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INTRODUCTION |
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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 might 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 mature
teratomas, struma-ovarii, leiomyoma of ovary, and benign tumors of the fallopian
tube or uterus.
Atypical Meigs: Two case reports have been made of atypical Meigs
characterized by a benign pelvic mass with right-sided pleural effusion but
without ascites. As in Meigs syndrome, pleural effusion resolves after removal
of pelvic mass.
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Pathophysiology:
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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 myxoid component to the stroma
are associated with ascites. These authors believe that their observations
favored 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 theorize that ascitic fluid is transferred via
transdiaphragmatic lymphatic channels. The size of the pleural effusion is
largely independent of the amount of ascites.
Study by Efskind: Efskind injected ink into the lower abdomen of a woman with
Meigs syndrome and found that, within half an hour, the ink particles had
accumulated in the lymphatics of the pleural surface. Blockage of these
lymphatics prevented accumulation of pleural fluid and caused an increase in
ascitic fluid.
Study by Terada: In 1992, Terada et al injected labeled albumin into the
peritoneum and found that within 3 hours the maximum concentration was detected
in the right pleura.
Nature of the ascitic and pleural fluid: Ascitic fluid and pleural fluid in
Meigs syndrome can be either transudate or exudate. 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.
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Frequency:
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- In the US: 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% of them.
- Internationally: Prevalence is unknown.
Mortality/Morbidity: Meigs syndrome, being a benign
condition, has a very good prognosis if properly managed, though it mimics a
malignant condition. Life expectancy after surgical removal of the tumor is the
same as in the general population.
Age: Incidence of ovarian tumor begins to increase in the
third decade and increases progressively to peak in the seventh decade. Case
reports have been made of Meigs syndrome in prepubertal girls with benign
teratomas and cystadenomas.
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CLINICAL |
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History: Patients may have a family
history of ovarian cancer. The chief complaints are vague and generally present
over a period of time.
- Increased abdominal girth
- Amenorrhea for premenopausal women
Physical: Positive signs include the following:
- Decreased tactile and vocal fremitus
- Decreased breath sounds are noted, suggesting pleural effusion. Pleural
effusion is seen mostly on the right side but can also be left-sided.
- Examination can be positive for a pelvic mass, small or large, or no
mass may be felt.
- Ascites will be present, with shifting dullness and/or fluid thrill.
- Pelvis: Examination reveals a pelvic mass.
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DIFFERENTIALS |
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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
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WORKUP |
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Lab Studies:
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- Complete blood count: This provides information about levels of
hemoglobin, hematocrit and platelets. A low hemoglobin count requires further
workup, including reticulocyte count, total iron-binding capacity, and iron
and ferritin levels. If anemia is present in patients with Meigs syndrome it
is most likely due to iron deficiency. Anemia can be corrected emergently by
blood transfusion in those 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.
Corrections of these electrolytes are made, if necessary.
- 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 giving vitamin K to the patient or by transfusing fresh
frozen plasma.
- Other than serum electrolytes and complete blood count, the lab test of
interest is serum CA 125. Tumor marker serum CA 125 can be elevated in Meigs
syndrome, but the degree of elevation does not correlate with malignancy. CA
125 level is not used as a screening test. (The highest reported level of CA
125 after laparotomy was 336 U/mL).
- Physiologic sources of CA 125 are fetal coelomic epithelium and its
derivatives, including the following:
- Mulerian epithelium
- Pleura
- Pericardium
- Peritoneum
- Pathologic conditions related to elevated CA 125
- Pelvic inflammatory disease (PID)
- Peritoneal damage or regeneration, eg, abdominal surgery
- Ovarian malignancy
- In 1992, Jeffery and Lin carried out a study to determine whether the
ovarian fibroma was the source of serum CA 125 elevation. They localized CA
125 expression in the omentum and peritoneal surfaces, rather than fibroma,
using immunohistochemical technique specific for the tumor marker.
Imaging Studies:
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- 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 seen.
Procedures:
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- Paracentesis: Ascitic fluid mostly is transudative. Fluid is negative for
malignant cells but can be positive for reactive mesothelial cells.
- Thoracentesis: Pleural fluid usually is transudative. It can be exudative
and negative for malignant cells.
- Papanicolaou smear: Smear is normal.
Histologic Findings: Ovarian tumors are divided into the
following histologic subgroups, and Meigs syndrome can be seen 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 teratoma and
gonadoblastoma, which are always benign.
- Mature teratoma
- Immature teratoma
- Dysgerminoma
- Gonadoblastoma
- Endodermal sinus
- Embryonal carcinoma
- Nongestational choriocarcinoma
- Gonadal-stromal cell tumor: These constitute 3-5% of all tumors.
- Granulosa cell
- Fibroma (<5% malignant)
- Thecoma (<5% malignant)
- Sertoli-Leydig cell (<5% malignant)
- Lipid cell type (30% malignant)
- Gynandroblastoma (100% malignant)
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TREATMENT |
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Medical Care:
- Provide symptomatic relief of ascites and pleural effusion by means of
therapeutic paracentesis and thoracentesis.
Surgical Care:
- In women of reproductive age, perform unilateral salpingo-oophorectomy.
- In postmenopausal women, options include bilateral salpingo-oophorectomy
with total hysterectomy and unilateral salpingo-oophorectomy.
- In prepubertal girls, options include wedge resection of ovary and
unilateral salpingo-oophorectomy.
Consultations: Gynecologic-surgical consult for surgical
management of the case
Activity: Activity as tolerated
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FOLLOW-UP |
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Further Inpatient Care:
Further Outpatient Care:
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- As described by Meigs, ascites and pleural effusion dramatically resolve
within a few weeks to months after removal of the pelvic mass without any
recurrence.
- The serum CA 125 level also returns to normal levels after surgery.
Prognosis:
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- Life expectancy of patients with Meigs syndrome is the same as in the
general population after surgery.
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MISCELLANEOUS |
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Medical/Legal Pitfalls:
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- This benign condition can be confused with malignant disease because of
the presence of ascites and pleural effusion with pelvic mass.
- An elevated serum CA 125 does not always indicate malignancy.
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BIBLIOGRAPHY |
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- Aoshima M, Tanaka H, Takahashi M, et al: 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].
- 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].
- Jones OW, Surwit EA: Meigs syndrome and elevated CA 125. Obstet Gynecol
1989 Mar; 73(3 Pt 2): 520-1[Medline].
- Lacson AG, Alrabeeah A, Gillis DA, et al: Secondary massive ovarian edema
with Meig's syndrome. Am J Clin Pathol 1989 May; 91(5): 597-603[Medline].
- Lin JY, Angel C, Sickel JZ: Meigs syndrome with elevated serum CA 125.
Obstet Gynecol 1992 Sep; 80(3 Pt 2): 563-6[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.
- 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].