Desmoplastic Small Round Cell Tumor of the Spinal Canal: A Rare Presentation in a Female Pediatric Patient

Abstract Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive malignancy primarily affecting young individuals, typically originating in the abdominal cavity. While spinal involvement by DSRCT is exceedingly rare, especially in pediatric patients, recognizing such atypical presentations is crucial for optimal management. Here, we present a case of DSRCT involving the spinal canal in a 3-year-old female child, representing one of the youngest documented instances in the literature. Initial symptoms included weakness, lower limb pain, and urinary incontinence. Magnetic resonance imaging revealed an enhancing soft tissue lesion in the spinal canal, prompting tumor excision and histopathological examination. The tumor exhibited characteristic features of DSRCT, including small round cells within a desmoplastic stroma. Immunohistochemistry confirmed the diagnosis, showing positivity for Wilms tumor 1, epithelial membrane antigen, desmin, and synaptophysin. This case highlights the challenges in diagnosing and managing rare tumors like spinal DSRCT, emphasizing the importance of a comprehensive approach to differential diagnosis and individualized treatment strategies.