Case Report


Desmoplastic small round cell tumor of abdomen: Case report of a very rare sarcoma with diagnostic challenge

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1 Consultant Medical Oncology, Adult Medical Oncology Department, King Fahad Hospital, Medina, Saudi Arabia

2 Assistant Professor of Molecular Pathology, Clinical Laboratory Science Department, Faculty of Applied Medical Science, Taibah University, Medina, Saudi Arabia

3 Assistant Professor, Faculty of Medicine, Taibah University, Medina, Saudi Arabia

4 Consultant Medical Oncology, Adult Oncology Department, King Salman Specialist Hospital, Hail, Saudi Arabia

Address correspondence to:

Samir Alfotih

King Fahad Hospital, Medina,

Saudi Arabia

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Article ID: 100084Z10SA2021

doi: 10.5348/100084Z10SA2021CR

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How to cite this article

Alfotih S, Zahid H, Mulla N, Wazir KB. Desmoplastic small round cell tumor of abdomen: Case report of a very rare sarcoma with diagnostic challenge. J Case Rep Images Oncology 2021;7:100084Z10SA2021.

ABSTRACT


Introduction: A desmoplastic small round blue cell tumor (DSRCT) is a very rare soft tissue sarcoma. The first case was reported in 1989 by Gerald and Rosai. Diagnosis can be challenging, and a differential diagnosis should be considered when a young patient presents with wide-spread abdominal disease without definitive organ involvement as shown in imaging studies. A typical radiological finding in DSRCT is a dominant solid mass in a retrovesical or rectouterine pouch with widespread peritoneal nodules.

Case Report: In this case report, we present a case of a very rare sarcoma in a young Saudi male patient who presented with abdominal distension and widespread intra-abdominal disease with liver metastases. The dominant mass had a cystic component and was not in a typical site.

Conclusion: Diagnosis of such sarcoma is challenging and it needs the correlation of clinical, radiological, morphological, immunohistochemical, and even genetic data.

Keywords: Desmoplastic small round cell tumor, Diagnosis, Radiological finding

SUPPORTING INFORMATION


Author Contributions

Samir Alfotih - Acquisition of data, Analysis of data, Interpretation of data, Final approval of the version to be published

Heba Zahid - Acquisition of data, Revising it critically for important intellectual content, Final approval of the version to be published

Nasser Mulla - Acquisition of data, Revising it critically for important intellectual content, Final approval of the version to be published

Khaled Ba Wazi - Acquisition of data, Revising it critically for important intellectual content, Final approval of the version to be published

Guaranter of Submission

The corresponding author is the guarantor of submission.

Source of Support

None

Consent Statement

Written informed consent was obtained from the patient for publication of this article.

Data Availability

All relevant data are within the paper and its Supporting Information files.

Conflict of Interest

Authors declare no conflict of interest.

Copyright

© 2021 Samir Alfotih 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.