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Giant desmoplastic small round cell tumor of the abdomen: A case report

BACKGROUND: Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive, mesenchymal malignancy of a separate clinicopathological entity. It has a predilection for young men, with no evidence of any ethnic predilection. The current diagnostic gold standard for DSRCT includes histopathologic, i...

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Autores principales: Wang, Wuke, Chen, Yunjie, Wang, Chunnian, Su, Hui
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Frontiers Media S.A. 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9723381/
https://www.ncbi.nlm.nih.gov/pubmed/36483026
http://dx.doi.org/10.3389/fonc.2022.992346
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author Wang, Wuke
Chen, Yunjie
Wang, Chunnian
Su, Hui
author_facet Wang, Wuke
Chen, Yunjie
Wang, Chunnian
Su, Hui
author_sort Wang, Wuke
collection PubMed
description BACKGROUND: Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive, mesenchymal malignancy of a separate clinicopathological entity. It has a predilection for young men, with no evidence of any ethnic predilection. The current diagnostic gold standard for DSRCT includes histopathologic, immunohistochemical, and cytogenetic studies in order to confirm the variable phenotypic expression and characteristic chromosomal translocation. CASE SUMMARY: A 65-year-old man presented with a sensation of an abdominal mass and a presentation of an incomplete bowel obstruction. Initial lab tests were in the normal range except for carbohydrate antigen. Contrast-enhanced CT showed that a large, mass-confounding density was occupied in the omentum majus area of the middle and lower abdominal wall. A 3D reconstruction of the images was performed to clarify the relationship between the tumor and the colon and was confirmed by a colonoscopy. After surgery, immunohistochemistry and fluorescence in situ hybridization (FISH) revealed EWSR1-WT1 gene rearrangement at 22q12, confirming the diagnosis of desmoplastic small round cell tumor. CONCLUSION: Being different from the predilection of DSRCT for young men, the patient in our case is a 65-year-old man with a huge mass involving the transverse colon and the bladder.
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spelling pubmed-97233812022-12-07 Giant desmoplastic small round cell tumor of the abdomen: A case report Wang, Wuke Chen, Yunjie Wang, Chunnian Su, Hui Front Oncol Oncology BACKGROUND: Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive, mesenchymal malignancy of a separate clinicopathological entity. It has a predilection for young men, with no evidence of any ethnic predilection. The current diagnostic gold standard for DSRCT includes histopathologic, immunohistochemical, and cytogenetic studies in order to confirm the variable phenotypic expression and characteristic chromosomal translocation. CASE SUMMARY: A 65-year-old man presented with a sensation of an abdominal mass and a presentation of an incomplete bowel obstruction. Initial lab tests were in the normal range except for carbohydrate antigen. Contrast-enhanced CT showed that a large, mass-confounding density was occupied in the omentum majus area of the middle and lower abdominal wall. A 3D reconstruction of the images was performed to clarify the relationship between the tumor and the colon and was confirmed by a colonoscopy. After surgery, immunohistochemistry and fluorescence in situ hybridization (FISH) revealed EWSR1-WT1 gene rearrangement at 22q12, confirming the diagnosis of desmoplastic small round cell tumor. CONCLUSION: Being different from the predilection of DSRCT for young men, the patient in our case is a 65-year-old man with a huge mass involving the transverse colon and the bladder. Frontiers Media S.A. 2022-11-22 /pmc/articles/PMC9723381/ /pubmed/36483026 http://dx.doi.org/10.3389/fonc.2022.992346 Text en Copyright © 2022 Wang, Chen, Wang and Su https://creativecommons.org/licenses/by/4.0/This is an open-access article distributed under the terms of the Creative Commons Attribution License (CC BY). The use, distribution or reproduction in other forums is permitted, provided the original author(s) and the copyright owner(s) are credited and that the original publication in this journal is cited, in accordance with accepted academic practice. No use, distribution or reproduction is permitted which does not comply with these terms.
spellingShingle Oncology
Wang, Wuke
Chen, Yunjie
Wang, Chunnian
Su, Hui
Giant desmoplastic small round cell tumor of the abdomen: A case report
title Giant desmoplastic small round cell tumor of the abdomen: A case report
title_full Giant desmoplastic small round cell tumor of the abdomen: A case report
title_fullStr Giant desmoplastic small round cell tumor of the abdomen: A case report
title_full_unstemmed Giant desmoplastic small round cell tumor of the abdomen: A case report
title_short Giant desmoplastic small round cell tumor of the abdomen: A case report
title_sort giant desmoplastic small round cell tumor of the abdomen: a case report
topic Oncology
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9723381/
https://www.ncbi.nlm.nih.gov/pubmed/36483026
http://dx.doi.org/10.3389/fonc.2022.992346
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