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Meigs Syndrome

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
Rajeshwari Chavda, MD, Consulting Staff, Emergency Care Group of Northwest; Ayesha Akhter, MD, Consulting Staff, Department of Internal Medicine, Columbia Tech Center, Vancouver Clinic; Mir Omar Ali, MD, Fellow, Department of Pulmonary Medicine, Lenox Hill Hospital, New York University; Lalit K Kanaparthi, MD, Fellow in Pulmonary Medicine, Lenox Hill Hospital

Updated: Oct 9, 2008

Introduction

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, 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.

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

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.

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.

Clinical

History

Patients with Meigs syndrome may have a family history of ovarian cancer. The chief complaints are vague and generally manifest over time.

  • Fatigue
  • Shortness of breath
  • Increased abdominal girth
  • Weight loss
  • Nonproductive cough
  • Bloating
  • Amenorrhea for premenopausal women
  • Menstrual irregularity

Physical

Positive signs include the following:

  • Vital signs
    • Tachypnea
    • Tachycardia
  • Lungs
    • Dullness to percussion
    • Decreased tactile fremitus
    • Decreased vocal resonance
    • Decreased breath sounds are noted, suggesting pleural effusion. Pleural effusion is mostly observed on the right side, but it can also be left sided.
  • Abdomen
    • Examination may reveal a small or large pelvic mass, or no mass may be felt.
    • Ascites is present, with shifting dullness and/or fluid thrill.
  • Pelvis: Examination reveals a pelvic mass.

Causes

When an ovarian mass is associated with Meigs syndrome and an elevated CA-125 serum level, a malignant process may be suspected. A negative cytologic examination result of ascitic effusion, the absence of peritoneal implantation, and benign histology should limit surgical procedures. This decision should be made by an experienced gynecologic surgeon or a gynecologic oncologist.

  • Case reports exist of pseudo-Meigs syndrome associated with malignant struma ovarii and elevated CA-125 levels.5,6 The choice of not performing adjuvant therapy is feasible after optimal surgery and adequate staging procedure given to the usually clinical benign course and the low incidence of metastases in malignant struma ovarii. Careful patient counseling is required.
  • Struma ovarii is a rare cause of ascites, hydrothorax, elevated CA-125 levels, and hyperthyroidism.6 This rare condition should be considered in the differential diagnosis in patients with ascites and pleural effusions but with negative cytologic test results.
  • The combination of ascites, pleural effusion, CA-125 level elevation, and no tumor in a patient with systemic lupus erythematosus is either a Tjalma syndrome or due to the migrated Filshie clips a pseudo-Meigs syndrome.7

Differential Diagnoses

Ascites
Malignant Effusion
Cirrhosis
Milroy Disease
Colon Cancer, Adenocarcinoma
Nephrotic Syndrome
Hypoalbuminemia
Ovarian Cancer
Lung Cancer, Non-Small Cell
Pleural Effusion
Lung Cancer, Oat Cell (Small Cell)
Tuberculosis

Other Problems to Be Considered

Congestive heart failure

Workup

Laboratory Studies

Lab studies for patients with Meigs syndrome include the following:

  • CBC count: This study provides information about hemoglobin, hematocrit, and platelet levels. A low hemoglobin count requires further workup, including reticulocyte count, total iron-binding capacity, and iron and ferritin levels. Anemia in patients with Meigs syndrome is most likely due to iron deficiency. Anemia can be corrected emergently by blood transfusion in patients undergoing surgery for Meigs syndrome. Anemia can be treated with iron supplementation postoperatively.
  • Basic metabolic profile: Studies of sodium, potassium, chloride, bicarbonate, blood urea nitrogen, creatinine, and glucose levels are included. These electrolytes are checked before the patient undergoes surgery. If necessary, corrections of these electrolytes are made.
  • Prothrombin time: Prothrombin time is checked before surgery. If elevated, it is a marker of coagulopathy. Elevated prothrombin time is corrected before surgery, either by administering vitamin K to the patient or by transfusing fresh frozen plasma.
  • Other than serum electrolytes and CBC count, the study of interest is the serum cancer antigen 125 (CA-125) test. Tumor marker serum levels of CA-125 can be elevated in Meigs syndrome, but the degree of elevation does not correlate with malignancy. In fact, a normal CA-125 level does not exclude the possibility of malignancy.8 The CA-125 level is not used as a screening test. The highest reported level of CA-125 after laparotomy is 1808 U/mL. This would be a false-positive result.
    • Physiologic sources of CA-125 are fetal coelomic epithelium and its derivatives, including the following:
      • Müllerian epithelium
      • Pleura
      • Pericardium
      • Peritoneum
    • Pathologic conditions related to an elevated CA-125 level include the following:
      • Pelvic inflammatory disease (PID)
      • Peritoneal damage or regeneration (eg, abdominal surgery)
      • Ovarian malignancy
      • Endometriosis
    • In 1992, Lin et al conducted a study to determine whether the ovarian fibroma was the source of serum CA-125 elevation. Using an immunohistochemical technique specific for the tumor marker, they localized CA-125 expression in the omentum and peritoneal surfaces rather than in the fibroma.9

Imaging Studies

  • Chest radiography confirms pleural effusion.
  • Abdominal and pelvic ultrasound confirms the ovarian mass and ascites.
  • CT scan of the abdomen and pelvis
    • CT scan confirms ascites and ovarian, uterine, fallopian tube, or broad ligament mass.
    • No signs of distant metastasis are observed.

Other Tests

  • Papanicolaou test findings are normal.

Procedures

  • Paracentesis: Ascitic fluid is mostly transudative. Findings are negative for malignant cells but can be positive for reactive mesothelial cells.
  • Thoracentesis: Pleural fluid is usually transudative. Findings can be exudative and negative for malignant cells.

Histologic Findings

Ovarian tumors are divided into the following histologic subgroups, and Meigs syndrome can be observed with any of the benign tumors.

  • Coelomic epithelial tumors: These tumors, which originate from the coelomic epithelium, constitute 80-85% of all ovarian tumors.
    • Serous cystadenoma and mucinous cystadenoma: 15-20% are malignant.
    • Endometrioid type and clear cell: 95-98% are malignant.
    • Brenner tumor: 2% are malignant.
  • Germ cell tumors: These tumors originate from the germ cell and constitute 10-15% of all ovarian tumors. All are malignant except mature teratomas and gonadoblastomas, which are always benign.
    • Mature teratoma
    • Immature teratoma
    • Dysgerminoma
    • Gonadoblastoma
    • Endodermal sinus
    • Embryonal carcinoma
    • Nongestational choriocarcinoma
  • Gonadal-stromal cell tumors constitute 3-5% of all tumors.
    • Granulosa cell
    • Fibroma: Fewer than 5% are malignant.
    • Thecoma: Fewer than 5% are malignant.
    • Sertoli-Leydig cell: Fewer than 5% are malignant.
    • Lipid cell type: 30% are malignant.
    • Gynandroblastoma: 100% are malignant.

Treatment

Medical Care

Medical care of patients with Meigs syndrome is intended to provide symptomatic relief of ascites and pleural effusion by means of therapeutic paracentesis and thoracentesis.

Surgical Care

  • Exploratory laparotomy with surgical staging is the treatment of choice.
    • Perform a frozen section of the ovarian mass during exploratory laparotomy. If the frozen section is consistent with benign tumor, conservative surgery (salpingo-oophorectomy or oophorectomy) is appropriate.
    • Findings of lymph node biopsies and omentum and pelvic washings are negative for malignancy if these procedures are performed during surgery.
  • In women of reproductive age, perform unilateral salpingo-oophorectomy.
  • In postmenopausal women, options include bilateral salpingo-oophorectomy with total hysterectomy and unilateral or occasionally bilateral salpingo-oophorectomy.
  • In prepubertal girls, options include wedge resection of ovary and unilateral salpingo-oophorectomy.
  • The cure rate after either type of surgery is high and recurrence is rare.

Consultations

Consult with a gynecologic surgeon for surgical management of the patient.

Activity

Patients can maintain activities as tolerated.

Follow-up

Further Inpatient Care

Observe standard postsurgical management protocols.

Further Outpatient Care

As described by Meigs, ascites and pleural effusion resolve dramatically within a few weeks to months after removal of the pelvic mass without any recurrence.

The serum CA-125 level also returns to normal after surgery.

Prognosis

Life expectancy of patients with Meigs syndrome mirrors that of the general population after surgery.

Patient Education

For excellent patient education resources, visit eMedicine's Cancer and Tumors Center. Also, see eMedicine's patient education article Ovarian Cancer.

Miscellaneous

Medicolegal Pitfalls

Meigs syndrome is benign but can be confused with malignant disease because of the presence of ascites and pleural effusion with pelvic mass. An elevated serum CA-125 level does not always indicate malignancy.

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. Cissé 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].

Keywords

Meigs syndrome, Meigs-Salmon syndrome, pseudo-Meigs syndrome, pseudo-pseudo Meigs syndrome, fibromyoma, hydroperitoneum, hydrothorax, atypical Meigs, benign ovarian tumor, ovarian cancer, ascites, pleural effusion, ovarian fibromas, pelvic tumor, pelvic mass, thecoma, cystadenoma, granulosa cell tumor, teratomas, struma ovarii

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 Society for Artificial Internal Organs, American Thoracic Society, Physicians for Social Responsibility, and 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.

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, Fellow in Pulmonary Medicine, Lenox Hill Hospital
Lalit K Kanaparthi, MD is a member of the following medical societies: American College of Chest Physicians and American Thoracic Society
Disclosure: Nothing to disclose.

Medical Editor

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.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

CME Editor

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

Carl V Smith, MD, The Distinguished Chris J and Marie A Olson Chair of Obstetrics and Gynecology, Professor, Department of Obstetrics and Gynecology, University of Nebraska Medical Center
Carl V Smith, MD is a member of the following medical societies: American College of Obstetricians and Gynecologists, American Institute of Ultrasound in Medicine, American Medical Association, Arkansas Medical Society, Association of Professors of Gynecology and Obstetrics, Central Association of Obstetricians and Gynecologists, Council of University Chairs of Obstetrics and Gynecology, Nebraska Medical Association, and Society for Maternal-Fetal Medicine
Disclosure: Nothing to disclose.

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