Meigs Syndrome: Definition and Clinical Features
Meigs syndrome is a rare benign condition characterized by the classic triad of a solid benign ovarian tumor (typically fibroma or fibrothecoma), ascites, and pleural effusion, all of which completely resolve after surgical removal of the tumor. 1, 2, 3
Core Diagnostic Criteria
The syndrome requires all three components to be present 1, 2, 3:
- Benign solid ovarian tumor - most commonly ovarian fibroma or fibrothecoma, though granulosa cell tumors and Brenner tumors have been reported 1, 4
- Ascites - peritoneal fluid accumulation 2, 3
- Pleural effusion - typically unilateral, more commonly right-sided 2, 3
- Complete resolution of both ascites and pleural effusion after tumor resection is essential for diagnosis 1, 2, 3
Clinical Presentation Patterns
Patients typically present to respiratory or internal medicine departments rather than gynecology, as respiratory symptoms often dominate the clinical picture. 1, 3
Common presenting symptoms include 1, 2, 3:
- Progressive dyspnea on exertion
- Cough and expectoration
- Abdominal distension
- Chest oppression
Critical Diagnostic Pitfall: Elevated CA-125
A major diagnostic challenge is that CA-125 levels are frequently markedly elevated in Meigs syndrome (ranging from 150-1040 U/mL), which strongly mimics ovarian malignancy and can lead to inappropriate chemotherapy if tissue diagnosis is not obtained. 1, 4, 5
Key points about CA-125 elevation 4, 5:
- Levels can exceed 1000 U/mL despite benign pathology
- Immunohistochemical staining may show CA-125 reactivity in the omentum only, not the tumor itself 4
- CA-125 normalizes completely after tumor resection 1, 5
Rare Variant: Pericardial Involvement
While classically involving pleural and peritoneal spaces, pericardial effusion can rarely occur in Meigs syndrome and may even precede pleural effusion development. 2
Diagnostic Approach
Cytologic or histologic confirmation is imperative before initiating any treatment, as the combination of pelvic mass, ascites, hydrothorax, and elevated CA-125 in postmenopausal women has high probability of malignancy but can be Meigs syndrome. 4, 5
Essential workup includes 1, 3, 5:
- Thoracentesis and paracentesis with cytology - should be negative for malignant cells
- Pelvic imaging (ultrasound, CT, or MRI) to characterize the ovarian mass
- Fine-needle aspiration may show spindle cell tumor consistent with sex-cord stromal tumor 5
- Serum CA-125 (elevated but non-specific)
Definitive Management
Surgical resection of the ovarian tumor is both diagnostic and curative, with complete resolution of ascites and pleural effusion expected postoperatively. 1, 2, 3, 5
Typical surgical approach 5:
- Total abdominal hysterectomy with bilateral salpingo-oophorectomy in postmenopausal women
- Laparoscopic excision may be appropriate in selected cases 3
- Resolution of effusions typically occurs within days to 2 months postoperatively 1, 5
Clinical Significance
Despite presenting identically to disseminated ovarian malignancy, Meigs syndrome is completely curable by surgery alone without need for chemotherapy, making accurate diagnosis critical to avoid overtreatment. 2, 4