# Multifocal Desmoplastic Small Round Cell Tumor: A Case Report of a Rare Neoplasm

## TL;DR

This case report describes a rare and aggressive cancer called desmoplastic small round cell tumor in an 11-year-old boy with unusual symptoms and a poor outcome.

## Contribution

The report highlights a rare multifocal presentation of DSRCT with orbital involvement in a pediatric patient.

## Key findings

- The patient had widespread lesions in the skull, orbit, thorax, and abdomen.
- Orbital DSRCT is extremely rare, with fewer than 10 documented cases.
- The patient's condition led to neutropenic sepsis and renal failure despite treatment.

## Abstract

Desmoplastic small round cell tumor (DSRCT) is a rare, highly aggressive malignancy predominantly affecting adolescents and young adults. We report a case of multifocal DSRCT in an 11-year-old male who presented with complaints of unilateral forehead swelling, proptosis, and ophthalmoplegia for four months along with abdominal pain and dysphagia for six months. A whole-body computed tomography revealed widespread lesions in the skull, orbit, thorax, and abdomen with local infiltration. Ultrasound-guided biopsy of the forehead lump was performed. Based on histopathological and immunohistochemical investigations, it was diagnosed to be a DSRCT with multifocal presentation. The patient underwent chemo-radiation but unfortunately succumbed to neutropenic sepsis and renal failure. DSRCT is a very rare, highly aggressive malignancy with an extremely poor prognosis. Orbital presentations are even rarer, with less than 10 such cases currently described in English medical literature.