www.elsevier.com/locate/ygyno

Gynecologic Oncology 94 (2004) 226–228

Case Report

Pseudo-Meigs’ syndrome associated with malignant struma ovarii:

a case report

Gian Franco Zannoni,a Valerio Gallotta,b Francesco Legge,b Elisabetta Tarquini,a

Giovanni Scambia,c and Gabriella Ferrandinab,*

a Institute of Pathology, Catholic University, 00168, Rome, ItalybGynecologic Oncology Unit, Department of Obstetrics and Gynecology, Catholic University, 00168, Rome, Italy

cDepartment of Oncology, ‘‘Centro di Ricerca e Formazione ad Alta Tecnologia nelle Scienze Biomediche’’, Catholic University,

Contrada Tappino, 86100, Campobasso, Italy

Received 8 January 2004

Available online 18 May 2004

Abstract

Background. Malignant struma ovarii is a rare tumor, consisting of a struma ovarii with malignant transformation. The association of a

malignant struma ovarii with pseudo-Meigs’ syndrome and elevated Ca-125 levels has been never reported.

Case. A 66-year-old woman presented with monolateral ovarian mass, ascites, hydrothorax, and elevated Ca-125 levels. Optimal medical

staging was performed. Definitive histological examination revealed a malignant struma ovarii. The immediate and complete resolution of

symptoms and rapid decline of both Ca-125 and thyroglobulin levels to normal value were achieved post-operatively. After counseling, strict

follow-up was planned, and no adjuvant therapy was administered.

Conclusion. We report the first case of pseudo-Meigs’ syndrome associated with malignant struma ovarii and elevated Ca-125 levels. The

choice of not performing adjuvant therapy is feasible after optimal surgery and adequate staging procedure given to the usually clinical

benign course and the low incidence of metastases in malignant struma ovarii. Careful patient counseling is required.

D 2004 Elsevier Inc. All rights reserved.

Keywords: Struma ovarii; Pseudo-Meigs’ syndrome

Introduction Case report

Struma ovarii is a specialized teratoma consisting of

normal thyroid tissue. Malignant transformation of struma

ovarii is a rare event representing only 5% of all strumae

[1,2]. Rare cases of struma ovarii have been associated with

pseudo-Meigs’ syndrome (i.e., pelvic mass other than fibro-

ma, thecoma, granulosa cell tumor, plus hydrothorax and

ascites). There are no reports about the association of

pseudo-Meigs’ syndrome with malignant struma ovarii

and elevation of Ca-125 levels. A case report is presented.

0090-8258/$ - see front matter D 2004 Elsevier Inc. All rights reserved.

doi:10.1016/j.ygyno.2004.03.045

* Corresponding author. Gynecologic Oncology Unit, Department

Obstetrics/Gynecology, Catholic University of the Sacred Heart, Largo A.

Gemelli, 8, 00168, Rome, Italy. Fax: +39-06-35508736.

E-mail address: gabriella.ferrandina@libero.it (G. Ferrandina).

The present case is a 66-year-old, Caucasian, white

female, gravida 6, para 5, who was admitted to a local

hospital, complaining of fatigue, and abdominal swelling.

Her medical history included only colecistectomy. Meno-

pause occurred at the age of 50 years. The patient did not

complain of any pain or changes in micturition or bowel

habits. Marked right pleural effusion, ascites, and a large

abdominal tumor were detected by total body CT scan. The

patient was then transferred to our hospital. Abdominal

examination revealed a palpable mass reaching the trans-

verse umbilical line. Abdominal ultrasound confirmed the

presence of ascites and a large (95 � 56 � 70 mm),

irregular, solid mass with mixed echogenicity in left ovary.

Thoracentesis yielded an exudate (350 ml), found to be

negative for malignancy and microorganisms. Paracentesis

yielded 5.5 l of serous fluid consistent with an exudative

Table 1

Ca-125, thyroglobulin (TG) and thyroid function (TSH, FT3, FT4) tested

before (day 0) and at different time intervals after surgery

G.F. Zannoni et al. / Gynecologic Oncology 94 (2004) 226–228 227

process. Cytology revealed only reactive mesothelial cells.

Blood chemistry was within normal limits except for the

serum Ca-125 levels of 1636 U/ml (normal below 35 U/ml).

Fig. 1. (A) Microscopic appearance of the left ovary showing thyroid

follicles of varying sizes. H&E; original magnification, �10. (B) Malignant

struma ovarii. Typical cytologic atypia of thyroid papillary carcinoma:

‘‘ground glass’’ nuclei, grooves, and nuclear overlapping. H&E; original

magnification, �25. (C) Malignant struma ovarii. Neoplastic cells are

positive for HBME-1. Original magnification, �25.

Pre-operative Post-operative

(day 0) (day 4) (day 60) (day 180)

Ca-125 (U/ml) 1636 27 19 22

TG (ng/ml) 299.8 58.9 31 34

TSH (AIU/ml) 0.8 0.69 0.57 0.62

FT3 (pg/ml) 2.5 2.7 3.1 2.9

FT4 (Ag/ml) 12.7 14.7 10.1 10.5

The patient was taken to the operating room. At laparot-

omy, the left ovarian mass appeared pale, smooth, and firm.

The ovarian capsule appeared intact and there were no

palpable pelvic or paraortic adenopathies. The right ovary,

the uterus, diaphragm, bowel, and omentum appeared also

free of disease.

The patient underwent total abdominal hysterectomy,

bilateral salpingo-oophorectomy, and omentectomy; multi-

ple peritoneal biopsies, and sampling of pelvic lymphnodes

were also performed. At histopathological analysis on

frozen section, the diagnosis was ‘‘struma ovarii’’.

Macroscopically, the left ovary was replaced by a cystic

lesion (120 � 90 mm) with a solid area showing a smooth

outer surface. Microscopically, the mass was composed of

follicles varying in size filled with colloid that resembled

normal thyroid tissue. Some areas showed a microfollicular

and solid pattern (Fig. 1A). In the area of thyroid tissue,

cells were markedly atypical, with scant cytoplasm, over-

lapping nuclei, several nuclear grooves and nuclear pseu-

doinclusions (Fig. 1B). These histologic and cytologic

features were consistent with the diagnosis of follicular

variant of papillary carcinoma. Neither capsular nor vascular

invasion were identified. The mitotic activity was < 2

mitosis per 10 high-power fields. Neoplastic cells showed

a positive reaction for thyroglobulin, HBME-1 (Fig. 1C),

and galectin and a focal positivity for cytokeratin 19. The

immunostaining of sinaptofisin and chromogranin, which

was performed to exclude the diagnosis of carcinoid, gave

negative results. Thyroglobulin (TG) levels were measured

on preoperative serum sample and were 299.8 ng/ml (nor-

mal value: < 65 ng/ml). On day 4, after surgery, serum TG

levels were 58.9 ng/ml (Table 1). Ultrasound examination of

the thyroid was found to be normal as well as thyroid

function tests and other hematological and biochemical

tests. A conservative approach consisting of a strict fol-

low-up with clinical examination, periodic CT scan, TG, and

Ca-125 serial amount was discussed and accepted by the

patient after counseling. After 6 months of follow-up, the

patient is free of disease and in good health.

Discussion

Struma ovarii is a highly specialized form of mature

ovarian teratoma. While approximately 15% of teratoma

G.F. Zannoni et al. / Gynecologic Oncology 94 (2004) 226–228228

contain normal thyroid tissue, the diagnosis of struma ovarii

can only be made when thyroid tissue is the predominant

element [1,3], which occurs approximately in 2% of terato-

mas [1]. Malignant transformation of struma ovarii occurs in

5% of cases, and is currently diagnosed from the criteria

described by Devaney et al. [1] and including ‘‘ground

glass’’ nuclei, increased mitotic activity and/or vascular

invasion [1,2,4].

Meigs and Cass [5] first described the syndrome con-

sisting of ovarian fibroma/thecoma, with ascites and hydro-

thorax, characterized by the resolution of symptoms with

removal of the benign tumor. In 1954, Meigs [6] proposed

to limit true Meigs’ syndrome to benign and solid tumors

with the gross appearance of a fibroma (fibroma, thecoma,

granulosa cell tumor), accompanied by ascites and hydro-

thorax. When other ovarian tumors are found associated

with the criteria seen in Meigs’ syndrome, the term pseudo-

Meigs’ syndrome is employed.

The origin of the effusions remains obscure in both

Meigs’ and pseudo-Meigs’ syndrome, although some mech-

anisms have been suggested such as active fluid secretion by

the tumor or peritoneum, venous and/or lymphatic obstruc-

tion, low serum proteins, and presence of toxins, and

inflammatory products [1,5–7]. Anyway, the direct connec-

tion between the presence of pelvic tumor and the effusions

is demonstrated by the rapid regression of the latter ones

when the neoplasm is excised.

In literature, very few cases of struma ovarii associated

with pseudo Meigs’ syndrome have been described, and

only in four of them this condition is accompanied by the

elevation of Ca-125 levels [8–11].

We describe the first case of malignant struma ovarii

presenting with pseudo-Meigs’ syndrome and elevated Ca-

125 serum levels. Since it has been reported that some cases

defined as malignant struma ovarii can be re-classified as

stromal carcinoid from immunohistochemistry for neuroen-

docrine markers [12,13], we performed the immunoreaction

for sinaptofisin and chromogranin, to distinguish carcinoid

elements, which resulted to be absent in our sample.

Because of the rarity of malignant struma ovarii, the

optimal surgical and postoperative management of the

tumor has not been defined, as emphasized in the recently

reported review by DeSimone et al. [4].

To plan the therapeutic postoperative strategies several

issues were considered in our case: (a) tumor capsule was

intact and there was no evidence of disease in the contro-

lateral ovary (FIGO stage IA); (b) there was no microscopic

sign of vascular space invasion and mitotic activity was low;

(c) serum levels of TG, which is considered reliable marker

for this disease [12], rapidly normalized after surgery; (d)

Ca-125 levels also dropped to a normal values in a few

days; (e) thyroid gland and function were normal; (f) 2

weeks after surgery ascites and hydrothorax disappeared.

For these reasons, after patient counseling, we decided to

supersede to thyroidectomy and I131 ablation and to strictly

follow-up the patient with serial assessment of TG and Ca-

125 levels, thyroid ultrasound, CT scan.

In conclusion, we describe the first case of pseudo-

Meigs’ syndrome associated with malignant struma ovarii

and elevated Ca-125 levels. Complete regression of ascites

and thorax effusion, with rapid normalization of Ca-125 and

TG levels were documented after radical surgery.

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