Meigs' syndrome

Overview

Meigs' syndrome is a rare but benign medical condition characterized by the triad of ascites (accumulation of fluid in the peritoneal cavity), pleural effusion (fluid in the pleural space around the lungs), and a benign ovarian tumor, typically a fibroma. The syndrome is named after American gynecologist Dr. Joe Vincent Meigs, who described it in the 1930s.

Although the presentation may mimic advanced ovarian cancer or other malignancies, Meigs' syndrome is not cancerous and resolves completely once the tumor is removed. It is important for clinicians to distinguish Meigs’ syndrome from malignant conditions to prevent overtreatment.

Causes

The exact mechanism of Meigs’ syndrome is not fully understood, but it is always associated with a benign ovarian tumor, most commonly an ovarian fibroma. Less commonly, other benign sex cord-stromal tumors such as thecomas or granulosa cell tumors may be involved.

Proposed mechanisms include:

• Mechanical irritation: The tumor may irritate the peritoneal surfaces, causing ascitic fluid production.

• Fluid imbalance: Large tumors may obstruct lymphatic drainage or venous return, leading to fluid accumulation in the abdomen and pleural cavity.

• Transdiaphragmatic movement: Ascitic fluid may migrate through small diaphragmatic defects into the pleural space, usually on the right side.

True Meigs’ syndrome resolves with tumor removal. The term pseudo-Meigs’ syndrome is used when similar symptoms are caused by other pelvic tumors, including malignant ones.

Symptoms

The clinical presentation of Meigs’ syndrome varies depending on the size of the tumor and the amount of fluid accumulation. Common symptoms include:

1. Abdominal Symptoms (due to Ascites)

• Abdominal distension and bloating

• Pelvic or lower abdominal discomfort

• Early satiety and gastrointestinal fullness

2. Respiratory Symptoms (due to Pleural Effusion)

• Shortness of breath (dyspnea)

• Pleuritic chest pain

• Non-productive cough

3. Menstrual or Reproductive Changes

• Irregular menstruation (occasionally)

• Palpable pelvic mass during gynecological exam

In most cases, the pleural effusion is unilateral and occurs on the right side. These symptoms typically resolve after surgical removal of the tumor.

Diagnosis

Diagnosing Meigs’ syndrome involves identifying the triad of ascites, pleural effusion, and a benign ovarian tumor, along with excluding malignancy. Diagnostic steps include:

1. Imaging

• Pelvic ultrasound: First-line imaging to detect ovarian mass

• CT or MRI: Detailed evaluation of the tumor and fluid accumulation

• Chest X-ray: Confirms pleural effusion, typically on the right

2. Laboratory Tests

• Serum CA-125: May be mildly elevated but not to levels typical of malignancy

• Thoracentesis and paracentesis: Fluid analysis typically shows transudative or low-protein fluid with no malignant cells

3. Histopathology

• Definitive diagnosis: Made after surgical excision and pathological examination of the ovarian tumor

Because the presentation mimics advanced ovarian carcinoma, careful assessment is needed to avoid misdiagnosis. Resolution of symptoms following tumor removal supports the diagnosis of Meigs' syndrome.

Treatment

The definitive treatment for Meigs’ syndrome is surgical removal of the ovarian tumor. Treatment steps include:

1. Surgical Management

• Oophorectomy: Removal of the affected ovary, typically via laparoscopy or laparotomy

• Salpingo-oophorectomy: Removal of the ovary and fallopian tube if necessary

• Total abdominal hysterectomy with bilateral salpingo-oophorectomy: May be considered in postmenopausal women or when other pathologies are suspected

2. Supportive Management

• Thoracentesis or paracentesis: For symptomatic relief prior to surgery if effusions are large

• Monitoring: Fluid balance and respiratory function in patients with large pleural effusions

No chemotherapy or radiation is required, as the tumor is benign. Hormonal or medical therapy is not indicated.

Prognosis

The prognosis for Meigs’ syndrome is excellent. Key points include:

• Complete resolution: Ascites and pleural effusion resolve rapidly after tumor removal

• No recurrence: Symptoms do not recur once the benign tumor is excised

• No malignant transformation: The tumor is benign, and long-term outcomes are favorable

Follow-up includes postoperative monitoring and periodic pelvic imaging to detect any recurrence or development of new pelvic masses. Because Meigs’ syndrome mimics cancer, early recognition can prevent unnecessary anxiety and avoid overtreatment.