Desmoplastic small round cell tumor involving the uterine cervix: The first reported case in the literature, and brief review of gynecologic presentations

BACKGROUND: Desmoplastic small round cell tumors are exceedingly rare, usually involve abdominal organs and predominantly affect male patients. We describe the first reported case arising from the uterine cervix and provide a summary of 20 previously reported cases involving gynecologic organs. CASE: A 54 year-old was diagnosed with a rapidly growing 13 cm desmoplastic small round cell tumor of the cervix. She was treated through a multimodal approach involving neoadjuvant chemotherapy and surgery. She subsequently recurred, and this was successfully treated with radiation therapy. She is well and without evidence of disease 22 months after initial diagnosis. CONCLUSION: We report successful treatment through multidisciplinary and multimodal management. This can guide management of future patients as no gold-standard treatment has yet been described.