Edit concept Question Editor Create issue ticket

Meigs Syndrome

Meig Syndrome

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.


Presentation

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.

Fishing
  • The detailed cytogenetic and FISH analysis revealed the presence of two subclones with a complex karyotype, 46,XX,t(2;12)(q31;q21),ins(14;12)(q23-24;q15q21).ish del(12)(q15q15) (LL12NC01-142H1-,LL12NC01-27E12-),der(12)t(2;12)(LL12NC01-142H1 ,LL12NC01-[ncbi.nlm.nih.gov]
Pleural Effusion
  • Because the transdiaphragmatic lymphatic channels are larger in diameter on the right, the pleural effusion is classically on the right side. The etiologies of the ascites and pleural effusion are poorly understood.[ncbi.nlm.nih.gov]
  • Because the transdiaphragmatic lymphatic channels are larger in diameter on the right, the pleural effusion is classically on the right side. The causes of the ascites and pleural effusion are poorly understood.[en.wikipedia.org]
  • Subsequently, ascites and bilateral pleural effusion resolved rapidly.[ncbi.nlm.nih.gov]
  • The classification of pleural effusion with the use of Light's criteria was possible in only 7 patients. In 6 of these patients pleural effusion met the criteria for an exudate.[ncbi.nlm.nih.gov]
  • She also had pleural effusion. Because echocardiography revealed Ebstein anomaly, medical treatment was performed, under the diagnosis of heart failure. However, her pleural effusion and ascites did not completely disappear.[ncbi.nlm.nih.gov]
Increased Abdominal Girth
  • abdominal girth, bloating, intermittent abdominal pain, dyspnea, nonproductive cough may help in differentiating potential local factor causing such symptoms.[en.wikipedia.org]
  • abdominal girth over the past month.[medbullets.com]
Abdominal Fullness
  • A 41-year-old woman was admitted for investigation of abdominal fullness and dyspnea. Preoperative examinations revealed a huge pelvic tumor, adenocarcinoma of the sigmoid colon, marked ascites, and bilateral pleural effusion.[ncbi.nlm.nih.gov]
  • Abstract A 50-year-old woman was admitted because of abdominal fullness due to bilateral ovarian tumors, pleural effusion, and ascites associated with breast cancer.[ncbi.nlm.nih.gov]
Irritability
  • An elevated serum level of CA-125 is also unusual and it has been thought that it is the consequence of physical irritation and inflammation.[ncbi.nlm.nih.gov]
  • It was initially suggested that irritation of the peritoneal surfaces by a hard, solid ovarian tumor could stimulate peritoneal fluid production.[radiopaedia.org]
  • Meigs suggested that irritation of the peritoneal surfaces by a hard, solid ovarian tumor could stimulate the production of peritoneal fluid.[www1.cgmh.org.tw]
  • It has also been proposed that the tumor may irritate the peritoneum thereby causing it to secrete copious amounts of fluid into the cavity or that hormonal changes and inflammatory mediators associated with the tumor may lead to edema.[healthhype.com]

Workup

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 [6]. It can also be elevated in pleural and ascitic fluids but normal levels of CA-125 can also be present even in malignancy [7] [8]. 

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 [9].

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.

Pleural Effusion
  • Because the transdiaphragmatic lymphatic channels are larger in diameter on the right, the pleural effusion is classically on the right side. The etiologies of the ascites and pleural effusion are poorly understood.[ncbi.nlm.nih.gov]
  • Because the transdiaphragmatic lymphatic channels are larger in diameter on the right, the pleural effusion is classically on the right side. The causes of the ascites and pleural effusion are poorly understood.[en.wikipedia.org]
  • Subsequently, ascites and bilateral pleural effusion resolved rapidly.[ncbi.nlm.nih.gov]
  • The classification of pleural effusion with the use of Light's criteria was possible in only 7 patients. In 6 of these patients pleural effusion met the criteria for an exudate.[ncbi.nlm.nih.gov]
  • She also had pleural effusion. Because echocardiography revealed Ebstein anomaly, medical treatment was performed, under the diagnosis of heart failure. However, her pleural effusion and ascites did not completely disappear.[ncbi.nlm.nih.gov]
Pleural Exudate
  • When the protein concentration 3.0 g/dL was applied as a criterion of pleural exudate, 88.8% (80/90) of effusions were classified as exudates.[ncbi.nlm.nih.gov]

Treatment

Surgery is the cornerstone of treatment as Meigs syndrome is a benign condition that usually fully resolves after resection of the tumor [10]. 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. 

Prognosis

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.

Etiology

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 [7]. 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. 

Epidemiology

Meigs syndrome is a rare condition and is very uncommon before the age of 40 [5]. 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%.

Sex distribution
Age distribution

Pathophysiology

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.

Prevention

There are no current preventive measures for Meigs syndrome.

Summary

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 [1]. 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 [2] [3] [4].

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.

Patient Information

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).

References

Article

  1. Riker D, Goba D. Ovarian mass, pleural effusion, and ascites: revisiting meigs syndrome. J Bronchology Interv Pulmonol. 2013 Jan; 20(1):48-51. 
  2. 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. 
  3. Iavazzo C, Vorgias G, Sampanis D, et al. Meig's or Pseudomeig's syndrome? Bratisl Lek Listy. 2007; 108(3):158-60.
  4. Schmitt R, Weichert W, Schneider W, Luft FC, Kettritz R. Pseudo-pseudo Meigs' syndrome. Lancet. 2005 Nov 5; 366(9497):1672.
  5. Agaba EI, Ekwempu CC, Ugoya SO, et al; Meigs' syndrome presenting as haemorrhagic pleural effusion. West Afr J Med. 2007 Jul-Sep; 26(3):253-5.
  6. Moran-Mendoza A, Alvarado-Luna G, Calderillo-Ruiz G, et al. Elevated CA125 level associated with Meigs' syndrome: case report and review of the literature. Int J Gynecol Cancer. 2006 Jan-Feb; 16(1):315-8
  7. Jones OW, Surwit EA. Meigs syndrome and elevated CA 125. Obstet Gynecol. 1989 Mar. 73(3 Pt 2):520-1.
  8. 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.
  9. Lanitis S, Sivakumar S, Behranwala K, et al. A case of Meigs syndrome mimicking metastatic breast carcinoma. World J Surg Oncol. 2009 Jan; 22: 7-10.
  10. Samanth KK, Black WC. Benign ovarian stromal tumors associated with free peritoneal fluid. Am J Obstet Gynecol. 1970 Jun 15; 107(4):538-45.

Ask Question

5000 Characters left Format the text using: # Heading, **bold**, _italic_. HTML code is not allowed.
By publishing this question you agree to the TOS and Privacy policy.
• Use a precise title for your question.
• Ask a specific question and provide age, sex, symptoms, type and duration of treatment.
• Respect your own and other people's privacy, never post full names or contact information.
• Inappropriate questions will be deleted.
• In urgent cases contact a physician, visit a hospital or call an emergency service!
Last updated: 2018-06-22 10:59