Desmoplastic Small Round Cell Tumor
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Desmoplastic Small Round Cell Tumor Overview

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What is the definition of Desmoplastic Small Round Cell Tumor?
Desmoplastic small round cell tumor (DSRCT) is a rare type of soft tissue cancer (sarcoma) that usually begins in the abdomen. It primarily affects children and young adults and is more common in males. It is formed by small, round cancer cells surrounded by scar-like tissue and is often found in the tissue (peritoneum) that lines the inside of the abdomen and pelvis. The tumor cells have a characteristic genetic change involving a translocation between chromosomes 11 and 22, which is important in differentiating from other similar tumors. The genetic change involved in DSRCT is acquired throughout a person's lifetime and is not inherited. Symptoms may include abdominal pain and a feeling of fullness, abdominal mass, and symptoms of gastrointestinal obstruction, such as constipation.
What are the alternative names for Desmoplastic Small Round Cell Tumor?
  • Desmoplastic small round cell tumor
  • DSRCT
  • Desmoplastic small round-cell tumor
Who are the top Desmoplastic Small Round Cell Tumor Local Doctors?
Elite in Desmoplastic Small Round Cell Tumor
Elite in Desmoplastic Small Round Cell Tumor
Referral may be required
Milan, IT 

Andrea Ferrari practices practicing medicine in Milan, Italy. Ms. Ferrari is rated as an Elite expert by MediFind in the treatment of Desmoplastic Small Round Cell Tumor. She is also highly rated in 21 other conditions, according to our data. Her clinical expertise encompasses Rhabdomyosarcoma, Adult Soft Tissue Sarcoma, Desmoplastic Small Round Cell Tumor, Thyroidectomy, and Pancreatectomy.

Elite in Desmoplastic Small Round Cell Tumor
Elite in Desmoplastic Small Round Cell Tumor
Referral may be required

Rockefeller Outpatient Pavilion

160 E 53rd St, 
New York, NY 
929-379-4794
Languages Spoken:
English
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Offers Telehealth

Mark Dickson is an Oncologist practicing medicine in New York, New York. Dr. Dickson is rated as an Elite provider by MediFind in the treatment of Desmoplastic Small Round Cell Tumor. He is also highly rated in 31 other conditions, according to our data. His clinical expertise encompasses Adult Soft Tissue Sarcoma, Liposarcoma, Undifferentiated Pleomorphic Sarcoma, Synovial Sarcoma, and Endoscopy. Dr. Dickson is currently accepting new patients.

 
 
 
 
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Elite in Desmoplastic Small Round Cell Tumor
Elite in Desmoplastic Small Round Cell Tumor
Referral may be required
Milan, IT 

Silvia Stacchiotti practices practicing medicine in Milan, Italy. Ms. Stacchiotti is rated as an Elite expert by MediFind in the treatment of Desmoplastic Small Round Cell Tumor. She is also highly rated in 28 other conditions, according to our data. Her clinical expertise encompasses Solitary Fibrous Tumor, Adult Soft Tissue Sarcoma, Alveolar Soft Part Sarcoma, Hemangioendothelioma, and Synovectomy.

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What are the latest Desmoplastic Small Round Cell Tumor Clinical Trials?
Phase II Study of Lurbinectedin and Irinotecan in Adult and Young Adult Patients With Advanced Desmoplastic Small Round Cell Tumor (DSRCT)
Phase II Study of Lurbinectedin and Irinotecan in Adult and Young Adult Patients With Advanced Desmoplastic Small Round Cell Tumor (DSRCT)
Enrollment Status: Recruiting
Publish Date: June 11, 2026
Intervention Type: Drug
Study Phase: Phase 2

Summary: Patients participating in this study have DSRCT that has spread locally or to other parts of the body and can no longer be surgically removed without causing significant harm. Treatment will continue until the tumor progresses further, severe side effects occur, or either patient or investigator decision. In addition, patients may participate in an optional biological study. The study will analyze...

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ANGIO-A: Safety and Tolerability of Oral Cyclophosphamide and Sorafenib With Intravenous Bevacizumab With the Addition of Atezolizumab in Pediatric Solid Tumor Patients
ANGIO-A: Safety and Tolerability of Oral Cyclophosphamide and Sorafenib With Intravenous Bevacizumab With the Addition of Atezolizumab in Pediatric Solid Tumor Patients
Enrollment Status: Recruiting
Publish Date: May 19, 2026
Intervention Type: Drug
Study Phase: Phase 1/Phase 2

Summary: This is a phase I/II study to evaluate the safety of combining intravenous (IV) atezolizumab and bevacizumab every three weeks, with daily oral cyclophosphamide and pharmacokinetic (PK)-guided sorafenib in children and adolescent and young adults (AYA) with relapsed or refractory solid malignancies (Part 1), and then evaluate the response rate of this combination in children, AYA with relapsed or ...

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Who are the sources who wrote this article ?

Published Date: May 02, 2022
Published By: Genetic and Rare Diseases Informnation Center