Meigs Syndrome

What is Meigs syndrome?

Meigs syndrome is associated with a benign tumor in the ovary. Meigs syndrome accompanied with pleural effusion. Pleural effusion is developed due to the accumulation of fluid, which can occur due to excess fluid production and/or due to decrease rate of fluid absorption into the pleural space.  This condition is treated by resectioning the tumor. Another classic feature of Meigs syndrome is Ovarian fibromas, as it is linked up with benign tumors^1,2.

The name of this syndrome comes from Joe Vincent Meigs, who was first provided awareness about this syndrome and finally in 1937 when Meigs and Cass showed the consequences of this syndrome, it is termed as Meigs syndrome¹.

Image 2- Ovary with cyst in Meigs syndrome

 

Image 1 – Plueral Effusion X ray in Meigs Syndrome

It is a complicated form of leimyomas, which is developed in the female genital tract. Leimyomas is a type of commonly occurred ovarian tumor².

Pathophysiology

Meigs syndrome is a rare syndrome with unclear pathophysiology. The presence of mass in overy, ascites, and pleural effusion are usually identified as malignancy. Moreover, an elevated level of serum CA125 with ovarian mass in postmenopausal female also suggested as a malignancy. Serum CA125 is considered as a biomarker for ovarian tumors. In Meigs’ syndrome, the etiology of the ascitic fluid is unclear.

Some researchers predicted that transude of the fluid from the surface of the tumor is the possible reason of ascitic fluid accumulation. Another possible mechanism of development in Meigs’ syndrome is an ascitic fluid production with direct pressure development on the associated lymphatics  vessels, which resulting hormonal stimulation and tumor torsion^6,7.

Sign and Symptoms

The family history of ovarian cancer has a link with Meigs syndrome.

The manifestation  of Meigs syndrome are as follows^2,8:

• Frequent exhaustion
• Shortness of breathing
• Increased diameter of the abdomen
• Unexplained weight loss/ weight gain
• Nonproductive cough
• Flatulence
• Abnormal cessation of menstrual cycle before menopause

Following are signs observed by the doctor  during physical examination2:

Vital signs

• Shallow breathing (tachypnea)
• Increased heart rate (tachycardia)

Lung

• Bass beat becomes dull
• Shrinking of tactile fremitus
• Reduce vocal resonance
• Reduced breath sounds
• Mostly pleural effusion occurs at right side, but can also occur at left sided

Abdominal

• Solid unilateral pelvic mass usually found at left-hand side
• Mass size vary, but commonly large in size
• No mass is felt for small-sized mass

Pelvis

• Presence of pelvic mass

Causes

The exact cause of occurrence of Meigs syndrome is unknown.  But inflammatory reaction leads to accumulation of pleural fluid and/or ascitis⁴.

The presence of ovarian mass is primary findings of Meigs syndrome. The swelling is one of the primary reason of , In addition, elevated the level of serum  level. Histological findings can confirm the presence of malignancy. But the association of malignancy is not always accompanied and that can identify through negative findings of the cytological examination.

The amalgamation of rising of the CA-125 level, ascites and pleural effusion with no tumor but the presence of systemic lupus erythematosus in a patient is either termed as a Tjalma syndrome or due to the migrated Filshie clips a pseudo-Meigs syndrome^1,2.

Pseudo – Meigs syndrome

The tumor types varied in Meigs syndrome, which is the main differentiation between Meigs syndrome and pseudo-Meigs syndrome. In Meigs syndrome, the type of ovarian syndrome is fibroma, whereas in  pseudo-Meigs syndrome cystadenoma, thecoma or a granulosa tumor types are prominent in ovary⁵.

Diagnosis

The simple way to detect Meigs syndrome is chest examination and usual finding is dullness, because of effusion. Moreover, shallow breathing sound and reduced transmitted sound measured by tactile vocal fremitus.

The different imaging diagnostic tools are used for correct detection of Meigs syndrome. The usual diagnostic tools used for the existence of pleural effusion, ascites , and the distinctive ovarian tumor and also for confirming the Meigs syndrome are:

• Ultrasonography,
• Computerized axial tomography (CAT)
• Magnetic resonance imaging (MRI)

The imaging system findings provide the presence of effusion only unilaterally (on the right-hand side) or some cases, it present bilaterally.

Other than these, the following tests are also conducted in postoperative cases to detect the presence of  malignancy of the ovarian tumor that is associated with the syndrome:

• Cytomorphology of the pleural and ascitic liquid
• Estimation of carcinogenic antigen-125 (CA-125) in the serum level http://www.obgyn.net/laparoscopy-and-hysteroscopy/meigs-syndrome-case-presentation-and-revision-literature^1,2,3

The ovarian tumor may prominent or sometimes hidden by ascites. In the case of a malignant tumor, it may spread to surroundings area including colon and lung⁴.

Treatment

The management of the Meigs syndrome requires surgical intervention. But pleural effusion aspiration and ascites may need before operating out the tumor, as this improves pulmonary functionality.

The surgical procedure of full laparotomy usually requires to complete eradication of  probable presence of malignancy, including colon:

• Unilateral salpingo-oophorectomy is preferred for women at reproductive age
• Wedge resection may be preferable for girls before puberty
• Total abdominal hysterectomy with bilateral salpingo-oophorectomy is preferable after menopause^3,4

Prognosis

Surgical intervention for pleural effusion and ascites and removal of this help to lower down the CA 125 level within normal range. The nature of the tumor is benign and the prognosis rate is excellent. Fertility should be preserved for functioning ovarian tissue⁵.

References

1. Humberto Perez Sarria, Carlos del Rosario Zayas Bazan, John Essien, Jessica Bardales Mitac, (2011); Meigs’ Syndrome: a case presentation and revision of the literature; http://www.obgyn.net/laparoscopy-and-hysteroscopy/meigs-syndrome-case-presentation-and-revision-literature
2. Klaus-Dieter Lessnau; Meigs Syndrome Clinical Presentation; Online Available; http://emedicine.medscape.com/article/255450-clinical#b5
3. Riker D, Goba D; Ovarian mass, pleural effusion, and ascites: revisiting Meigs syndrome. J Bronchology Interv Pulmonol. 2013 Jan;20(1):48-51. doi: 10.1097/LBR.0b013e31827ccb35.
4. Dr. Reeja Tharu; Meigs’ Syndrome; Online Available;  http://www.medindia.net/patients/patientinfo/meigs-syndrome.htm
5. Meigs’ Syndrome; Online Available;  http://patient.info/in/doctor/meigs-syndrome
6. Qianhe Liao, Shuhong Hu; Meigs’ Syndrome and Pseudo-Meigs’ Syndrome: Report of Four Cases and Literature Reviews; Journal of Cancer Therapy, 2015, 6, 293-298 Published Online April 2015 in SciRes. http://www.scirp.org/journal/jct http://dx.doi.org/10.4236/jct.2015.64032
7. 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
8. 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