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Intra-abdominal desmoplastic small round cell tumor in a 45-year-old man: a case report
Mandana Ashrafi1, Hiva Saffar, Seyed Rasoul Mirsharifi
1Department of Pathology, Tehran University of Medical Sciences, Tehran, Iran. Tavangar@ams.ac.ir.
Abstract:
Intra-abdominal desmoplastic small round cell tumor is a rare entity with a few reports worldwide. This tumor commonly occurs in children and adolescent and occurrence in adult age group is very rare. Here, we describe an adult male with symptoms of intestinal obstruction due to abdominal mass, located in splenic flexure. After resection, the diagnosis of intra-abdominal desmoplastic small round cell tumor was established by pathologic and immunohistochemical studies. We emphasize that albeit being rare, this tumor should be mentioned as one of the differential diagnoses of widespread intra-abdominal lesions in any age group. A brief review of epidemiology, clinical manifestations as well as pathological and molecular features is also included in the paper.
Insights
Intra-abdominal desmoplastic small round cell tumors are rare, typically affecting children. This case highlights a rare adult occurrence, emphasizing its consideration in adult abdominal masses.
Area of Science:
- Oncology
- Pathology
Background:
- Desmoplastic small round cell tumor (DSRCT) is an exceptionally rare malignancy.
- Primarily diagnosed in pediatric and adolescent males, adult cases are exceedingly uncommon.
Observation:
- A rare case of intra-abdominal desmoplastic small round cell tumor presented in an adult male.
- The patient exhibited symptoms of intestinal obstruction caused by an abdominal mass in the splenic flexure.
Findings:
- Pathologic and immunohistochemical analyses confirmed the diagnosis of intra-abdominal desmoplastic small round cell tumor post-resection.
- The tumor's presence in an adult male underscores its potential, though rare, occurrence across age groups.
Implications:
- This case broadens the differential diagnosis for intra-abdominal lesions, even in adult patients.
- Highlights the importance of considering rare tumors in adult oncology, irrespective of typical age demographics.