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Desmoplastic small round cell tumor of the abdomen: A case report

RATIONALE: Desmoplastic small round cell tumor (DSRCT) is a rare malignant tumor with poor prognosis, usually involving the peritoneum. There are currently no standardized treatment approaches. This study helped to further advance our understanding of DSRCT, and help to guide therapy. PATIENT CONCER...

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Detalles Bibliográficos
Autores principales: Li, Runze, Liu, Weizhen, Ye, Lin
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Lippincott Williams & Wilkins 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10637438/
https://www.ncbi.nlm.nih.gov/pubmed/37960784
http://dx.doi.org/10.1097/MD.0000000000035965
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author Li, Runze
Liu, Weizhen
Ye, Lin
author_facet Li, Runze
Liu, Weizhen
Ye, Lin
author_sort Li, Runze
collection PubMed
description RATIONALE: Desmoplastic small round cell tumor (DSRCT) is a rare malignant tumor with poor prognosis, usually involving the peritoneum. There are currently no standardized treatment approaches. This study helped to further advance our understanding of DSRCT, and help to guide therapy. PATIENT CONCERNS: The patient, a 19-year-old male, presented with left-sided back pain with no obvious cause and occasional abdominal pain, and underwent abdominal electron computed tomography examination in our hospital suggesting consideration of small bowel mesenchymal tumor with possible multiple implantation metastasis in the abdominopelvic cavity. DIAGNOSES: After surgical treatment, the pathology report suggested a DSRCT, and immunohistochemistry and fluorescence in situ hybridization revealed EWSR1-WT1 gene rearrangement. Lung computer tomography and abdominal magnetic resonance imaging performed half a month later showed multiple solid nodules on the proximal septal surface of the right lung base, right posterior cardiac/right anterior inferior vena cava nodules, and multiple nodules in the abdominopelvic cavity, omenta, peritoneum, and around the liver or liver, all of which were considered as metastatic foci. INTERVENTIONS AND OUTCOMES: Patient received 5 cycles of chemotherapy after surgery. The review results showed a smaller size than before. Currently, he continues to receive treatment. LESSONS: The reported case has raised awareness of the importance of DSRCT in the treatment of chemotherapy, including its role in the differential diagnosis of abdominal tumors.
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spelling pubmed-106374382023-11-15 Desmoplastic small round cell tumor of the abdomen: A case report Li, Runze Liu, Weizhen Ye, Lin Medicine (Baltimore) 4500 RATIONALE: Desmoplastic small round cell tumor (DSRCT) is a rare malignant tumor with poor prognosis, usually involving the peritoneum. There are currently no standardized treatment approaches. This study helped to further advance our understanding of DSRCT, and help to guide therapy. PATIENT CONCERNS: The patient, a 19-year-old male, presented with left-sided back pain with no obvious cause and occasional abdominal pain, and underwent abdominal electron computed tomography examination in our hospital suggesting consideration of small bowel mesenchymal tumor with possible multiple implantation metastasis in the abdominopelvic cavity. DIAGNOSES: After surgical treatment, the pathology report suggested a DSRCT, and immunohistochemistry and fluorescence in situ hybridization revealed EWSR1-WT1 gene rearrangement. Lung computer tomography and abdominal magnetic resonance imaging performed half a month later showed multiple solid nodules on the proximal septal surface of the right lung base, right posterior cardiac/right anterior inferior vena cava nodules, and multiple nodules in the abdominopelvic cavity, omenta, peritoneum, and around the liver or liver, all of which were considered as metastatic foci. INTERVENTIONS AND OUTCOMES: Patient received 5 cycles of chemotherapy after surgery. The review results showed a smaller size than before. Currently, he continues to receive treatment. LESSONS: The reported case has raised awareness of the importance of DSRCT in the treatment of chemotherapy, including its role in the differential diagnosis of abdominal tumors. Lippincott Williams & Wilkins 2023-11-10 /pmc/articles/PMC10637438/ /pubmed/37960784 http://dx.doi.org/10.1097/MD.0000000000035965 Text en Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc. https://creativecommons.org/licenses/by/4.0/This is an open access article distributed under the Creative Commons Attribution License 4.0 (CCBY) (https://creativecommons.org/licenses/by/4.0/) , which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
spellingShingle 4500
Li, Runze
Liu, Weizhen
Ye, Lin
Desmoplastic small round cell tumor of the abdomen: A case report
title Desmoplastic small round cell tumor of the abdomen: A case report
title_full Desmoplastic small round cell tumor of the abdomen: A case report
title_fullStr Desmoplastic small round cell tumor of the abdomen: A case report
title_full_unstemmed Desmoplastic small round cell tumor of the abdomen: A case report
title_short Desmoplastic small round cell tumor of the abdomen: A case report
title_sort desmoplastic small round cell tumor of the abdomen: a case report
topic 4500
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10637438/
https://www.ncbi.nlm.nih.gov/pubmed/37960784
http://dx.doi.org/10.1097/MD.0000000000035965
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