Meigs Syndrome with Highly Elevated CA125 Volume 46 - Issue 2

Shiomi Ushida¹, Satoshi Ichigo¹, Hiroshi Takagi¹, Kazutoshi Matsunami¹, Yuji Ito² and Atsushi Imai¹*

• ¹Department of Obstetrics and Gynecology, Matsunami General Hospital, Japan
• ²Department of Obstetrics and Gynecology, Ena Municipal Hospital, Japan

Received: September 05, 2022;   Published: September 15, 2022

*Corresponding author: Atsushi Imai, Department of Obstetrics and Gynecology, Matsunami General Hospital, 185-1 Dendai, Kasamatsu, Gifu 501-6062, Japan

DOI: 10.26717/BJSTR.2022.46.007313

Abstract PDF

Meigs syndrome is an unusual condition consisting of benign solid ovarian tumors accompanied by ascites and/or pleural effusion. Usually Meigs syndrome does not result in serum CA125 elevation. The elevated CA125 level (>1000 U/ml) occurs more frequently in those with larger size tumor (>10cm) of Meigs syndrome. It has a clinical picture closely resembled to malignant ovarian cancer. Because diagnosis can only be made after pathological examination, surgery is an excellent tool for treatment with subsequent resolution of the ascites and hydrothorax.

Meigs syndrome is an unusual condition and fist classified as a triad of benign solid ovarian tumors (fibroma, fibrothecoma, granulosa cell tumor, or Brenner tumor) with both ascites and pleural effusion [1,2]. The characteristic feature of the syndrome is complete disappearance of ascites and pleural effusion following the surgical removal of the tumor. This rare clinical form occurs with a low incidence rate, with only 1% of ovarian solid tumors present as Meigs syndrome [1,2]. Meigs syndrome with serum CA125 elevation is not frequently encountered in clinical practice. We aim to understand the unusual manifestations of Meigs syndrome combined with an elevated CA125 level (Table 1).

Table 1: Reported cases of Meigs’ syndrome with elevated serum CA125.

Note: *normal being <35 U/ml.

Characteristics of CA125

Figure 1: A case of Meigs syndrome with highly elevated CA125. A 49-year-old woman presented with ascites and pleural effusion. MRI sagittal (a) and horizontal (b) Images of pelvis (T2-weighted) showed a large heterogenous solid mass originating from the left ovary measuring 11x14x11 cm and huge amount of ascites. Serum CA125 was found to be elevated at 3969 U/ml. The patient was submitted to an exploratory laparotomy which detected 10200 ml of serous ascites. She underwent a total abdominal hysterectomy and bilateral salpingo-oophorectomy. Pathology revealed ovarian fibromas. After removal of pelvic tumor the symptoms completely relieved, and CA125 level on the 14th postoperative day was 22U/ml.

CA125 is a large mucinous membrane glycoprotein and recognized by a monoclonal antibody OC-125. It is produced in the normal and abnormal tissues of Müllerian origin including amniotic membrane and embryonic coelomic epithelium [3]. This antigen can also appear in many adult tissues such as the fallopian tubes epithelium, endometrium, cervix, and ovaries [4]. It is also found in mesothelial cells of peritoneum, pericardium and pleura. Therefore, some normal body tissues may express a constant low level of circulatory or serum CA125, whereas high levels of CA125 are exceptionally rarely observed. This marker is found to be elevated during menstruation or pregnancy and in some benign conditions including endometriosis, peritonitis or cirrhosis, particularly with ascites [5,6]: these cause peritoneum irritation and release of inflammatory mediators. It is also increase in vascular invasion, inflammation and tissue destruction associated with malignant disease [7].

Cases with Severely Elevated CA125

Ovarian fibroma/fibrothecoma originates from the sex-cord stromal and belongs to the subgroup of granulosa-theca cell tumors and the thecoma-fibroma group, according to the WHO classification. Its incidence in all ovarian tumors is estimated to be less than5% [8]. This tumor often exhibits benign behavior and a good prognosis. The most common manifestation is a unilateral, asymptomatic pelvic mass. Ovarian fibroma/fibrothecoma with elevated serum CA125 level rarely occurs in clinical practice, and is mentioned in only 10-20% of Meigs’ syndrome patients in the published literature [9,10,11]. A literature review using PubMed yielded 11 cases with Meigs syndrome showing elevated CA125 level (>1000 U/ml) (Table 1). Mean tumor size was 18.7 cm. All patients were released from the clinical symptoms following surgical removal. Meigs syndrome is most common in postmenopausal women with an average age of about 50 years [12,13]. It is extremely rare in women under the age of 30. Several analyses have postulated that high CA125 level may be age-related (most of the cases were in their 70s) [11], it was distributed from 30s to 70s (Table 1). includes one patient with ovarian fibroma and elevated serum CA125 (3969 U/ml). Details are given in (Figure 1).

Possible Explanations of CA125 Elevation

The source of serum CA125 in ovarian fibroma/fibrothecoma has not yet been elucidated, but mesothelial expression of CA125, rather than expression by the fibroma/fibrothecoma, may result in the elevation [14,15]. In an immunohistochemical staining study by (Shen, et al. [13]), the CA125 expression is negative in all fibroma/ fibrothecoma tissues examined, but positive in all epithelial ovarian cancer tissues. CA125 staining is localized in the cytomembrane. The elevated serum CA125 levels in ovarian fibroma/fibrothecoma are nontumor originated and occur more frequently in patients with larger-sized tumor of Meigs’ syndrome. The mechanism consists of previous findings that the peritoneal mesothelial cells might play a critical role in the mechanism of developing elevated serum CA125 [15,16].

Meigs syndrome presenting with an elevated CA125 is a rare clinical entity [17-24]. This condition mimics an ovarian malignancy, but Meigs syndrome should be considered in the differential diagnosis. It has a clinical picture closely resembled to malignant ovarian cancer. Because diagnosis can only be made after pathological examination, surgery is an excellent tool for treatment with subsequent resolution of the ascites and hydrothorax.

The authors declare that they have no conflict of interest.

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