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Meigs Syndrome

  • Author: Klaus-Dieter Lessnau, MD, FCCP; Chief Editor: Warner K Huh, MD  more...
 
Updated: Mar 24, 2016
 

Background

Meigs syndrome is defined as the triad of benign ovarian tumor with ascites and pleural effusion that resolves after resection of the tumor. Ovarian fibromas constitute the majority of the benign tumors seen in Meigs syndrome. Meigs syndrome, however, is a diagnosis of exclusion, only after ovarian carcinoma is ruled out.[1]

In 1934, Salmon described the association of pleural effusion with benign pelvic tumors. In 1937, Meigs and Cass described 7 cases of ovarian fibromas associated with ascites and pleural effusion.[2] The syndrome was named as Meig’s syndrome by Rhoads and Terrel in 1937.[3] In 1954, Meigs proposed limiting true Meigs syndrome to benign and solid ovarian tumors accompanied by ascites and pleural effusion, with the condition that removal of the tumor cures the patient without recurrence. Histologically, the benign ovarian tumor may be a fibroma, thecoma, cystadenoma, or granulosa cell tumor.

Pseudo-Meigs syndrome consists of pleural effusion (an example of which can be seen in the image below), ascites, and benign tumors of the ovary other than fibromas. These benign tumors include those of the fallopian tube or uterus and mature teratomas, struma ovarii, and ovarian leiomyomas.[4] This terminology sometimes also includes ovarian or metastatic gastrointestinal malignancies.

Chest radiograph showing left-sided pleural effusi Chest radiograph showing left-sided pleural effusion.

Atypical Meigs characterized by a benign pelvic mass with right-sided pleural effusion but without ascites has been reported at least twice. As in Meigs syndrome, pleural effusion resolves after removal of the pelvic mass.

Pseudo-pseudo Meigs syndrome includes patients with systemic lupus erythematosus and enlarged ovaries.[5]

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Pathophysiology

Ascites is present in 10-15% of cases, and hydrothorax is found in only 1% of cases.[6, 7]

Etiology of ascitic fluid

The pathophysiology of ascites in Meigs syndrome is speculative. Meigs suggested that irritation of the peritoneal surfaces by a hard, solid ovarian tumor could stimulate the production of peritoneal fluid. Samanth and Black studied ovarian tumors accompanied by ascites and found that only tumors larger than 10 cm in diameter with a myxoid component to the stroma are associated with ascites.[8] These authors believe that their observations favor secretion of fluid from the tumor as the source of the ascites.

Other proposed mechanisms are direct pressure on surrounding lymphatics or vessels, hormonal stimulation, and tumor torsion. Development of ascites may be due to release of mediators (eg, activated complements, histamines, fibrin degradation products) from the tumor, leading to increased capillary permeability.

Origin of pleural effusion

The etiology of pleural effusion is unclear. Efskind and Terada et al theorize that ascitic fluid is transferred via transdiaphragmatic lymphatic channels. The size of the pleural effusion is largely independent of the amount of ascites. The pleural fluid may be located on the left side or may be bilateral.[1, 9, 10]

Efskind's study

Efskind injected ink into the lower abdomen of a woman with Meigs syndrome and found that the ink particles accumulated in the lymphatics of the pleural surface within half an hour. Blockage of these lymphatics prevented accumulation of pleural fluid and caused an increase in ascitic fluid.

Terada and colleagues' study

In 1992, Terada and colleagues injected labeled albumin into the peritoneum and found that the maximum concentration was detected in the right pleura within 3 hours.

Nature of the ascitic and pleural fluid

Ascitic fluid and pleural fluid in Meigs syndrome can be either transudative or exudative. [9]  Meigs performed electrophoresis on several cases and determined that pleural and ascitic fluids were similar in nature. Tumor size, rather than the specific histologic type, is thought to be the important factor in the formation of ascites and accompanying pleural effusion.

In 2015, the findings of Krenke et al. in their systematic literature review of 541 cases reported with Meig’s syndrome revealed that an exudative origin in pleural effusions was significantly more prevalent than the ones from transudative origin.[11]

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Epidemiology

Frequency

United States

Ovarian tumors are more prevalent in women in upper socioeconomic groups. Ovarian fibromas represent approximately 2-5% of surgically removed ovarian tumors, and Meigs syndrome occurs in only 1-2% of these cases; thus, it is a rare condition. Ascites is present in 10-15% of women with ovarian fibroma, and hydrothorax is present in 1%, especially those with larger lesions.

Age-related demographics

The incidence of ovarian tumor begins to increase in the third decade and increases progressively in postmenopausal women, with an average of about 50 years.[1, 9] Meigs syndrome in prepubertal girls with benign teratomas and cystadenomas has been reported.

International statistics

The international prevalence is unknown.

Mortality/Morbidity

Life expectancy of patients with Meigs syndrome mirrors that of the general population after surgery, and less than 1% of fibromas progress to fibrosarcoma.

Although Meigs syndrome mimics a malignant condition, it is a benign disease and has a very good prognosis if properly managed. Life expectancy after surgical removal of the tumor is the same as the general population.[10]

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Contributor Information and Disclosures
Author

Klaus-Dieter Lessnau, MD, FCCP Clinical Associate Professor of Medicine, New York University School of Medicine; Medical Director, Pulmonary Physiology Laboratory; Director of Research in Pulmonary Medicine, Department of Medicine, Section of Pulmonary Medicine, Lenox Hill Hospital

Klaus-Dieter Lessnau, MD, FCCP is a member of the following medical societies: American College of Chest Physicians, American College of Physicians, American Medical Association, American Thoracic Society, Society of Critical Care Medicine

Disclosure: Nothing to disclose.

Coauthor(s)

Rajeshwari Chavda, MD Consulting Staff, Emergency Care Group of Northwest

Disclosure: Nothing to disclose.

Mir Omar Ali, MD Fellow, Department of Pulmonary Medicine, Lenox Hill Hospital, New York University

Mir Omar Ali, MD is a member of the following medical societies: American College of Physicians, Society of Critical Care Medicine

Disclosure: Nothing to disclose.

Lalit K Kanaparthi, MD Attending Physician, North Florida Lung Associates

Lalit K Kanaparthi, MD is a member of the following medical societies: American College of Chest Physicians, American Medical Association, American Thoracic Society

Disclosure: Nothing to disclose.

Dora E Izaguirre, MD Primary Care Physician; Researcher, Department of Medicine, Section of Pulmonary Medicine, Lenox Hill Hospital

Dora E Izaguirre, MD is a member of the following medical societies: American Heart Association, American Medical Association, American Public Health Association, Colegio Medico de Honduras

Disclosure: Nothing to disclose.

Jesus Lanza, MD Fellow in Pulmonary and Critical Care Medicine, Department of Medicine, Section of Pulmonary Medicine, Lenox Hill Hospital

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Chief Editor

Warner K Huh, MD Professor, Department of Obstetrics and Gynecology, Division of Gynecologic Oncology, Senior Scientist, Comprehensive Cancer Center, University of Alabama School of Medicine

Warner K Huh, MD is a member of the following medical societies: American College of Obstetricians and Gynecologists, American College of Surgeons, Massachusetts Medical Society, Society of Gynecologic Oncology, American Society of Clinical Oncology

Disclosure: I have received consulting fees for: Merck; THEVAX.

Acknowledgements

Ayesha Akhter, MD Consulting Staff, Department of Internal Medicine, Columbia Tech Center, Vancouver Clinic

Disclosure: Nothing to disclose.

Jeffrey B Garris, MD Chief, Assistant Professor, Department of Obstetrics and Gynecology, Division of Urogynecology and Reconstructive Pelvic Surgery, Tulane University School of Medicine

Jeffrey B Garris, MD is a member of the following medical societies: American College of Obstetricians and Gynecologists, American Institute of Ultrasound in Medicine, American Medical Association, American Urological Association, Association of Professors of Gynecology and Obstetrics, Louisiana State Medical Society, Royal Society of Medicine, and Sigma Xi

Disclosure: Nothing to disclose.

References
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  3. Liao Q, Hu S. Meigs’ Syndrome and Pseudo-Meigs’ Syndrome: Report of Four Cases and Literature Reviews. Journal of Cancer Therapy. Journal of cancer therapy. 2015 April. 6(04):293.

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Chest radiograph showing left-sided pleural effusion.
 
 
 
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