Abdominal Desmoplastic Small Round Cell Tumour: Two Clinical Cases

Desmoplastic small round cell tumour (DSRCT) is a rare and highly aggressive neoplasm, typically diagnosed in adolescents and young adults. With fewer than 500 cases described worldwide, treatment is multimodal and challenging, involving intensive chemotherapy, cytoreductive surgery, and radiotherapy. We present two clinical cases of adolescents diagnosed with advanced abdominal DSRCT, exhibiting distinct clinical courses. Emphasis is placed on the role of whole abdominal radiotherapy in locoregional control within a multidisciplinary approach. Therapeutic options, associated complications, and clinical management challenges are discussed.

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Publication Details

Journal
Cureus
Published
2026-05-27
DOI
https://doi.org/10.7759/cureus.109746
Primary Topic
Sarcoma Diagnosis and Treatment
Type
article
Field-Weighted Citation Impact
0.00
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article

Abdominal Desmoplastic Small Round Cell Tumour: Two Clinical Cases

Cristiana Couto, Sofia Conde, João Vitor Silva, Miguel Catalo et al.
Cureus
Sarcoma Diagnosis and Treatment
article

Abdominal Desmoplastic Small Round Cell Tumour: Two Clinical Cases

Cristiana Couto, Sofia Conde, João Vitor Silva, Miguel Catalo, Mariana Afonso, Cátia Sousa, Catarina Macedo
article en

Abstract

Desmoplastic small round cell tumour (DSRCT) is a rare and highly aggressive neoplasm, typically diagnosed in adolescents and young adults. With fewer than 500 cases described worldwide, treatment is multimodal and challenging, involving intensive chemotherapy, cytoreductive surgery, and radiotherapy. We present two clinical cases of adolescents diagnosed with advanced abdominal DSRCT, exhibiting distinct clinical courses. Emphasis is placed on the role of whole abdominal radiotherapy in locoregional control within a multidisciplinary approach. Therapeutic options, associated complications, and clinical management challenges are discussed.

Cureus
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Openalex Percentile: Top 8%
Sarcoma Diagnosis and Treatment
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