Meigs Syndrome 

  • Author: Klaus-Dieter Lessnau, MD, FCCP; Chief Editor: Warner K Huh, MD   more...
 
Updated: Jul 15, 2011
 

Background

Meigs syndrome is defined as the triad of benign ovarian tumor with ascites and pleural effusion that resolves after resection of the tumor. The ovarian tumor in Meigs syndrome is a fibroma.

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.[1] 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.[2] This terminology sometimes also includes ovarian or metastatic gastrointestinal malignancies.

Chest radiograph showing left-sided pleural effusiChest 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.[3]

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Pathophysiology

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.[4] 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.

  • 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. 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.

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Epidemiology

Frequency

United States

Ovarian tumors are more prevalent in upper socioeconomic groups. Ovarian fibroma is found in 2-5% of surgically removed ovarian tumors, and Meigs syndrome is observed in about 1%. Ascites is present in 10-15% of those with ovarian fibroma and hydrothorax in 1%, especially with larger lesions.

International

Prevalence is unknown.

Mortality/Morbidity

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 mirrors that of the general population.

Age

The incidence of ovarian tumor begins to increase in the third decade and increases progressively to peak in the seventh decade. Meigs syndrome in prepubertal girls with benign teratomas and cystadenomas has been reported.

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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, and Society of Critical Care Medicine

Disclosure: Sepracor None None

Coauthor(s)

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

Disclosure: Nothing to disclose.

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

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 and Society of Critical Care Medicine

Disclosure: Nothing to disclose.

Lalit K Kanaparthi, MD  Senior Fellow, Department of Pulmonary Medicine, Lenox Hill Hospital

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

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

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

Disclosure: Medscape Salary Employment

Frederick B Gaupp, MD  Consulting Staff, Department of Family Practice, Hancock Medical Center

Frederick B Gaupp, MD is a member of the following medical societies: American Academy of Family Physicians

Disclosure: Nothing to disclose.

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, American Society of Clinical Oncology, Massachusetts Medical Society, and Society of Gynecologist Oncologists

Disclosure: MERCK Consulting fee Consulting; GSK Consulting fee Consulting; ROCHE PHARMA/DIAGNOSTICS Consulting fee Consulting; HOLOGICS Consulting fee Consulting; HELIX BIOPHARMA Consulting fee Consulting; COVIDIEN Consulting fee Consulting; INTUITIVE SURGICAL Surgical Proctor

References
  1. Meigs JV, Cass JW. Fibroma of the ovary with ascites and hydrothorax: with a report of seven cases. Am J Obstet Gynecol. 1937;33:249-267.

  2. Dunn JS Jr, Anderson CD, Method MW. Hydropic degenerating leiomyoma presenting as pseudo-Meigs syndrome with elevated CA 125. Obstet Gynecol. Oct 1998;92(4 Pt 2):648-9. [Medline].

  3. Schmitt R, Weichert W, Schneider W, Luft FC, Kettritz R. Pseudo-pseudo Meigs' syndrome. Lancet. Nov 5 2005;366(9497):1672. [Medline].

  4. Samanth KK, Black WC. Benign ovarian stromal tumors associated with free peritoneal fluid. Am J Obstet Gynecol. Jun 15 1970;107(4):538-45. [Medline].

  5. Loizzi V, Cormio G, Resta L, Fattizzi N, Vicino M, Selvaggi L. Pseudo-Meigs syndrome and elevated CA125 associated with struma ovarii. Gynecol Oncol. Apr 2005;97(1):282-4. [Medline].

  6. Zannoni GF, Gallotta V, Legge F, Tarquini E, Scambia G, Ferrandina G. Pseudo-Meigs' syndrome associated with malignant struma ovarii: a case report. Gynecol Oncol. Jul 2004;94(1):226-8. [Medline].

  7. Tjalma WA. Ascites, pleural effusion, and CA 125 elevation in an SLE patient, either a Tjalma syndrome or, due to the migrated Filshie clips, a pseudo-Meigs syndrome. Gynecol Oncol. Apr 2005;97(1):288-91. [Medline].

  8. Jones OW, Surwit EA. Meigs syndrome and elevated CA 125. Obstet Gynecol. Mar 1989;73(3 Pt 2):520-1. [Medline].

  9. Lin JY, Angel C, Sickel JZ. Meigs syndrome with elevated serum CA 125. Obstet Gynecol. Sep 1992;80(3 Pt 2):563-6. [Medline].

  10. Agaba EI, Ekwempu CC, Ugoya SO, Echejoh GO. Meigs' syndrome presenting as haemorrhagic pleural effusion. West Afr J Med. Jul-Sep 2007;26(3):253-5. [Medline].

  11. Aoshima M, Tanaka H, Takahashi M, Nakamura K, Makino I. Meigs' syndrome due to Brenner tumor mimicking lupus peritonitis in a patient with systemic lupus erythematosus. Am J Gastroenterol. Apr 1995;90(4):657-8. [Medline].

  12. Carson SA, Mazur MT. Atypical endometrioid cystadenofibroma with Meigs' syndrome: ultrastructure and S-phase fraction. Cancer. Feb 1 1982;49(3):472-9. [Medline].

  13. Cisse CT, Ngom PM, Sangare M, Ndong M, Moreau JC. [Ovarian fibroma associated with Demons-Meigs syndrome and elevated CA 125]. J Gynecol Obstet Biol Reprod (Paris). May 2004;33(3):251-4. [Medline].

  14. Lacson AG, Alrabeeah A, Gillis DA, Salisbury S, Grantmyre EB. Secondary massive ovarian edema with Meig's syndrome. Am J Clin Pathol. May 1989;91(5):597-603. [Medline].

  15. Mitrou S, Manek S, Kehoe S. Cystic struma ovarii presenting as pseudo-Meigs' syndrome with elevated CA125 levels. A case report and review of the literature. Int J Gynecol Cancer. Mar-Apr 2008;18(2):372-5. [Medline].

  16. Morán-Mendoza A, Alvarado-Luna G, Calderillo-Ruiz G, Serrano-Olvera A, López-Graniel CM, Gallardo-Rincón D. Elevated CA125 level associated with Meigs' syndrome: case report and review of the literature. Int J Gynecol Cancer. Jan-Feb 2006;16 Suppl 1:315-8. [Medline].

  17. Peparini N, Di Matteo FM, Silvestri A, Caronna R, Chirletti P. Abdominal hypertension in Meigs' syndrome. Eur J Surg Oncol. Sep 29 2007;[Medline].

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Chest radiograph showing left-sided pleural effusion.
 
 
 
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