Key result
A 32-year-old male was diagnosed with a rare desmoplastic small round cell tumor presenting as a large cystic abdominal mass with liver metastases, confirmed by immunohistochemistry.
Why the study?
Desmoplastic small round blue cell tumor is a very rare soft tissue sarcoma with diagnostic challenges, particularly when presenting without typical radiological findings.
Case Report (n=1)
Diagnosis of DSRCT is challenging and requires correlation of clinical, radiological, morphological, immunohistochemical, and genetic data, especially in atypical presentations.
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.
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Alfotih et al. (2021) conducted a case report in Desmoplastic small round cell tumor (n=1). Desmoplastic small round cell tumor was evaluated. A 32-year-old male was diagnosed with a rare desmoplastic small round cell tumor presenting as a large cystic abdominal mass with liver metastases, confirmed by immunohistochemistry.
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