to Achieve Trust (U-CHAT)
 

Status: Recruiting

Condition Summary: Neuroblastoma; Sarcoma; Carcinoma; Desmoplastic Small Round Cell Tumor; Retinoblastoma; Wilms Tumor; Melanoma

 

Last Updated: 26 Jul 2016

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Gemcitabine Hydrochloride With or Without Pazopanib Hydrochloride in Treating Patients With Refractory Soft Tissue Sarcoma
 

Status: Recruiting

Condition Summary: Adult Alveolar Soft Part Sarcoma; Adult Angiosarcoma; Adult Desmoplastic Small Round Cell Tumor; Adult Epithelioid Hemangioendothelioma; Adult Epithelioid Sarcoma; Adult Extraskeletal Myxoid Chondrosarcoma; Adult Extraskeletal Osteosarcoma; Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Mesenchymoma; Adult Malignant Peripheral Nerve Sheath Tumor; Adult Rhabdomyosarcoma; Adult Synovial Sarcoma; Adult Undifferentiated Pleomorphic Sarcoma; Malignant Adult Hemangiopericytoma; Recurrent Adult Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

 

Last Updated: 5 Apr 2016

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