Background: Atypical Meigs' syndrome has been observed in patients with dermoid tumors, struma ovarii, uterine leiomyomata and other benign pelvic tumors except for ovarian fibromas. Meigs' and atypical Meigs' syndrome present management decisions complicated by a high index of suspicion for malignancy.
Case: A 43-year-old woman, gravida 1, para 1, with ascites; a pleural effusion; radiologic evidence of enlarged, cystic adnexa; and a normal CA-125 level was found to have cortical stromal hyperplasia on bilateral ovarian pathologic evaluation.
Conclusion: This is the first case of cortical stromal hyperplasia presenting with bilateral involvement of small ovaries, ascites and a pleural effusion. Meigs' syndrome and its variants develop with clinical pictures suggestive of malignancy. Thorough evaluation and individualized treatment are necessary.