Computed Tomography Findings of Meigs Syndrome

Posted By Sana Hatoum
Computed Tomography Findings of Meigs Syndrome

Gender, Age

Female, 12

Diagnosis

Meigs syndrome

History

A previously healthy 12-year-old female presented with a chief complaint of a three-month-history of abdominal distention which was exacerbated by abdominal pain in the previous week. Also, the patient described an unintentional weight loss of 3 Kg over the last nine months. The initial examination revealed a shifting dullness suggestive of ascites. The workup showed CA 125 to be elevated to 1160 U/mL with normal CEA, CA 19·9, AFP, and LDH. The ultrasound revealed a large left adnexal mass of 14x12x6 cm (in maximum craniocaudal, transverse, and anteroposterior diameters, respectively) with significant ascites. Computed Tomography showed moderate to large right pleural effusion and atelectasis, and a large mid-abdomen heterogeneously peripherally enhancing cystic lesion arising from the left ovary, measuring 14·8x12·5x6 cm (in maximum craniocaudal, transverse, and anteroposterior diameters, respectively), along with a large ascites. The magnetic resonance imaging with gadolinium showed an enhancing complex cystic mass with internal septations. There was mild mass effect on the adjacent structures with secondary mild to moderate dilatation of the right collecting system and fullness of the left collecting system. There was no invasion to adjacent structures. The pleural effusion appeared large and non-loculated on ultrasound-guided drainage. The obtained serosanguinous fluid was negative for malignant cells. A fertility-sparing left salpingo-oophorectomy and ascitic fluid drainage were performed. The pathology showed a sclerosing stromal tumor of the ovary. The ascitic fluid was devoid of malignant cells. The findings of a benign tumor, the spontaneous postoperative resolution of ascites and pleural effusion, and the normalization of CA 125 level to 11·7 U/mL confirmed the diagnosis of Meigs Syndrome.

Findings

The initial examination revealed a shifting dullness suggestive of ascites. The workup showed CA 125 to be elevated to 1160 U/mL with normal CEA, CA 19·9, AFP, and LDH. The ultrasound revealed a large left adnexal mass of 14x12x6 cm (in maximum craniocaudal, transverse, and anteroposterior diameters, respectively) with significant ascites. Computed Tomography showed moderate to large right pleural effusion and atelectasis, and a large mid-abdomen heterogeneously peripherally enhancing cystic lesion arising from the left ovary, measuring 14·8x12·5x6 cm (in maximum craniocaudal, transverse, and anteroposterior diameters, respectively), along with a large ascites. The magnetic resonance imaging with gadolinium showed an enhancing complex cystic mass with internal septations. There was mild mass effect on the adjacent structures with secondary mild to moderate dilatation of the right collecting system and fullness of the left collecting system. There was no invasion to adjacent structures. The pleural effusion appeared large and non-loculated on ultrasound-guided drainage. The obtained serosanguinous fluid was negative for malignant cells. A fertility-sparing left salpingo-oophorectomy and ascitic fluid drainage were performed. The pathology showed a sclerosing stromal tumor of the ovary. The ascitic fluid was devoid of malignant cells.

Discussion

In 1937, Meigs and Cass first described seven patients with ovarian fibromas, ascites, and hydrothorax. Complete resolution of their signs and symptoms occurred after surgical removal of the ovarian tumor. Meigs syndrome is rare and complicates 1-3% of ovarian fibromas, which represent 2-5% of surgically removed ovarian tumors. In children, the annual incidence of ovarian masses is 2.6 cases/100,000 girls per year. It’s uncommon before the third decade, and peaks in the seventh. Many hypotheses were suggested in regard to the formation of ascites and pleural effusion in association with the ovarian tumor, but the pathophysiology is still unknown. The specific mechanism of CA 125 elevation is controversial as well. The triad of Meigs syndrome comprises an abdominal mass, ascites, and pleural effusion. These elements can produce several symptoms and/or complications. Diagnosing Meigs syndrome is challenging, as it is only confirmed after the ascites and pleural effusion resolve postoperatively, and when the tumor is histologically confirmed. Imaging plays an important role but doesn’t gives certainty to the presumptive diagnosis. An abdominopelvic ultrasound can confirm both the ascites and the mass. A CT scan further characterizes the tumor, confirms its ovarian origin, and removes suspicion of metastasis. Of course, ascites will also be evident. The pleural effusion can be confirmed via chest X-ray. CA 125 is also not diagnostic, as it is not specific. Exploratory laparotomy is the treatment of choice where the tumor is resected, and its benign nature is confirmed via frozen section. When the case is managed properly, prognosis is excellent, as life expectancy is the same as that of the general population. The differential diagnosis list includes malignant ovarian tumors, other cancers including bowel and lung, liver cirrhosis, congestive heart failure, nephrotic syndrome, and tuberculosis.

Pearls

- Meigs syndrome is rare and mimics malignant conditions; - Early diagnosis and proper management are crucial to avoid unnecessary practices and allow for quick recovery and excellent prognosis.

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Comments
  • Roland Talanow 2020-06-03 12:39:03

    Thank you for this nice case example.
    If you intend to publish: Since this entity sufficiently published in the established literature, would this case provide any new knowledge?

    Roland Talanow 2020-09-21 15:14:42

    Thank you.

    Sana Hatoum 2020-08-16 07:46:42

    Thank you for your comment. We believe that our case highlights that Meigs syndrome is a serious yet a potentially curable entity in children. Data shows that the annual incidence of ovarian masses is only 2.6 cases/100,000 girls per year in children. Since Meigs syndrome complicates only 1-3% of ovarian fibromas, which represent 2-5% of surgically removed ovarian tumors, then Meigs cases are truly rare, making this syndrome not on top of the differential diagnoses list. In addition, our case had elevated CA-125 levels, which can be correlated with ovarian malignancies. We aim to remind physicians from all specialties and levels of training that encountering Meigs triad is room for a quick diagnosis of a benign etiology without unnecessary practices including imaging and subsequent radiation exposure in children.

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