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Meigs Syndrome
Article Last Updated: Jan 25, 2007
AUTHOR AND EDITOR INFORMATION
Section 1 of 9  
Author: Klaus-Dieter Lessnau, MD, FCCP, Clinical Assistant 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 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
Coauthor(s):
Rajeshwari Chavda, MD, Consulting Staff, Emergency Care Group of Northwest;
Ayesha Akhter, MD, Staff Physician, Department of Internal Medicine, Brooklyn Hospital Center;
Mir Omar Ali, MD, Fellow, Department of Pulmonary Medicine, Lenox Hill Hospital, New York University;
Mir Mustafa Ali, Deccan College of Medical Sciences, Owaisi Hospital and Research Center, Princess Esra Hospital, India
Editors: Jeffrey B Garris, MD, Chief, Assistant Professor, Department of Obstetrics and Gynecology, Division of Urogynecology and Reconstructive Pelvic Surgery, Tulane University School of Medicine; Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine; 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; Frederick B Gaupp, MD, Consulting Staff, Department of Family Practice, Assumption Community Hospital; Lee P Shulman, MD, Professor of Obstetrics and Gynecology, Feinberg School of Medicine, Northwestern University; Chief, Division of Reproductive Genetics, Department of Obstetrics and Gynecology, Prentice Women's Hospital, Northwestern Memorial Hospital
Author and Editor Disclosure
Synonyms and related 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
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 1936, Meigs and Cass described 7 cases of ovarian fibromas associated with ascites and pleural effusion. 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. 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.
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. 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.
History
Patients 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
- 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 CA125 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 CA125 levels. 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 CA125 levels, and hyperthyroidism. 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, CA125 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.
Ascites
Cirrhosis
Colon Cancer, Adenocarcinoma
Hypoalbuminemia
Lung Cancer, Non-Small Cell
Lung Cancer, Oat Cell (Small Cell)
Nephrotic Syndrome
Ovarian Cancer
Pleural Effusion
Tuberculosis
Other Problems to be Considered
Congestive heart failure
Lab Studies
- 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 (CA125) test. Tumor marker serum levels of CA125 can be elevated in Meigs syndrome, but the degree of elevation does not correlate with malignancy. In fact, a normal CA125 level does not exclude the possibility of malignancy. The CA125 level is not used as a screening test. The highest reported level of CA125 after laparotomy is 1808 U/mL. This would be a false-positive result.
- Physiologic sources of CA125 are fetal coelomic epithelium and its derivatives, including the following:
- Müllerian epithelium
- Pleura
- Pericardium
- Peritoneum
- Pathologic conditions related to an elevated CA125 level include the following:
- Pelvic inflammatory disease (PID)
- Peritoneal damage or regeneration (eg, abdominal surgery)
- Ovarian malignancy
- Endometriosis
- In 1992, Jeffery and Lin conducted a study to determine whether the ovarian fibroma was the source of serum CA125 elevation. Using an immunohistochemical technique specific for the tumor marker, they localized CA125 expression in the omentum and peritoneal surfaces rather than in the fibroma.
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 (Pap smear) 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: Fifteen to 20% are malignant.
- Endometrioid type and clear cell: Ninety-five to 98% are malignant.
- Brenner tumor: Two percent 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: Thirty percent are malignant.
- Gynandroblastoma: One hundred percent are malignant.
Medical Care
- 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.
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 CA125 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
Medical/Legal Pitfalls
- This benign condition can be confused with malignant disease because of the presence of ascites and pleural effusion with pelvic mass.
- An elevated serum CA125 level does not always indicate malignancy.
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Meigs Syndrome excerpt Article Last Updated: Jan 25, 2007
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