Meigs Syndrome

Meigs Syndrome

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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.

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]

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

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.

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.

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]

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.

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]

Riker D, Goba D. Ovarian mass, pleural effusion, and ascites: revisiting meigs syndrome. J Bronchology Interv Pulmonol. 2013 Jan. 20(1):48-51. [Medline].

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.

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.

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

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

Loue VA, Gbary E, Koui S, Akpa B, Kouassi A. Bilateral Ovarian Fibrothecoma Associated with Ascites, Bilateral Pleural Effusion, and Marked Elevated Serum CA-125. Case Rep Obstet Gynecol. 2013. 2013:189072. [Medline]. [Full Text].

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). 2004 May. 33(3):251-4. [Medline].

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

CIFDS G, André SA, Maggi L, Nogueira FJ. Syndrome with Elevated CA 125: Case Report with a Journey through Literature. J Pulm Respir Med. 2015. 5(303):2.

Park JW, Bae JW. Postmenopausal Meigs’ Syndrome in Elevated CA-125: A Case Report. J Menopausal Med. 2015 Apr. 21 (1):56-9. [Medline].

Krenke R, Maskey-Warzechowska M, Korczynski P, Zielinska-Krawczyk M, Klimiuk J, Chazan R, et al. Pleural Effusion in Meigs’ Syndrome-Transudate or Exudate?: Systematic Review of the Literature. Medicine (Baltimore). 2015 Dec. 94 (49):e2114. [Medline].

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

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. 2004 Jul. 94(1):226-8. [Medline].

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. 2005 Apr. 97(1):288-91. [Medline].

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

Danilos J, Michał Kwaśniewski W, Mazurek D, Bednarek W, Kotarski J. Meigs’ syndrome with elevated CA-125 and HE-4: a case of luteinized fibrothecoma. Prz Menopauzalny. 2015 Jun. 14 (2):152-4. [Medline].

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

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. 1995 Apr. 90(4):657-8. [Medline].

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

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

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. 2008 Mar-Apr. 18(2):372-5. [Medline].

Moran-Mendoza A, Alvarado-Luna G, Calderillo-Ruiz G, et al. Elevated CA125 level associated with Meigs’ syndrome: case report and review of the literature. Int J Gynecol Cancer. 2006 Jan-Feb. 16 Suppl 1:315-8. [Medline].

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

Fremed MA, Levin TL, Sun KY, Weiser DA. Ovary preservation in the treatment of childhood Meigs syndrome. Pediatr Blood Cancer. 2015 Nov. 62(11):2011-4. [Medline].

Jin C, Dong R, Bu H, Yuan M, Zhang Y, Kong B. Coexistence of benign struma ovarii, pseudo-Meigs’ syndrome and elevated serum CA 125: Case report and review of the literature. Oncol Lett. 2015 Apr. 9(4):1739-1742. [Medline].

Riker D, Goba D. Ovarian mass, pleural effusion, and ascites: revisiting Meigs syndrome. J Bronchology Interv Pulmonol. 2013 Jan. 20(1):48-51. [Medline].

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.

Dora E Izaguirre Anariba, MD, MPH  Physician, Department of Medicine, Wyckoff Heights Medical Center

Dora E Izaguirre Anariba, MD, MPH is a member of the following medical societies: American College of Physicians, 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.

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.

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

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

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.

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.

Meigs Syndrome

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