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

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.