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ABSTRACT

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INTRODUCTION

Meigs’ syndrome consists of a benign ovar-ian tumor accompanied by ascites and hydro-thorax. Elevated serum carbohydrate antigen125 (CA 125) levels in postmenopausalwomen with solid adnexal masses, ascites andpleural effusion are highly suggestive for ma-lignant ovarian tumor. However, patients withMeigs’ syndrome can also have elevated se-rum CA 125 (a tumor marker) levels.1,2 Wepresent a case of Meigs’ syndrome due to leftovarian thecoma with elevated CA 125.

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CASE REPORT

A 65-year-old Brazilian woman had pre-sented progressive dyspnea, weight loss anddecline in general condition over the 7 monthspreceding admission to our service. In anotherhospital, the patient had been submitted tothoracic drainage due to pleural effusion inher left lung and at that time she had beguntherapeutic tests for tuberculosis, using isoni-azid, rifampicin and pyrazinamide, withoutclinical improvement. With recurrence of thepleural effusion and increase in abdominalvolume due to ascites and a pelvic mass, thepatient sought our service.

Chest examination revealed dullness topercussion and lack of breath sounds in herleft lung. There was arching in the lower ab-domen due to a firm and immobile pelvicmass. The gynecological examination broughtout evidence of arching of the vesicouterineand rectouterine pouches due to extrinsic com-pression, and the uterine cervix was normal.The patient was interned and submitted to

pleural drainage and biopsy that indicatednon-specific chronic pleurisy. Ultrasoundshowed an extensive adnexal solid mass ofabout 16.4 x 10.8 cm located in the pelvis,without exact limits. The serum CA 125 levelwas 319 IU/ml (normal is 5-35 IU/ml).

Due to clinical suspicion of malignantovarian tumor, the patient was submitted toexploratory laparotomy that brought out evi-dence of the presence of serohematic ascitesand a left ovarian tumor measuring 14 x 12 x8 cm with a solid lobular appearance (Figure1). The frozen section diagnosis of the ovar-ian tumor was thecoma. Total abdominal hys-terectomy was performed, with bilateralsalpingo-oophorectomy and cholecystectomybecause of the presence of cholelithiasis. His-topathological examination of the tumor re-vealed a benign stromal lesion with tumor cellsarranged in a fascicular fashion consistent withthecoma (Figure 2).

The postoperative period was uneventfuland the patient was discharged from hospitalon the 7th postoperative day. The serum CA125 level on the 4th postoperative day was406.3 IU/ml and was 53.7 IU/ml on the 30th

postoperative day. The patient was asympto-matic with a normal serum CA 125 level 20months after the operation.

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DISCUSSION

Only 14 cases of Meigs’ syndrome with el-evated CA 125 levels have been described in theliterature.1 The most common histological typesreported have been cellular fibroma (4 cases),fibroma thecoma (3 cases), fibroma (3 cases),thecoma (3 cases) and granulosa cell tumor (1

• Sabas Carlos Vieira

• Leonardo Halley CarvalhoPimentel

• José Carlos CasteloBranco Ribeiro

• Argemiro Ferreira deAndrade Neto

• Jerúsia Oliveira Ibiapinade Santana

Meigs’ syndrome withelevated CA 125: case reportHospital São Marcos, Teresina, Piauí, Brazil

CONTEXT: Meigs’ syndrome consists of a benign ovar-ian tumor accompanied by ascites and hydrotho-rax. Elevated serum CA 125 levels in postmeno-pausal women with solid adnexal masses, ascitesand pleural effusion are highly suggestive formalignant ovarian tumor. However, patients withMeigs’ syndrome can also have elevated serumCA 125 levels. The authors report a case of Meigs’syndrome with elevated CA 125 level.

OBJECTIVE: This is a case report of Meigs’ syndromewith elevated CA 125 level.

CASE REPORT: A 65-year-old Brazilian woman hadpresented progressive dyspnea, weight loss anddecline in general condition over the 7 monthspreceding admission to our service. In anotherhospital, the patient had been submitted to tho-racic drainage due to pleural effusion. With re-currence of the pleural effusion and increase inabdominal volume due to ascites and a pelvicmass, the patient sought our service. Transvagi-nal ultrasound showed an extensive adnexal solidmass of 16.4 x 10.8 cm located in the pelvis with-out exact limits, and the serum CA 125 level waselevated. With a preoperative diagnosis of ovar-ian carcinoma, the patient was submitted to ex-ploratory laparotomy, which revealed a left ovar-ian tumor. The frozen section diagnosis was the-coma. Total abdominal hysterectomy with bilat-eral salpingo-oophorectomy was performed. Thehistology of the specimen confirmed the diagno-sis of thecoma. The patient was asymptomatic witha normal serum CA 125 level 20 months after theoperation.

KEY WORDS: Meigs’ syndrome. CA 125 Antigen.Ovarian tumor. Ovarian carcinoma. Thecoma.

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São Paulo Medical Journal — Revista Paulista de Medicina 211

case). Serum CA 125 levels have ranged from42.3 to 2,120 IU/ml, and one patient presentedlevels higher than 5,000 IU/ml.1

Thecoma is a rare ovarian tumor and it isassociated with Meigs’ syndrome in just 2%of the cases.2 Several hypotheses might explainthe formation of the ascitic fluid. The maintheory is that the transudation mechanismthrough the tumor surface exceeds the capac-ity for peritoneal reabsorption. Anothermechanism implicates the congestion of theperitoneal lymphatic vessels and regional veinscaused by the tumoral mass itself or vasoac-tive substances released by the tumor.1 It isthought that the occurrence of hydrothoraxis secondary to the passage of ascitic fluid tothe pleural space through the diaphragm ordiaphragmatic lymphatic vessels, or alterna-tively because of congenital defects, which aremore common on the right side.3

The CA 125 tumor marker is generallyelevated in patients with malignant ovariantumor. It can, however, be elevated in benigndisorders, such as endometriosis, pelvic inflam-matory disease and uterine leiomyoma. SerumCA 125 levels can also increase in pericardial,pleural and peritoneal irritation or inflamma-tion.1.4 Laparotomy and histopathological ex-amination are required for the correct diag-nosis and treatment of ovarian tumors, sinceelevated serum CA 125 levels can be falselypositive for ovarian malignancy.1

Postmenopausal women with clinical con-ditions of palpable pelvic masses, ascites, pleu-ral effusion and elevated serum CA 125 levelsprobably have malignant ovarian tumors.However, thecoma forming part of Meigs’ syn-drome is also a diagnostic possibility. Figure 2. Microscopic appearance of left ovarian thecoma (hematoxylin-eosin, 400 x).

Figure 1. Macroscopic appearance of surgical specimen of a left ovarian thecoma.

1. Abad A, Cazorla E, Ruiz F, et al. Meigs’ syndrome with elevated

CA 125: case report and review of the literature. Eur J Obstet

Gynecol Reprod Biol 1999;82(1):97-9.

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REFERENCES

2. Turan YH, Demirel LC, Ortac F. Elevated CA 125 in Meigs

syndrome. Int J Gynaecol Obstet 1993;43(1):64-5.

3. Agranoff D, May D, Jameson C, Knowles GK. Pleural effusion

and a pelvic mass. Postgrad Med J 1998;74(871):265-7.4. Niloff JM, Knapp RC, Schaetzl E, Reynolds C, Bast RC.

CA125 antigen levels in obstetric and gynecologic patients.Obstet Gynecol 1984;64(5):703-7.

Sao Paulo Med J 2003; 121(5):210-212.

São Paulo Medical Journal — Revista Paulista de Medicina212

Síndrome de Meigs com CA 125 elevado: relatode caso

CONTEXTO: A síndrome de Meigs consiste deum tumor ovariano benigno em associação comascite e hidrotórax. Níveis elevados de CA 125sérico em mulheres pós-menopáusicas commassa anexial sólida, ascite e derrame pleuralsão muito sugestivos de tumor ovariano malig-no. Entretanto, pacientes com síndrome deMeigs podem apresentar níveis elevados de CA125. Os autores relatam um caso de síndromede Meigs com CA 125 elevado.

OBJETIVO: Relatar um caso de síndrome deMeigs com CA 125 elevado.

RELATO DO CASO: Mulher de 65 anos, brasi-leira, apresentou dispnéia progressiva, perdade peso e queda do estado geral durante osúltimos sete meses antes da admissão em nos-so serviço. A paciente foi submetida, em ou-tro hospital, a drenagem torácica devido der-

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RESUMO

Presented as a poster at: X Jornada Piauiense deGinecologia e Obstetrícia. [10th Piauí Gynecology andObstetrics Workshop]. May 22 to 25, 2002, Teresina,Piauí, Brazil.

Sabas Carlos Vieira, MD. Auxiliary Professor, School ofMedicine, Universidade Federal do Piauí, Piauí, Brazil.

Leonardo Halley Carvalho Pimentel, MD. Graduateof Universidade Federal do Piauí, Piauí, Brazil.

José Carlos Castelo Branco Ribeiro. UndergraduateStudent, School of Medicine, Universidade Federal do Piauí,Piauí, Brazil.

Argemiro Ferreira de Andrade Neto. UndergraduateStudent, School of Medicine, Universidade Federal do Piauí,Piauí, Brazil.

Jerúsia Oliveira Ibiapina de Santana, MD. Patholo-gist, Hospital São Marcos, Teresina, Piauí, Brazil.

Sources of funding: None

Conflict of interest: None

Date of first submission: November 11, 2002

Last received: November 11, 2002

Accepted: February 14, 2003

Address for correspondenceJosé Carlos Castelo Branco Ribeiro

R. Deputado Costa Andrade, 480Bairro São JoãoTeresina/PI – Brasil – CEP 64046-490Tel. (+55 86) 233-4464E-mail: castelobrancoribeiro@ig.com.br

COPYRIGHT © 2003, Associação Paulista de Medicina

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Publishing information

rame pleural. Com a recidiva do derramepleural e aumento do volume abdominal porascite e massa pélvica, a paciente procurounosso serviço. O ultra-som transvaginal mos-trou uma massa anexial sólida de 16,4 x 10,8cm de limites imprecisos localizada na pélvise o CA 125 sérico estava elevado. Com diag-nóstico pré-operatório de carcinoma ovaria-no, a paciente foi submetida a laparotomiaexploradora, que revelou um tumor ovaria-no esquerdo. Biópsia de congelação diagnos-ticou tecoma. Realizou-se uma histerectomiatotal abdominal com salpingo-ooforectomiabilateral. O exame histopatológico confirmouo diagnóstico de tecoma. A paciente encon-trava-se assintomática e o CA 125 estava nor-mal 20 meses após a operação.

PALAVRAS-CHAVE: Síndrome de Meigs. Cân-cer de ovário. Tumor ovariano. Carcinomaovariano. Tecoma.

Sao Paulo Med J 2003; 121(5):210-212.