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Case Reports

Ovarian Mass, Pleural Effusion, and Ascites: Revisiting Meigs Syndrome

Riker, David MD, FCCP; Goba, Daniel MD

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Journal of Bronchology & Interventional Pulmonology: January 2013 - Volume 20 - Issue 1 - p 48-51
doi: 10.1097/LBR.0b013e31827ccb35
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A 54-year-old woman G2 P0 with probable ovarian cancer presented seeking a second medical opinion for abdominal distension, fullness, constipation, weight loss, and dyspnea on exertion. She was a life-long nonsmoker and received oral contraceptives for 10 years during her child-bearing age. She reached menopause at the age of 47. She first sought medical attention 4 years earlier with similar complaints to an outside hospital and underwent a thoracentesis for a right pleural effusion. The pathology from the pleural fluid demonstrated a probable ovarian serous adenocarcinoma, positive for cytokeratin 7 and Wilms tumor protein 1, negative for cytokeratin 20 and thyroid transcription factor. The pleural fluid cancer antigen-125 (CA-125) level was 1191 U/mL. She was referred for oncologic evaluation and possible surgical treatment but she declined and proceeded with alternative ayurvedic therapy. She again presented to an outside hospital a year later with identical complaints, underwent a thoracentesis but was lost to follow-up. On her current physical examination, she was a cachectic female without lymphadenopathy, but had reduced breath sounds, more on the right than the left, and positive fluid wave from ascites without a palpable abdominal mass.

Her white blood cell count was 8.4/mm3, hemoglobin 14/mm3, hematocrit 42.3%, and platelet 674/mm3. Serum CA-125 level was 238 U/mL (normal value, <35 U/mL). She underwent an ultrasound-guided right thoracentesis for a large pleural effusion. Pleural fluid analysis revealed white blood cell count 135/mm3, red blood cell count 38/mm3, neutrophils 23%, lymphocytes 30%, macrophages 47%, pH 7.49, glucose 108 mg/dL, lactate dehydrogenase (LDH) 88 IU/L (serum LDH was 140 IU/L), and total protein 5.3 g/dL (plasma protein was 7.1 g/dL). Pleural fluid CA-125 was 395 U/mL. Pleural fluid was consistent with an exudative pleural effusion. Pleural fluid cytology showed inflammation and reactive mesothelial cells without evidence of malignancy. She declined thoracoscopy to evaluate the pleura for ovarian cancer metastasis and subsequently had recurrent episodes of pleural fluid reaccumulation needing multiple thoracentesis. A month later she consented for right thoracoscopy, pleural abrasion with talc pleurodesis. Interestingly upon entrance into the right pleural space, no abnormal pathology was noted as the pleura was relatively translucent without evidence of tumor studding. A repeat computed tomography (CT) scan of the abdomen/pelvis revealed a stable 11.78×13.4 cm heterogenous mass occupying the majority of the pelvis. It was clear that the ovarian mass did not follow a typical course consistent with ovarian cancer, supported by lack of growth over several years and absence of significant pleural disease observed on thoracoscopy (Fig. 1).

Computed tomography scan of the pelvis showing an 11.78×13.4 cm mass occupying the pelvis.

Worsening ascites was noted on CT and paracentesis resulted in short-term relief from progressing abdomen symptoms. Peritoneal fluid cytology showed reactive mesothelial cells without atypical or malignant cells. The patient declined biopsy or surgical removal of the pelvic mass; therefore a peritoneal pleurX catheter (CareFusion, Waukegan, IL) was placed under ultrasound guidance. She was referred to gynecology/oncology service for surgical treatment but the patient refused that option. She required biweekly drainage of ascites.

Eight months after initially presenting to our medical center for a second opinion she agreed to have a CT-guided fine needle aspiration of the pelvic mass. Biopsy showed spindle cell lesions, which was consistent with a benign ovarian tumor. However, due to small sampling size, ovarian carcinoma could not be completely excluded. Finally she agreed for surgery and underwent an exploratory laparotomy, total abdominal hysterectomy, and bilateral salpingo-oophorectomy. Intraoperative findings were ascites, small anteverted uterus, and a 15 cm multilobulated, solid left ovarian mass. Frozen section showed fibrothecoma. Biopsy of the ovarian mass revealed fibroma of the ovary.

The patient was seen for follow-up after 12 weeks in clinic and there was no evidence of recurrent pleural effusion (Figs. 2, 3).

A and B, Posterior-anterior and lateral chest x-rays revealing bilateral pleural effusions.
Fibroma: ×200 hematoxylin and eosin staining.


Meigs syndrome is defined as the presence of pleural effusion and ascites in association with a benign ovarian tumor. The ascites and the pleural effusion resolve soon after the tumor is removed. Meigs and Cass1 first reported a series of 7 patients who had ascites and pleural effusions associated with benign ovarian fibroma. The ascites and pleural effusion resolved after the removal of the ovarian tumor. Ovarian fibromas constitute the majority of the benign tumors seen in Meigs syndrome.2 Fibromas represent approximately 4% of ovarian tumors and Meigs syndrome occurs in only 1% to 2% of these cases, thus is a rare condition.3 Although cases have been reported in children and women who are younger than 30 years, Meigs syndrome is more common in postmenopausal women with an average age of about 50 years.4

The pathophysiology for the formation of the ascites and pleural effusion is largely unknown. Several hypotheses have been suggested. Meigs et al5 suggested that the ascitic fluid results from edematous fibromas that can leak fluid. Another theory is that the pressure exerted on pelvic and abdominal lymphatics by the tumor itself hampers lymphatic drainage, resulting in intraperitoneal fluid accumulation.5 Inflammatory cytokines (IL-1B, IL-6, IL-8, and TNF A) have been implicated in the pathophysiology of ascites and hydrothorax formation probably by causing hyperpermeability of ovarian and/or peritoneal vasculature with subsequent transudation of fluid into the peritoneal cavity.6 It is believed that the pleural effusion arises secondary to passage of ascitic fluid into the pleural space through the diaphragm, either through congenital defects that tend to be more common on the right or through diaphragmatic lymphatics. The pleural fluid may be located on the left side or may be bilateral. In 1 case series, 65% of the pleural effusions were on the right side, 10% on the left side, and 22% were bilateral.7 The pleural effusion in Meigs syndrome is classically transudative8 but it can be exudative as is in our patient or hemorrhagic.9

CA-125 antigen is a glycoprotein with a high molecular weight and is recognized by a monoclonal antibody (OC-125). It is a tumor marker associated with ovarian carcinoma. There have been reports in the literature of elevated CA-125 levels in patients with Meigs syndrome.10 Serum CA-125 levels >1000 U/mL is unusual.10 Immunohistochemical studies suggest that serum CA-125 elevation in patients with Meigs syndrome is caused by mesothelial expression of the antigen rather than by the fibroma.11 On review of cases of Meigs syndrome with elevated serum CA-125 levels, the volume of the ascites was positively correlated with higher levels of CA-125 but the tumor size was not linearly associated with CA-125 levels.12

The ultrasound appearance of fibromas are nonspecific13 and the spectrum of CT findings in ovarian fibroma is broad.14 The inability to confirm ovarian benign tumor on imaging and the presence of ascites/pleural effusion and elevation of CA-125 makes tissue diagnosis absolutely necessary.

The treatment is exploratory laparotomy that includes biopsy of the ovarian mass, lymph node biopsies, biopsy of omentum, and pelvic washings. Unilateral salpingo-oophorectomy is performed in women of reproductive age, whereas total hysterectomy is preferred in postmenopausal women.15

Tunneled drainage catheters are designed for use in patients with malignant pleural effusion and ascites. In a retrospective study comparing patients who underwent abdominal pleurx catheter placement and repeated large volume paracentesis, the complication rates was similar but pleurx reduced the separate patient encounter and is associated with more patient satisfaction16 as in our patient. Our patient had a presumptive diagnostic of ovarian cancer and preferred for pleurx catheter placement rather than repeated large volume paracentesis.

The possibility of a malignant pleural effusion should be considered in all patients with undiagnosed exudative pleural effusions. The easiest way to establish the diagnosis of a malignant pleural effusion is pleural fluid cytology. If the cause remains undiagnosed after a cytology examination, thoracoscopy or cutting needle biopsy of the pleura can be used with high diagnostic yield.17 In our patient there was no lung or pleural abnormality for biopsy and underwent thoracoscopy, with pleurodesis performed for symptom management.

The prognosis of Meigs syndrome is good and <1% of fibromas progress to fibrosarcoma.18 The pleural effusion and ascites resolve within a few weeks after tumor resection. We favor the use of chest ultrasound to follow pleural effusion progression, as it is superior to chest x-rays in identifying residual pleural effusion and can detect amounts as small as 3 to 5 mL.19


Although exudative pleural effusion associated with pelvic mass and elevated CA-125 portends a grave prognosis, it can also be seen in benign processes such as our patient. In patients with an ovarian mass, pleural effusion, and ascites, a thorough investigation is warranted to exclude ovarian cancer.


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Meigs syndrome; exudative pleural effusion; ovarian mass; CA-125

© 2013 Lippincott Williams & Wilkins, Inc.