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Case Report
Desmoplastic small round cell tumor of the pleura with brain metastasis: A case report and literature review
1 MD, Anatomical Pathology resident, Department of Laboratory Medicine and Pathology, University of Alberta Hospital, Edmonton, Alberta, Canada
2 MD, FRCPC, Neuropathologist, Department of Laboratory Medicine and Pathology, University of Alberta Hospital, Edmonton, Alberta, Canada
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Rebecca Phillips
Department of Laboratory Medicine and Pathology, University of Alberta, 5B2.19, Walter C. Mackenzie Centre, 8440-112 Street NW, Edmonton, Alberta T6G 2B7,
Canada
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Article ID: 100074Z11RP2023
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Phillips R, Das S. Desmoplastic small round cell tumor of the pleura with brain metastasis: A case report and literature review. J Case Rep Images Pathol 2023;9(2):5–12.ABSTRACT
Introduction: Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive mesenchymal neoplasm characterized by a chromosomal translocation involving the EWSR1 and WT1 genes. It typically arises in intra-abdominal tissues (i.e., mesentery, omentum, pelvic organs), and commonly spreads to regional lymph nodes, lungs, or liver, while central nervous system (CNS) metastasis is rare.
Case Report: We present a unique case of DSRCT originating from the pleura in a young female, which was metastatic to intrathoracic and mediastinal tissue at diagnosis and later spread to the brain. The initial tumor samples exhibited characteristic histopathological features, including small round cells with minimal cytoplasm and indistinct borders embedded in a fibroblastic stroma. Notably, the brain metastasis demonstrated distinct histological characteristics, lacking the desmoplastic stroma observed in previous biopsies.
Conclusion: This case underscores the diagnostic challenges associated with DSRCT, emphasizes the significance of early recognition and appropriate histological evaluation, and contributes to the limited literature on the histological features and metastatic behavior of this rare tumor.
Keywords: Brain, Central nervous system, Desmoplastic small round cell tumor, DSRCT, EWSR1
SUPPORTING INFORMATION
Author Contributions
Rebecca Phillips - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Interpretation of data, Drafting the article, Final approval of the version to be published
Sumit Das - Substantial contributions to conception and design, Acquisition of data, Analysis of data, Drafting the article, Revising it critically for important intellectual content, Final approval of the version to be published
Guarantor of SubmissionThe corresponding author is the guarantor of submission.
Source of SupportNone
Consent StatementWritten informed consent was obtained from the patient for publication of this article.
Data AvailabilityAll relevant data are within the paper and its Supporting Information files.
Conflict of InterestAuthors declare no conflict of interest.
Copyright© 2023 Rebecca Phillips et al. This article is distributed under the terms of Creative Commons Attribution License which permits unrestricted use, distribution and reproduction in any medium provided the original author(s) and original publisher are properly credited. Please see the copyright policy on the journal website for more information.
