Meigs Syndrome

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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 (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 effusion.

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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.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
o Tachypnea
o

Lungs

Tachycardia

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
o

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.
o

Physiologic sources of CA-125 are fetal coelomic epithelium and its derivatives,
including the following:

Mllerian 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

o

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.
o Serous cystadenoma and mucinous cystadenoma: 15-20% are malignant.
o

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.
o

Mature teratoma

Immature teratoma

Dysgerminoma

Gonadoblastoma

Endodermal sinus

Embryonal carcinoma

Nongestational choriocarcinoma

Gonadal-stromal cell tumors constitute 3-5% of all tumors.

o

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.

o Perform a frozen section of the ovarian mass during exploratory laparotomy. If
the frozen section is consistent with benign tumor, conservative surgery (salpingooophorectomy or oophorectomy) is appropriate.
o

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 salpingooophorectomy.

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.