A regressive Meigs syndrome after definitive adnexal torsion

The Meigs syndrome is a rare but well known syndrome in which removal of the tumor results in cure. We report a case of a regressive Meigs syndrome after a definitive adnexal torsion which highlights the major role of the vascular phenomena in the physiopathology of this puzzling syndrome.

The Meigs syndrome is a rare but well-known syndrome defined as the association of ascites, pleural effusion, and a benign solid ovarian tumor with the gross appearance of a fibroma in which removal of the tumor results leads to complete resolution of symptoms and signs. Despite the significant number of reported cases and a large number of theories suggested in the literature, the pathogenesis of this syndrome has not been clearly elucidated. We report a case of a regressive Meigs syndrome that highlights the central role of the vascular phenomenon in the physiopathology of this syndrome.

Case Report

A 72-year old woman with temperature up to 38°C was referred for an acute left lower quadrant abdominal pain that appeared 8 hours, previously. Past medical history was unremarkable, except that she complained from intermittent bouts of a less severe pain for a year in the same location. Computed tomography (CT) scan with contrast material intravenous injection displayed a large solid lobulated left adnexal mass, with a rotation forward and above the uterus associated with ascites and right pleural effusion ( Figure 1 ). As the pain subsided, the patient preferred deferring a magnetic resonance imaging (MRI) for 5 days. MRI then depicted a large well-circumscribed ovarian mass, in the same location to that seen on the CT scan, with a predominant low signal on T1 and T2-weighted images, consistent with a fibrous lesion. A thickened and twisted tube with a “whirlpool sign” was very suggestive of adnexal torsion ( Figure 2 , A). This last finding was already present on the CT scan ( Figure 1 , A). Post-contrast MR images did not show any enhancement of the twisted adnexal suggestive of necrosis ( Figure 2 , B) and pleural effusion and ascites had disappeared ( Figure 3 ). Her CA 125 tumor marker level was 41 U/mL (normal <35 U/mL). A provisional diagnosis of regressive Meigs syndrome with a left adnexal torsion was assigned. A laparotomy was performed 7 days after the acute episode and found a necrotic left twisted adnexa with a solid ovarian mass, torsion of the tube (3 laps to a turn) and 8 mL of bloody peritoneal fluid. All other macroscopic findings were normal. Pathologic examination revealed a complete hemorrhagic necrosis of an ovarian fibroma without evidence of stromal edema (weight = 1244 g and 17 cm major axis) ( Figure 4 ) and a 10-cm long necrotic tube. Peritoneal cytology was hemorrhagic without malignant cells. Six months later, the patient was asymptomatic and doing well.