Management of a rapidly enlarging new adnexal mass: a rare case of desmoplastic small round cell tumor of the ovary arising in pregnancy

BACKGROUND: Desmoplastic small round cell tumor (DSRCT) is an extremely rare sarcomatous tumor, which is most commonly seen in men. Clinicians managing a patient with a rapidly enlarging mass in pregnancy should be aware of the risk for malignancy. CASE: A 31-year-old woman was found to have a newly enlarged ovarian mass in the second trimester. She subsequently underwent a laparotomy for removal, with chemotherapy for presumed poorly differentiated ovarian malignancy. Ultimately she was diagnosed with a desmoplastic small round cell tumor of the ovary and had progression at time of delivery. Following cesarean delivery, she had a tumor reductive surgery. She has completed 12 cycles of intensive chemotherapy and is alive with disease at 14 months. CONCLUSION: Care should be taken not to delay evaluation of a rapidly enlarging mass in pregnancy. While this tumor type is extremely rare, a malignancy in pregnancy must be ruled out in this clinical scenario.