Meigs syndrome describes the occurrence of a solid benign ovarian tumor, usually a fibroma, along with the presence of ascites and pleural effusions. The majority of cases usually resolve after surgical resection of the ovarian mass.
Patients with Meigs syndrome usually present with symptoms related to pleural effusions and ascites and, in case of premenopausal women, with menstrual symptoms. They can also a have a family history of ovarian cancer. The most common presenting symptoms include fatigue, dyspnea that is initially present only with exertion, weight gain associated with swelling in the abdomen, a dry cough and menstrual symptoms such as amenorrhea or irregular cycles.
On physical exam, they may also have the following signs: tachypnea, tachycardia, decreased tactile fremitus, decreased breath sounds mostly on the right side but can also be present on the left, decreased vocal resonance and a shifting dullness or a fluid thrill in the abdomen indicating the presence of ascites. The majority of patients also present with a large, solid mass in the pelvis that is usually not associated with any symptoms and that is most commonly left sided but can also be bilateral.
Workup of patients with Meigs syndrome is broad and involves a range of tests to detect abnormalities in laboratory values as well as imaging and procedural tests to localize and identify the tumors involved. A complete blood count is required and low hemoglobin should prompt additional investigations. It is mostly associated with iron deficiency anemia, thus the need to measure reticulocyte count, serum iron levels, ferritin and total iron binding capacity. Anemia prior to surgery is an emergency and can be immediately treated with blood transfusions. Iron therapy can be then administered postoperatively.
A basic metabolic profile is necessary prior to surgery and includes measurements of sodium, chloride, potassium, blood urea nitrogen, bicarbonate, creatinine and glucose serum levels. Prothrombin time is assessed to detect any possible coagulopathies and, if present, fresh frozen plasma or vitamin K injections are administered. Serum cancer antigen (CA-125), a marker for several tumors (particularly ovarian tumors) is usually elevated although its increase is not related to malignancy . It can also be elevated in pleural and ascitic fluids but normal levels of CA-125 can also be present even in malignancy  .
Diagnostic imaging tests include a chest X-ray to detect any pleural effusions, an ultrasound of of the abdomen and the pelvis to identify ascites as well as ovarian masses and a computed tomography (CT) to rule out distant metastasis. The CT may additionally detect tumors in the ovaries, uterus, fallopian tubes or broad ligament masses .
Therapeutic and diagnostic procedures include a paracentesis or an aspiration of pleural fluids. These usually help in relieving symptoms and make it possible to distinguish malignant ascites from Meigs syndrome. Ascitic and pleural fluids are commonly transudative but they can also be exudative, and protein content is frequently elevated with ovarian cancer. Samples from the ascites and the pleural effusions are sent for laboratory tests to evaluate the levels of protein, glucose, amylase, cell counts and the presence of microorganisms.
Surgery is the cornerstone of treatment as Meigs syndrome is a benign condition that usually fully resolves after resection of the tumor . Surgical treatment starts with an exploratory laparotomy and a frozen section of the tumor is sent for pathological analysis. If the pathology report indicates absence of malignant features, the surgeon can proceed with a salpingo-oophorectomy or an oophorectomy, although the choice of the procedure depends on the age and reproductive status of the woman. Premenopausal women usually undergo a unilateral salpingo-oophorectomy, while postmenopausal women can undergo a bilateral salpingo-oophorectomy with total hysterectomy or a bilateral or unilateral salpingo-oophorectomy. A wedge resection of the ovaries along with a unilateral salpingo-oophorectomy is the treatment of choice in prepubertal girls.
An ovarian mass that is associated with elevated levels of CA-125 is malignant until proven otherwise. Malignancy is ruled out with negative cytology, benign histological reports and absence of any peritoneal implantation. It is critical to consult a gynecologic surgeon and a gynecologic oncologist during the treatment process.
Non-surgical treatment with thoracentesis and paracentesis can additionally help in relieving symptoms.
Meigs syndrome is a benign disease with an excellent prognosis if adequately diagnosed and treated, even when some features resemble more ominous malignant disorders. Patients with Meigs syndrome also tend to have similar life expectancy to patients in the general population. Fertility is usually not affected if the ovaries are still functional. It is important to note that the resolution of ascites and pleural effusions after surgery is a defining characteristic of the disease.
The causative mechanisms responsible for pleural effusions in Meigs syndrome remain unknown and seem to be independent of the amount of ascites. Samanth and Black propose that the ascitic fluid is directly secreted from the tumors and report that ovarian tumors only larger than 10 cm and with a myxoid component are associated with ascites . On the other hand, Efskind and Terada suggest that pleural effusions result from the passage of fluid in the lymphatic channels through the diaphragm. Other mechanisms that are thought to be involved include the presence of mechanical pressure on the lymphatic channels and vessels, hormonal activation and tumor torsion.
Meigs syndrome is a rare condition and is very uncommon before the age of 40 . It becomes more frequent with age although some studies report teratomas and cystadenomas in prepubertal girls. The incidence of ovarian tumors is more elevated in women with higher socioeconomic status. It increases in the third decade of life and keeps on progressively increasing after menopause, with an average of 50 years of age. Ovarian fibromas represent 2% to 5% of tumors removed surgically, with Meigs syndrome occurring in 1% to 2% of them. Ascites occurs in 10-15% of women with ovarian fibromas while hydrothorax is associated with larger lesions and has an incidence of 1%. Life expectancy in Meigs syndrome is similar to the general population after surgical removal of the tumor and the risk of progression from fibroma into fibrosarcoma is 1%.
The pathophysiology underlying ascites remains uncertain. It is hypothesized that contact of the peritoneal surface with a hard mass such as an ovarian solid tumor stimulates peritoneal fluid production. The development of ascites can also be caused by a secretion of mediators that increase capillary permeability such as activated complements, histamines and fibrin degradation products.
The fluids that collect within the pleura and the peritoneum can either be transudative or exudative. Both pleural and ascitic fluids tend to have a similar composition. It is usually tumor size and not its histological features that determines the presence and consistency of pleural effusions and ascites.
There are no current preventive measures for Meigs syndrome.
Meigs syndrome is defined by the simultaneous presence of a solid ovarian tumor along with ascites and pleural effusions. In the majority of cases, the tumor involved is a fibroma. A diagnosis of Meigs syndrome can only be established after a complete exclusion of other potential malignant causes . Pseudo-Meigs is a similar condition that involves benign tumors other than fibromas. These tumors are not limited to the ovaries and may be present in the uterus or fallopian tubes and may also consist of mature teratomas, struma ovarii or ovarian leiomyomas. Some cases involve ovarian or gastrointestinal metastatic tumors   .
The underlying etiological and physiological mechanisms are still not completely elucidated. Some hypothesize that pleural effusions result from the passage of ascitic fluid through transdiaphragmatic channels. On the other hand, ascites is thought to be caused direct pressure from the ovarian tumor over the peritoneum. Neurochemical mediators such as histamine, fibrin degradation products and activated complements are also involved. These increase vascular permeability and ultimately lead to fluid extravasation within the peritoneum.
Incidence and prevalence of Meigs syndrome are low, with the condition occurring in only 1 to 2% of all cases with ovarian fibromas. The latter constitute 2 to 5% of ovarian malignancies treated surgically.
Patients with Meigs syndrome usually present with symptoms that are related to the ascites and the pleural effusions such as weight gain, fatigue, shortness of breath or abdominal swelling. Physical exam shows tachypnea, tachycardia, decreased breath sounds and tactile fremitus. Abdominal exam reveals fullness and dullness over the flank area because of the presence of ascites. Diagnosis is established after performing a biopsy and ruling out all malignant causes. Although a paracentesis or a pleural aspiration can provide symptomatic relief, surgical resection of the tumor is the cornerstone of the treatment. The type of surgery performed depends on the age of the patient as well as her reproductive status.
Meigs syndrome is defined by the presence of an ovarian tumor along with an accumulation of fluid in the abdomen (ascites) and effusions around the lungs (pleural effusions). The ovarian tumor involved in Meigs syndrome is usually a fibroma, a benign tumor. Cases that involve other types of ovarian tumors, such as a thecoma, cystadenoma or a granulosa cell tumor, are called pseudo-Meigs syndrome. The mechanisms underlying the development of Meigs syndrome are still not well understood, although it is thought that the ascites and the pleural effusions result from inflammatory reactions triggered by the tumor. Most women affected are older than 40, with the risk further increasing with age. Very rare tumors in prepubertal girls have also been reported.
Patients with Meigs syndrome usually present with symptoms related to the ascites and the pleural effusions. These range from shortness of breath, fatigue and swelling in the abdomen to weight gain and irregular menses. Physical exam is important for the diagnosis of Meigs syndrome. Dullness to percussion of the chest, decreased breath sounds and decreased tactile fremitus (transmitted sounds in the lungs) can indicate pleural effusions. Careful abdominal and pelvic examinations may detect a tumor beneath the ascites. Ascites usually manifests with fullness and dullness in the flank and abdominal areas.
Laboratory and imaging tests are broad and aim to detect the ovarian tumor as well as to characterize the ascitic and pleural fluids. Routine blood tests such as a complete blood count, liver function tests and assessment of proteins in the blood are performed. In addition, a specific marker called CA-125 is measured and is usually more elevated in ovarian cancer. A urinalysis can also be performed to detect proteins in the urine. Imaging tests such as an ultrasound, a CT scan or an MRI of the abdomen and pelvis can reveal the ascites and confirm the presence of the tumor. A chest X-ray may also detect the pleural effusions.
Procedural tests such as aspiration of pleural and ascitic fluids can relieve the bothering symptoms as well as provide samples of the fluid that may be sent for further analyses. Cytologic tests on these samples help in distinguishing Meigs syndrome from frank malignant conditions.
Surgery is the cornerstone of treatment and the disease commonly resolves after the ovarian mass is removed. The type of surgery depends on the age and reproductive status of the women. A wedge section of the ovaries is usually performed in girls before the age of pubery whereas women of reproductive age undergo a resection of the ovary and associated fallopian tube (salpingo-oophorectomy). Women who are in the menopausal period can additionally undergo a full removal of the uterus, both ovaries and the fallopian tubes (bilateral salpingo-oophorectomy with hysterectomy).