Meigs Syndrome and Pseudo-Meigs Syndrome
Terminology
The pleural effusion you describe is called "Meigs syndrome" if the ovarian tumor is benign (typically a fibroma), or "pseudo-Meigs syndrome" if the ovarian tumor is malignant (ovarian cancer). 1, 2
Key Diagnostic Distinctions
Meigs Syndrome (Benign)
- Classic triad: benign solid ovarian tumor (usually fibroma or fibrothecoma), ascites, and pleural effusion 1, 2
- The effusion and ascites completely resolve after surgical removal of the benign ovarian tumor 1, 2
- CA-125 can be markedly elevated (>1800 U/mL reported), mimicking malignancy 2
- Ascites cytology may show false-positive results for malignancy despite benign pathology 2
Pseudo-Meigs Syndrome (Malignant)
- Same clinical presentation (ascites and pleural effusion) but associated with malignant ovarian tumors, including metastatic disease to the ovary 3
- Effusions and ascites improve or resolve after oophorectomy, even in metastatic cases 3
- In ovarian cancer specifically, pleural effusion with positive cytology indicates Stage IV disease 4, 5
Critical Management Approach
For Suspected Malignant Disease (Ovarian Cancer)
Prioritize systemic chemotherapy before definitive pleural procedures in ovarian cancer, as this is a chemotherapy-sensitive malignancy. 4
Initial Assessment
- Drain the effusion before starting chemotherapy to prevent drug accumulation and increased myelosuppression 4
- Obtain pleural fluid cytology—positive cytology confirms Stage IV disease 4, 5
- Note that approximately 30% of malignant pleural effusions have false-negative cytology 5
Surgical Staging Considerations
- If pleural effusion is present, positive cytology is required to assign Stage IV 4
- Video-assisted thoracoscopic surgery (VATS) serves as diagnostic, staging, and therapeutic modality when cytology is negative but malignancy suspected 5
- VATS quantifies pleural tumor burden and determines feasibility of optimal cytoreduction 5
Treatment Sequence
- Ovarian cancer has the longest median survival among malignant pleural effusions (compared to lung cancer which has the shortest) 4
- Taxane-platinum neoadjuvant chemotherapy should be offered to patients with extensive pleural disease not amenable to optimal cytoreduction 5
- Maximal cytoreduction (no visible disease) or optimal cytoreduction (no residual implant >1 cm) remains the primary surgical goal, even in Stage IV disease 5
- Median overall survival with pleural space involvement is approximately 2 years 5
For Suspected Benign Disease (Meigs Syndrome)
Surgical excision of the ovarian mass is both diagnostic and curative, with complete resolution of pleural effusion and ascites expected postoperatively. 1, 2
Preoperative Management
- Symptomatic pleural drainage may be necessary for severe dyspnea 3, 1
- Paracentesis for tense ascites causing respiratory compromise 3
- Consider minimally invasive surgery (laparoscopy) for tissue diagnosis when Meigs syndrome suspected despite elevated CA-125 2
Postoperative Expectations
- Pleural effusion typically resolves within 7 days of ovarian tumor removal 1
- CA-125 normalizes postoperatively (from >1800 U/mL to <15 U/mL reported) 2
- No recurrence of effusion or ascites expected if truly Meigs syndrome 1
Common Pitfalls to Avoid
- Do not assume malignancy based solely on elevated CA-125 and positive ascites cytology—Meigs syndrome can present identically 2
- Do not perform definitive pleurodesis before attempting systemic chemotherapy in ovarian cancer—this is a chemotherapy-sensitive tumor that may respond without invasive pleural procedures 4
- Do not start chemotherapy without first draining the effusion—this increases risk of myelosuppression 4
- Do not forego surgical exploration in suspected Meigs syndrome—surgery is both diagnostic and curative 1, 2