Key clinical message: Desmoplastic round cell tumor, though rare, must be taken into consideration as a differential diagnosis, thus aiding in early evaluation and changing the trajectory of the natural history of the disease condition, and improving the prognosis of patients.
Abstract: Desmoplastic small round cell tumor is a rare, aggressive tumor of mesenchymal origin with an incidence of 0.74 cases per million. We present a young adult with a periumbilical mass who was diagnosed as a desmoplastic round cell tumor and later was treated with exploratory laparotomy and resection of the tumor with no recurrence during a 6-month follow-up period.
Keywords: case report; desmoplastic round cell tumor; mesenchymal; omentum.
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