Desmoplastic small round cell tumor of the ovary: A rare but devastating disease in young women
A 28-year-old female presented with one month of abdominal pain and pressure. CT showed multiple abdominopelvic masses, mesenteric lymph nodes, and a peri-splenic mass. CA-125 was 42 U/mL. Biopsy results were consistent with DSRCT, and she received 14 cycles of neoadjuvant IE/VDC with the removal of adriamycin after the eighth cycle due to cardiac toxicity. Following an incomplete response, the patient underwent an exploratory laparotomy. Intra-operative findings included a 10 × 8 cm right ovarian cystic mass, an 8 cm nodule between the stomach and the spleen and small implants in the cul-de-sac and omentum. A bilateral salpingo-oophorectomy, resection of cul-de-sac peritoneum, and splenectomy, and omentectomy were performed leaving her without visible disease. Microscopy showed cords of small round blue cells. Immunohistochemistry revealed strong and diffuse positivity for desmin and multifocal keratin positivity. CD99 was negative. Cytogenetics was negative for the EWS-WT1 translocation.\nShe returned on post-operative day 5 with a fascial dehiscence which was repaired without complication. The patient finished the last 2 cycles of her 16 cycle chemotherapy regimen and did well for 6 months. At that time, a CT showed increased abdominopelvic disease and a bone scan was consistent with metastasis. She was started on cytoxan/topotecan which she received from her local oncologist. The patient expired of her disease 40 months after her initial diagnosis.