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

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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Desmoplastic small round cell tumor of the abdomen and pelvis: clinicopathological characters of 12 cases.
 

Author(s): Guangzhao Zhang, Guangjun Liu, Dahua Zhao, Xijun Cui, Gang Li

Journal: ScientificWorldJournal. 2014 ;2014():549612.

 

To study the clinical, radiological, and pathological characteristics of abdominal desmoplastic small round cell tumor (DSRCT) and investigate the optimal therapy modalities.

Last Updated: 2 Jul 2014

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Clinical and computed tomography features of adult abdominopelvic desmoplastic small round cell tumor.
 

Author(s): Xun-Ze Shen, Jian-Guo Zhao, Jian-Jun Wu, Fang Liu

Journal: World J. Gastroenterol.. 2014 May;20(17):5157-64.

 

To investigate the clinical and computed tomography (CT) features of desmoplastic small round cell tumor (DSRCT), we retrospectively analyzed the clinical presentations, treatment and outcome, as well as CT manifestations of four cases of DSRCT confirmed by surgery and pathology. ...

Last Updated: 7 May 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 2 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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Desmoplastic small round cell tumor: a case report and review of the literature.
 

Author(s): Xiang Li, Jing Yu, Shibao Fang, Xiaoming Xing, Jie Zhao

Journal:

 

Desmoplastic small round cell tumor is a rare malignant tumor that has a poor prognosis. It affects predominantly young males. In the current report, a 14-year-old male patient was admitted to the hospital for evaluation of abdominal distension, and abdominal pain. Imaging examination ...

Last Updated: 8 Apr 2014

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Symptoms, Diagnosis, and Treatment

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Clinical Trial Information This information is provided by ClinicalTrials.gov

Therapeutic Trial for Patients With Ewing Sarcoma Family of Tumor and Desmoplastic Small Round Cell Tumors
 

Status: Recruiting

Condition Summary: Desmoplastic Small Round Cell Tumor; Ewing Sarcoma of Bone or Soft Tissue; Localized Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor; Metastatic Ewing Sarcoma/Peripheral Primitive Neuroectodermal Tumor

 

Last Updated: 4 Feb 2015

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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 Chondrosarcoma; Adult Extraskeletal Osteosarcoma; Adult Fibrosarcoma; Adult Leiomyosarcoma; Adult Liposarcoma; Adult Malignant Fibrous Histiocytoma; Adult Malignant Hemangiopericytoma; Adult Malignant Mesenchymoma; Adult Neurofibrosarcoma; Adult Rhabdomyosarcoma; Adult Synovial Sarcoma; Childhood Alveolar Soft-part Sarcoma; Childhood Angiosarcoma; Childhood Desmoplastic Small Round Cell Tumor; Childhood Epithelioid Hemangioendothelioma; Childhood Epithelioid Sarcoma; Childhood Fibrosarcoma; Childhood Leiomyosarcoma; Childhood Liposarcoma; Childhood Malignant Hemangiopericytoma; Childhood Malignant Mesenchymoma; Childhood Neurofibrosarcoma; Childhood Synovial Sarcoma; Dermatofibrosarcoma Protuberans; Metastatic Childhood Soft Tissue Sarcoma; Nonmetastatic Childhood Soft Tissue Sarcoma; Recurrent Adult Soft Tissue Sarcoma; Recurrent Childhood Soft Tissue Sarcoma; Stage III Adult Soft Tissue Sarcoma; Stage IV Adult Soft Tissue Sarcoma

 

Last Updated: 5 Jan 2015

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