Meigs syndrome is defined as the triad of benign ovarian tumor with ascites

andpleural effusion that resolves after resection of the tumor. Ovarian fibromas
constitute the majority of the benign tumors seen in Meigs syndrome. Meigs
syndrome, however, is a diagnosis of exclusion, only after ovarian carcinoma is
ruled out.
[1]
n 1!"#, $almon described the association of pleural effusion with benign pelvic
tumors. n 1!"%, Meigs and &ass described % cases of ovarian fibromas associated
with ascites and pleural effusion.
[']
n 1!(#, 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. )istologically, the benign ovarian tumor may be a fibroma, thecoma,
cystadenoma, or granulosa cell tumor.
*seudo+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. .hese benign tumors include those of the fallopian tube or uterus and
mature teratomas, struma ovarii, and ovarian leiomyomas.
["]
.his terminology
sometimes also includes ovarian or metastatic gastrointestinal malignancies.
&hest radiograph showing left+sided pleural effusion.
/typical Meigs characteri0ed by a benign pelvic mass with right+sided pleural
effusion but without ascites has been reported at least twice. /s in Meigs
syndrome, pleural effusion resolves after removal of the pelvic mass.
*seudo+pseudo Meigs syndrome includes patients with systemic lupus
erythematosus and enlarged ovaries.
[#]
*athophysiology
/scites is present in 11+1(2 of cases, and hydrothorax is found in only 12 of
cases.
[(, 3]
Etiology of ascitic fluid
.he 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. $amanth and 4lac5 studied
ovarian tumors accompanied by ascites and found that only tumors larger than 11
cm in diameter with a myxoid component to the stroma are associated with
ascites.
[%]
.hese 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. 6evelopment 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
.he etiology of pleural effusion is unclear. 7fs5ind and .erada et al theori0e that
ascitic fluid is transferred via transdiaphragmatic lymphatic channels. .he si0e of
the pleural effusion is largely independent of the amount of ascites. .he pleural
fluid may be located on the left side or may be bilateral.
[1]
7fs5ind8s study9 7fs5ind injected in5 into the lower abdomen of a woman
with Meigs syndrome and found that the in5 particles accumulated in the
lymphatics of the pleural surface within half an hour. 4loc5age of these
lymphatics prevented accumulation of pleural fluid and caused an increase in
ascitic fluid.
.erada and colleagues8 study9 n 1!!', .erada and colleagues injected
labeled albumin into the peritoneum and found that the maximum concentration
was detected in the right pleura within " hours.
Nature of the ascitic and pleural fluid
/scitic 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. .umor si0e, rather than the
specific histologic type, is thought to be the important factor in the formation of
ascites and accompanying pleural effusion.
7pidemiology
Frequency
United States
Ovarian tumors are more prevalent in women in upper socioeconomic groups.
Ovarian fibromas represent approximately '+(2 of surgically removed ovarian
tumors, and Meigs syndrome occurs in only 1+'2 of these cases: thus, it is a rare
condition. /scites is present in 11+1(2 of women with ovarian fibroma, and
hydrothorax is present in 12, especially those with larger lesions.
International
*revalence is un5nown.
Mortality/Morbidity
/lthough Meigs syndrome mimics a malignant condition, it is a benign disease
and has a very good prognosis if properly managed. ;ife expectancy after surgical
removal of the tumor mirrors that of the general population.
Age
.he incidence of ovarian tumor begins to increase in the third decade and
increases progressively in postmenopausal women, with an average of about (1
years.
[1]
Meigs syndrome in prepubertal girls with benign teratomas and
cystadenomas has been reported
)istory
*atients with Meigs syndrome may have a family history of ovarian cancer. .he
chief complaints are vague and generally manifest over time.
<atigue
$hortness of breath
ncreased abdominal girth
=eight gain>weight loss
?onproductive cough
4loating
/menorrhea for premenopausal women
Menstrual irregularity
*hysical
*ositive signs include the following9
@ital signs + .achypnea, tachycardia
;ungs + 6ullness to percussion: decreased tactile fremitus: decreased vocal
resonance: decreased breath sounds, suggesting pleural effusion, which is mostly
observed on the right side but can also be left sided
/bdomen + Most patients present with an asymptomatic, solid, and
unilateral pelvic mass, most often left sided: the mass may be large,
[(]
but
sometimes, no mass is felt: ascites is present, with shifting dullness and>or fluid
thrill
*elvis - 7xamination reveals a pelvic mass
&auses
=hen an ovarian mass is associated with Meigs syndrome and an elevated &/+
1'( serum level, a malignant process may be suspected until proven otherwise
histologically. / negative cytologic examination result of ascitic effusion, the
absence of peritoneal implantation, and benign histology should limit surgical
procedures. .his decision should be made by an experienced gynecologic surgeon
or a gynecologic oncologist.
&ase reports exist of pseudo+Meigs syndrome associated with
malignantstruma ovarii and elevated &/+1'( levels.
[A, !]
.he choice of not
performing adjuvant therapy is feasible after optimal surgery and adeBuate
staging procedure given to the usually clinical benign course and the low
incidence of metastases in malignant struma ovarii. &areful patient counseling is
reBuired.
$truma ovarii is a rare cause of ascites, hydrothorax, elevated &/+1'(
levels, and hyperthyroidism.
[!]
.his rare condition should be considered in the
differential diagnosis in patients with ascites and pleural effusions but with
negative cytologic test results.
.he combination of ascites, pleural effusion, &/+1'( level elevation, and
no tumor in a patient with systemic lupus erythematosus is either a .jalma
syndrome or due to the migrated <ilshie clips a pseudo+Meigs syndrome
6ifferential 6iagnoses
/scites
&irrhosis
&olon &ancer, /denocarcinoma
)ypoalbuminemia
;ung &ancer, ?on+$mall &ell
;ung &ancer, Oat &ell ,$mall &ell-
Malignant 7ffusion
Milroy 6isease
?ephrotic $yndrome
Ovarian &ancer
*leural 7ffusion
.uberculosis
;aboratory $tudies
;ab studies for patients with Meigs syndrome include the following9
CBC count
.his study provides information about hemoglobin, hematocrit, and platelet
levels. / low hemoglobin count reBuires further wor5up, including reticulocyte
count, total iron+binding capacity, and iron and ferritin levels. /nemia in patients
with Meigs syndrome is most li5ely due to iron deficiency. /nemia can be
corrected emergently by blood transfusion in patients undergoing surgery for
Meigs syndrome. /nemia can be treated with iron supplementation
postoperatively.
Basic metabolic profile
$tudies of sodium, potassium, chloride, bicarbonate, blood urea nitrogen,
creatinine, and glucose levels are included. .hese electrolytes are chec5ed before
the patient undergoes surgery. f necessary, corrections of these electrolytes are
made.
rothrombin time
*rothrombin time is chec5ed before surgery. f elevated, it is a mar5er of
coagulopathy. 7levated prothrombin time is corrected before surgery, either by
administering vitamin C to the patient or by transfusing fresh fro0en plasma.
!erum cancer antigen "#$ test
Other than serum electrolytes and &4& count, the study of interest is the serum
cancer antigen 1'( ,&/+1'(- test. .umor mar5er serum levels of &/+1'( can be
elevated in Meigs syndrome, but the degree of elevation does not correlate with
malignancy. n fact, a normal &/+1'( level does not exclude the possibility of
malignancy.
[11]
.he &/+1'( level is not used as a screening test.
mmunohistochemical studies suggest that serum &/+1'( elevation in patients
with Meigs syndrome is caused by mesothelial expression of the antigen rather
than by fibroma.
[1]
.he highest reported level of &/+1'( after laparotomy is 1A1A
D>m;. .his would be a false+positive result.
*hysiologic sources of &/+1'( are fetal coelomic epithelium and its derivatives,
including the following9
MEllerian epithelium
*leura
*ericardium
*eritoneum
*athologic conditions related to an elevated &/+1'( level include the following9
*elvic inflammatory disease ,*6-
*eritoneal damage or regeneration ,eg, abdominal surgery-
Ovarian malignancy
7ndometriosis
n 1!!', ;in et al conducted a study to determine whether the ovarian fibroma
was the source of serum &/+1'( elevation. Dsing an immunohistochemical
techniBue specific for the tumor mar5er, they locali0ed &/+1'( expression in the
omentum and peritoneal surfaces rather than in the fibroma.
[1']
maging $tudies
&hest radiography confirms pleural effusion.
/bdominal and pelvic ultrasound confirms the ovarian mass and ascites.
C% scan of the abdomen and pel&is
&. scan confirms ascites and ovarian, uterine, fallopian tube, or broad
ligament mass.
?o signs of distant metastasis are observed.
Other .ests
*apanicolaou test findings are normal.
*rocedures
*aracentesis9 /scitic fluid is mostly transudative. <indings are negative for
malignant cells but can be positive for reactive mesothelial cells.
.horacentesis9 *leural fluid is usually transudative. <indings can be
exudative and negative for malignant cells.
istologic <indings
Ovarian tumors are divided into the following histologic subgroups, and Meigs
syndrome can be observed with any of the benign tumors.
Coelomic epithelial tumors
.hese tumors, which originate from the coelomic epithelium, constitute A1+A(2
of all ovarian tumors.
$erous cystadenoma and mucinous cystadenoma9 1(+'12 are malignant.
7ndometrioid type and clear cell9 !(+!A2 are malignant.
4renner tumor9 '2 are malignant.
'erm cell tumors
.hese tumors originate from the germ cell and constitute 11+1(2 of all ovarian
tumors. /ll are malignant except mature teratomas and gonadoblastomas, which
are always benign.
Mature teratoma
mmature teratoma
6ysgerminoma
Fonadoblastoma
7ndodermal sinus
7mbryonal carcinoma
?ongestational choriocarcinoma
'onadal-stromal cell tumors
Fonadal+stromal cell tumors constitute "+(2 of all tumors.
Franulosa cell
<ibroma9 <ewer than (2 are malignant.
.hecoma9 <ewer than (2 are malignant.
$ertoli+;eydig cell9 <ewer than (2 are malignant.
;ipid cell type9 "12 are malignant.
Fynandroblastoma9 1112 are malignant.
Medical &are
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.
$urgical &are
7xploratory laparotomy with surgical staging is the treatment of choice.
*erform a fro0en section of the ovarian mass during exploratory laparotomy. f the
fro0en section is consistent with benign tumor, conservative surgery ,salpingo+
oophorectomy or oophorectomy- is appropriate. <indings of lymph node biopsies
and omentum and pelvic washings are negative for malignancy if these procedures
are performed during surgery.
n women of reproductive age, perform unilateral salpingo+oophorectomy.
n postmenopausal women, options include bilateral salpingo+
oophorectomy with total hysterectomy and unilateral or occasionally bilateral
salpingo+oophorectomy.
n prepubertal girls, options include wedge resection of ovary and
unilateral salpingo+oophorectomy.
.he cure rate after either type of surgery is high and recurrence is rare.
&onsultations
&onsult with a gynecologic surgeon for surgical management of the patient.
/ctivity
*atients can maintain activities as tolerated.
<urther npatient &are
Observe standard postsurgical management protocols.
<urther Outpatient &are
/s described by Meigs, ascites and pleural effusion resolve dramatically within a
few wee5s to months after removal of the pelvic mass, without any recurrence.
Dse of chest ultrasound to follow pleural effusion progression is superior to chest
radiography in identifying residual pleural effusion and can detect amounts as
small as "+( m;.
[1]
.he serum &/+1'( level also returns to normal after surgery
*rognosis
;ife expectancy of patients with Meigs syndrome mirrors that of the general
population after surgery, and less than 12 of fibromas progress to fibrosarcoma
*atient 7ducation
<or excellent patient education resources, visit eMedicine)ealth8s &ancer &enter.
/lso, see eMedicine)ealth8s patient education article Ovarian &ancer
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