Meigs Syndrome: Etiopathophysiological basis of Clinical Manifestations and Medico Surgical and Nursing Modalities.

 

Mrs. Sumangala B R

Lecturer, Department of Obstetrics and Gynecological Nursing, SDM Institute of Nursing Sciences, Sattur, Dharwad, 580009, Karnataka, India

 

ABSTRACT:

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]In 1934, Salmon described the association of pleural effusion with benign pelvic tumors. In 1937, Meigs and Cass described 7 cases of ovarian fibromas associated with ascites and pleural effusion.^[2] The syndrome was named as Meig’s syndrome by Rhoads and Terrel in 1937.^[3] 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. Histological, the benign ovarian tumor may be a fibroma, thecoma, cystadenoma, or granulosa cell tumor. Pseudo-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. These benign tumors include those of the fallopian tube or uterus and mature teratomas, struma ovarii, and ovarian leiomyomas.^[4] 

 

 

 

INTRODUCTION:

The ovarian tumor is usually a fibroma but may be a thecoma, cyst adenoma, or granulosa cell tumor. Meigs' syndrome is rare condition where triad of benign ovarian tumor with ascites and pleural effusionIt is very uncommon before 40 years of age and becomes more frequent as the years progress but there are some reports of it arising from teratomas or cystadenomas in pre-pubertal girls.

 

Most features are related to ascites and pleural effusion but before the menopause there may be menstrual symptoms too like Fatigue, Dyspnoea (initially on exertion), and swollen abdomen with associated weight gain, Non-productive cough, Amenorrhea or irregular menstruation. If the tumor is resected, the fluid resolves.^[5]

 

Definition:

Meigs' syndrome is the rare condition which is a triad of ascites, pleural effusion, and benign ovarian tumor and Atypical Meigs characterized by a benign pelvic mass with right-sided pleural effusion. Atypical Meigs characterized by a benign pelvic mass with right-sided pleural effusion but without ascites has been reported at least twice. As in Meigs syndrome, pleural effusion resolves after removal of the pelvic mass. Pseudo-pseudo Meigs syndrome includes patients with systemic lupus erythematosus and enlarged ovaries.^[5]

 

Etio-Pathophysiology:

Ascites is present in 10-15% of cases, and hydrothorax is found in only 1% of cases.^{[6, 7]}

 

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. Ovarian tumors accompanied by ascites and found that only tumors larger than 10 cm in diameter with a myxoid component to the stroma are associated with ascites.^[8] 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.

 

Pleural Effusion:

The etiology of pleural effusion is unclear. Efskind and Terada et al theorize that ascitic fluid is transferred via Trans diaphragmatic lymphatic channels. The size of the pleural effusion is largely independent of the amount of ascites. The pleural fluid may be located on the left side or may be bilateral. ^{[1, 9, 10]}

 

Nature of the Ascitic and Pleural Fluid:

Ascitic fluid and pleural fluid in Meigs syndrome can be either transudative or exudative.^[9] 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. ^[11]

 

Epidemiology:

United States:

Ovarian tumors are more prevalent in women in upper socioeconomic groups. Ovarian fibromas represent approximately 2-5% of surgically removed ovarian tumors, and Meigs syndrome occurs in only 1-2% of these cases; thus, it is a rare condition. Ascites is present in 10-15% of women with ovarian fibroma, and hydrothorax is present in 1%, especially those with larger lesions.

 

Age-Related Demographics:

The incidence of ovarian tumor begins to increase in the third decade and increases progressively in postmenopausal women, with an average of about 50 years.^{[1, 9]} Meigs syndrome in prepubertal girls with benign teratomas and cystadenomas has been reported.

 

Mortality/morbidity:

Life expectancy of patients with Meigs syndrome mirrors that of the general population after surgery, and less than 1% of fibromas progress to fibro sarcoma. ^[10]

 

Signs and symptoms:

Patients with Meigs syndrome may have a family history of ovarian cancer.

·         Fatigue

·         Shortness of breath

·         Increased abdominal girth

·         Weight gain/weight loss

·         Nonproductive cough

·         Bloating

·         Amenorrhea for premenopausal women

·         Menstrual irregularity

 

Physical Assessment:

Positive signs include the following:

·         Vital signs - Tachypnea, tachycardia

·         Lungs - Dullness 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

·         Abdomen- Most patients present with an asymptomatic, solid, and unilateral pelvic mass, most often left sided; the mass may be large,^[6] but sometimes, no mass is felt; ascites is present, with shifting dullness and/or fluid thrill

·         Pelvis - Examination reveals a pelvic mass

 

Differential Diagnoses:

·         Ascites

·         Cirrhosis

·         Colon Cancer

·         Hypoalbuminemia

·         Malignant Effusion

·         Milroy Disease

·         Nephrotic Syndrome

·         Non-Small Cell Lung Cancer

·         Ovarian Cancer

·         Pleural Effusion

·         Small Cell Lung Cancer

·         Tuberculosis

 

 

Laboratory Studies:

CBC Count:

This study provides information about hemoglobin, hematocrit, and platelet levels. A low hemoglobin count requires further workup, including reticulocyte count, total iron-binding capacity, and iron and ferritin levels. Anemia in patients with Meigs syndrome is most likely due to iron deficiency. Anemia can be corrected emergently by blood transfusion in 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. If necessary, corrections of these electrolytes are made. Prothrombin time is checked before surgery. If elevated, it is a marker of coagulopathy. Elevated prothrombin time is corrected before surgery, either by administering vitamin K to the patient or by transfusing fresh frozen plasma.

 

Serum cancer antigen 125 test:

Other than serum electrolytes and CBC count, the study of interest is the serum cancer antigen 125 (CA-125) tests. Tumor marker serum levels of CA-125 can be elevated in Meigs syndrome, but the degree of elevation does not correlate with malignancy. In fact, a normal CA-125 level does not exclude the possibility of malignancy.^[12] The CA-125 level is not used as a screening test. Immunohistochemical studies suggest that serum CA-125 elevation in patients with Meigs syndrome is caused by mesothelial expression of the antigen rather than by fibroma.^[1] The highest reported level of CA-125 after laparotomy is 1808 U/mL. This would be a false-positive result.

 

Papanicolaou test:

Papanicolaou test findings are normal.

 

Imaging Studies:

·         Chest radiography confirms pleural effusion.

·         Abdominal and pelvic ultrasound confirms the ovarian mass and ascites.

 

CT Scan of the Abdomen and Pelvis:

CT scanning confirms ascites and ovarian, uterine, fallopian tube, or broad ligament mass.

No signs of distant metastasis are observed.

 

Procedures Paracentesis:

Ascitic fluid is mostly transudative. Findings are negative for malignant cells but can be positive for reactive mesothelial cells.

Thoracentesis:

Pleural fluid is usually transudative. Findings can be exudative and negative for malignant cells.

 

Histologic Findings:

Ovarian tumors are divided into the following histologic subgroups, and Meigs syndrome can be observed with any of the benign tumors.

 

Coelomic Epithelial Tumor:

These tumors, which originate from the coelomic epithelium, constitute 80-85% of all ovarian tumors.

·         Serous cystadenoma and mucinous cystadenoma: 15-20% is malignant.

·         Endometrioid type and clear cell: 95-98% is malignant.

·         Brenner tumor: 2% are malignant.

 

Germ cell Tumour:

These tumors originate from the germ cell and constitute 10-15% of all ovarian tumors. All are malignant except mature teratomas and gonadoblastomas, which are always benign.

·         Mature teratoma

·         Immature teratoma

·         Dysgerminoma

·         Gonadoblastoma

·         Endodermal sinus

·         Embryonal carcinoma

·         Non-gestational choriocarcinoma

 

Gonadal-stromal cell tumors:

Gonadal-stromal cell tumors constitute 3-5% of all tumors.

·         Granulosa cell

·         Fibroma: Fewer than 5% are malignant.

·         Thecoma: Fewer than 5% are malignant.

·         Sertoli-Leydig cell: Fewer than 5% are malignant.

·         Lipid cell type: 30% are malignant.

·         Gynandroblastoma: 100% are malignant.

 

Medical Management:

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.

 

Surgical management

·         Resolution after tumor resection has been widely documented.^{[3, 13, 9]}

·         Exploratory laparotomy with surgical staging is the treatment of choice. Perform a frozen section of the ovarian mass during exploratory laparotomy. If the frozen section is consistent with benign tumor, conservative surgery (salpingo-oophorectomy or oophorectomy) is appropriate. Findings of lymph node biopsies and omentum and pelvic washings are negative for malignancy if these procedures are performed during surgery.

·         In women of reproductive age, perform unilateral salpingo-oophorectomy.

·         In postmenopausal women, options include bilateral salpingo-oophorectomy with total hysterectomy and unilateral or occasionally bilateral salpingo-oophorectomy.

·         In prepubertal girls, options include wedge resection of ovary and unilateral salpingo-oophorectomy.

·         The cure rate after either type of surgery is high and recurrence is rare.

 

Prognosis:

Meig's syndrome is a benign disease, if properly treated. No recurrence after sugical removal of the mass has been reported. Clinicans should be aware of this rare and treatable condition.

 

CONCLUSION:

Meigs syndrome is the triad of benign ovarian tumor with ascites and pleural effusion. There is variety of surgical modalities to treat this condition. Meigs symptoms resolve after resection of the tumor. There is no recurrence after surgical removal of the mass.

 

REFERENCES:

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.        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.

3.        Liao Q, Hu S. Meigs’ Syndrome and Pseudo-Meigs’ Syndrome: Report of Four Cases and Literature Reviews. Journal of Cancer Therapy. Journal of cancer therapy. 2015 April. 6(04):293.

4.        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.

5.        Available from http://patient.info/in/doctor/meigs-syndrome

6.        Schmitt R, Weichert W, Schneider W, Luft FC, Kettritz R. Pseudo-pseudo Meigs' syndrome. Lancet. 2005 Nov 5. 366(9497):1672.

7.        Loue VA, Gbary E, Koui S, Akpa B, Kouassi A. Bilateral Ovarian Fibrothecoma Associated with Ascites, Bilateral Pleural Effusion, and Marked Elevated Serum CA-125. Case Rep Obstet Gynecol. 2013. 2013:189072.

8.        Cisse CT, Ngom PM, Sangare M, Ndong M, Moreau JC. [Ovarian fibroma associated with Demons-Meigs syndrome and elevated CA 125]. J Gynecol Obstet Biol Reprod (Paris). 2004 May. 33(3):251-4.

9.        Samanth KK, Black WC. Benign ovarian stromal tumors associated with free peritoneal fluid. Am J Obstet Gynecol. 1970 Jun 15. 107(4):538-45.

10.     CIFDS G, André SA, Maggi L, Nogueira FJ. Syndrome with Elevated CA 125: Case Report with a Journey through Literature. J Pulm Respir Med. 2015. 5(303):2.

11.     Park JW, Bae JW. Postmenopausal Meigs' Syndrome in Elevated CA-125: A Case Report. J Menopausal Med. 2015 Apr. 21 (1):56-9.

12.     Krenke R, Maskey-Warzechowska M, Korczynski P, Zielinska-Krawczyk M, Klimiuk J, Chazan R, et al. Pleural Effusion in Meigs' Syndrome-Transudate or Exudate?: Systematic Review of the Literature. Medicine (Baltimore). 2015 Dec. 94 (49):e2114.

13.     Jones OW, Surwit EA. Meigs syndrome and elevated CA 125. Obstet Gynecol. 1989 Mar. 73(3 Pt 2):520-1. 13

 

 

 

Received on 08.11.2016          Modified on 14.03.2017

Accepted on 28.03.2017          © A&V Publications all right reserved