Demons-Meigs Syndrome with a High CA 125 Level: A Case Report at Soavinandriana Center Hospital Antananarivo

Introduction: Demons-Meigs syndrome combines a benign tumor of the ovary with ascites and pleural effusion. It is a rare disease and the pathophysiological mechanism is not yet well understood. The aim of our study is to report a case of Demons-Meigs syndrome, to determine its different characteristics and to make clinicians aware of the malignant predictive value of CA 125 in front of an ovarian tumor. Observation: The patient was 42 years old and had no previous history. She presented with intense abdominal pain of sudden onset, prompting a medical consultation. On clinical examination, the abdomen was distended with signs of peritoneal effusion and right pleural effusion. Ultrasound revealed a well-circumscribed, ovarian mass with an internal fluid component and hypervascularization on Doppler. The CA 125 level was elevated (293.9 U/ml). Exploratory laparotomy revealed two bilateral, firm, solid ovarian tumors without vegetation or peritoneal lesions. Macroscopically, one of the tumors was well circumscribed, lobulated with a smooth outer surface. It measured 20 × 17 × 8 cm and weighed 1400 g. The other tumor measured 19 × 11 × 5 cm, weighed 1090 g and had the same characteristics as the other tumor. Histologically, both tumors were a proliferation of fibroblastic spindle cells organized in short intersecting or storiform bundles, without cyto-nuclear atypia or excess mitoses. The diagnosis retained was bilateral ovarian fibroma in the context of a Demons-Meigs syndrome. Conclusion: Demons-Meigs syndrome is a rare entity. The concomitant elevation of the CA 125 level is not always an indicator of ovarian cancer. The curative treatment is surgical based on tumor removal ensuring the disappearance of peritoneal and pleural effusions.