Imaging Diagnosis of Desmoplastic Small Round Cell Tumor: A Report of Two Cases

Cureus. 2024 Apr 11;16(4):e58037. doi: 10.7759/cureus.58037. eCollection 2024 Apr.

Abstract

Desmoplastic small round cell tumor (DSRCT) is a rare multifocal peritoneal sarcoma, typically found in adolescent and young adult males. Symptoms are nonspecific and vary depending on tumor involvement. Diagnosis is primarily histopathological, although imaging results can assist in the diagnostic process. Although not pathognomonic, certain radiologic findings can help narrow down potential diagnoses and sometimes suggest the condition, as seen in our cases. Treatment options are not well-established or effective, and despite employing various therapeutic approaches, the prognosis remains poor. We present two cases of boys aged 11 and 10 with a final diagnosis of DSRCT, emphasizing the imaging findings.

Keywords: cross-sectional imaging; desmoplastic small round blue cell tumours; imaging diagnosis; intra-abdominal neoplasms; peritoneal malignancy; soft-tissue sarcoma.

Publication types

  • Case Reports