Meigs’Meigs’ SyndromeSyndrome

I. Introduction

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.

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.

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. Samantha 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 StatesOvarian 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.

InternationalPrevalence is unknown.

Mortality/MorbidityAlthough 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.

AgeThe 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.

TreatmentMedicalMedical 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 (salpingo-oophorectomy 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 salpingo-oophorectomy.

The cure rate after either type of surgery is high and recurrence is rare.

III. 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

History of Present illnessFour months prior to admission, the patient claimed that she was suffering from

menstrual irregularity and after two months her monthly period never came. The patient also reported feelings of bloating.

Past Medical History The patient has never been admitted to any hospital except for routine check-ups for

minor complaints like cough and colds.

Family History Birth order of Patient: 2nd

Total no. of siblings: 3

No. of living siblings: 2

Serious diseases/illnesses of siblings: None

Cause of death/serious illnesses of siblings: None

Mother side: Has known history of ovarian cancer.

Father side: No known history of any disease.

IV. Physical Assessment

Positive signs include the following:

Vital signso Tachypnea

o Tachycardia

Lungs

o Dullness to percussion

o Decreased tactile fremitus

o Decreased vocal resonance

o 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.

o Ascites is present, with shifting dullness and/or fluid thrill.

Pelvis: Examination reveals a pelvic mass.

Predisposing factors:Age: 64 years oldGender: Female

Etiology:

Idiopathic

Precipitating factors:Unknown

Ascitic fluid

Irritation to the peritoneal surfaces

Production of peritoneal fluid

Pleural effusion

Direct pressure on surrounding lymphatics

or vessels

Hormonal stimulation

Tumor torsion

Release of mediators like histamines, activated

components and fibrin degradation products

Increased capillary permeability

Through the route of transfer via transdiaphragmatic

lymphatic channels

Benign ovarian tumor

Tumors larger than 10 cm in diameter with

myxoid component to the stroma

Associated with ascites

Favored secretion of fluid from the tumor as the source of the ascites

pain

Lung compression

Weight gain

Increase in abdominal girth Difficulty of breathing

VI. Pathophysiology

VII. Laboratory

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.

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.

o No signs of distant metastasis are observed.

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.