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

TL;DR

This paper reports two cases of a rare tumor in young boys, highlighting how imaging can help in diagnosis despite the disease's poor prognosis.

Contribution

The novelty lies in emphasizing specific imaging findings in two rare pediatric cases of desmoplastic small round cell tumor.

Findings

Imaging findings can help narrow down the diagnosis of DSRCT, even though they are not definitive.

Two cases of DSRCT in young boys were presented with a focus on imaging characteristics.

Despite various treatments, the prognosis for DSRCT remains poor.

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.

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