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Gynecologic Oncology 94 (2004) 223–225

Short Communication

Bilateral ovarian fibromatosis presenting with ascites and hirsutism

Lutfu S. Onderoglu,a Murat Gultekin,a,* Polat Dursun,a Musturay Karcaaltincaba,b

Alp Usubutun,c Deniz Akata,b and Ali Ayhana

aDepartment of Obstetrics and Gynecology, Faculty of Medicine, Hacettepe University, Ankara, TurkeybDepartment of Radiology, Faculty of Medicine, Hacettepe University, Ankara, TurkeycDepartment of Pathology, Faculty of Medicine, Hacettepe University, Ankara, Turkey

Received 29 October 2003

Available online 8 May 2004

Abstract

Background. Ovarian fibromatosis is a very rare nonneoplastic disease. Due to the rarity and atypical clinical presentations, they may give

rise to a misdiagnosis of malignancy and unnecessary extensive surgical interventions. Literature lacks definitive data about this rare disease

and its preoperative evaluations. MRI together with the intraoperative frozen section may help us to define the benign nature of the disease. In

this report, we aimed to review the literature and give a highlight to the gynecologic oncologists about this rare disease.

Case History. A 19-year-old female patient admitted to our hospital with the complaints of menstrual irregularity, hirsutism, and increased

abdominal girth. Physical examination revealed bilateral ovarian mass, hirsutism, and ascites. Serum CA-125 levels were slightly elevated.

Preoperative MRI study showed bilateral hypointense lobulated ovarian masses. With the initial diagnosis of ovarian tumor, we performed

explorative laparotomy and excised both masses. Final pathology was reported as bilateral ovarian fibromatosis.

Conclusion. Ovarian fibromatosis commonly presents with ascites and solid pelvic mass and can be misdiagnosed as a malignant ovarian

tumor. In young patients, clinicians should consider ovarian fibromatosis in differential diagnosis of pelvic mass. Preoperative MRI findings

and intraoperative frozen examination may be used to avoid unnecessary aggressive surgical management.

D 2004 Elsevier Inc. All rights reserved.

Keywords: Ovarian fibromatosis; Pelvic mass; Ovarian tumor; Hirsutism; Ascites

Introduction

Ovarian fibromatosis is an unusual clinicopathological

entity in gynecology practice. It is first described as a

distinct clinical entity by Young and Scully in 1984 [2].

This rare entity may mimic malignant ovarian tumors

because of the similar presentation and radiological find-

ings. Because of the rarity, preoperative diagnosis is still a

problematic issue as in other benign solid ovarian tumors

such as fibromas, fibrotechomas, and massive ovarian

edemas (MOEs). In this report, we presented clinicopatho-

logical and MRI findings of the patient and discussed in the

light of literature.

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

doi:10.1016/j.ygyno.2004.03.042

* Corresponding author. Naci C�akır Mh. 1.Cd. Kubra Apt., 1/1 Dikmen

Ankara, Turkey. Fax: +90-312-311-6372.

E-mail address: drmuratgultekin@yahoo.com (M. Gultekin).

Case report

A 19-year-old female patient was admitted to our hos-

pital with complaint of increasing abdominal girth and

hirsutism. She has developed progressive hirsutismus

around the umbilicus, periareolar region, and under the

chin for 6 months. Secondary sexual characteristics and

pubertal growth were normal on physical examination.

Systemic examination were in normal limits except ascites

and pelvic mass. Her biochemical analysis and hormonal

investigations revealed no abnormality. Among blood anal-

ysis, prolactin was 40.0 ng/ml, DHEA-SO4 59.2 ug/dl, 17-

OH progesterone 1.21 ng/ml, free testosterone 1.32 pg/ml,

and TSH 1.49 UIU/ml; all of which were in normal ranges.

Preoperative CA-15-3, CA-19-9, and alpha-feto protein

were in normal limits, while CA-125 level was slightly

high, 48.33 U/ml. Ultrasonographic evaluation revealed

bilateral solid ovarian masses measuring 9.9 � 4.5 cm

and 3 � 3 cm in size on the right and left side, respectively.

MR examination was performed by 1.5-T superconducting

Fig. 2. Gross appearance of the tumor on the right side.

L.S. Onderoglu et al. / Gynecologic Oncology 94 (2004) 223–225224

magnet (Siemens Symphony). T1-weighted spin echo

images before contrast administration revealed lobulated

hypointense right ovarian mass. Fat-saturated T2-weighted

turbo spin echo images on axial, sagittal, and coronal (Fig.

1A) planes demonstrated bilateral lobulated hypointense

masses in both ovaries which enhanced strongly on T1-

weighted axial (Fig. 1B) images after gadolinium adminis-

tration consistent with a vascularized mass. Also, hyperin-

tense punctate areas were noted at the periphery of the right

ovarian mass on T2-weighted images showing no enhance-

ment on postcontrast images, consistent with cystic degen-

eration. Hypointense appearance on T1- and T2-weighted

images is a characteristic of fibrous tissue containing

tumors. Ascites was also recognized. Differential diagnoses

of these tumors were consistent with either fibrothecomas

or fibromatosis.

On explorative laparotomy, minimal-mild amounts of

ascites, together with bilateral ovarian masses, were present

in the abdomen. We excised both masses from the ovaries,

and the frozen examination did not show any signs of

malignancy. Final pathologic examination was reported as

bilateral ovarian fibromatosis. The mass on the left side was

3.5 � 2 � 2 cm, and the mass on the right side was 12 � 7

Fig. 1. MR findings of ovarian fibromatosis. Bilateral ovarian masses are

seen on T2-weighted coronal image (A). Fat-saturated T1-weighted

postcontrast axial image (B) demonstrates enhancement of ovarian lesions

consistent with well-vascularized solid masses.

� 5 cm in size. Both of them had lobulated, solid cut surface

and yellow-white in color (Fig. 2). No follicular structures

were seen by naked eye.

Microscopic examination revealed a lesion composed of

spindle-shaped cells with prominent collagen production,

which is proved by staining in green with Masson tri-

chrome stain. Cellularity varied, being low cellular in

collagen-rich fields. Small irregular calcifications were

found in some areas. Most characteristic microscopic

feature was the follicles entrapped within the lesion (Fig.

3). Foci of a rim of edematous stroma and ovarian cortex

containing entrapped follicles within collagen-rich fibrous

lesion also exist. Intact normal ovarian cortex was only

seen in a small foci. There were also luteinized cells

dispersed between the spindle cells, and all of them stained

immunohistochemically with inhibin. Postoperative period

was uneventful, and patient was discharged on the fourth

day. Six months after the operation, her hirsutism regressed

without any therapy.

Fig. 3. Microscopic appearance of ovarian fibromatosis.

L.S. Onderoglu et al. / Gynecologic Oncology 94 (2004) 223–225 225

Discussion

Ovarian fibromatosis is an uncommon entity that can be

seen among young females. It is first defined by Young

and Scully in 1984, and there are about 30 reported cases

in the English literature to our knowledge [1,2]. It is a

nonneoplastic disease, which can cause enlargement of the

ovaries up to 6–12 cm in size as solid or very rarely

cystic-appearing masses. It is usually seen unilaterally, but

few bilateral cases have been reported [3]. Ovarian fibro-

matosis usually presents with abnormal menstruation and

lower abdominal pain and rarely with some androgenic

effects such as hirsutism or virilization [1]. In the case we

present here, clinical features are typical for the disease

with enlargement of the ovaries, abnormal menstruation,

and hirsutism except for the hypervascular nature of the

tumor which, to our knowledge, is the first case in the

literature.

Due to the rarity of the disease, pathogenesis is not

well understood. Microscopically, it is composed of

spindle-cell proliferation with abundant collagen matrix

in between the normal follicular structures without any

infiltrative or distruptive growth pattern. Differential di-

agnosis includes other fibromatous diseases of the ova-

ries, such as ovarian fibromas and fibrothecomas. Ovarian

fibromas and fibrothecomas can easily be distinguished

because of a well-circumscibed mass compressing the

underlying parenchyma, unlike the diffuse infiltrative

pattern seen in fibromatosis [4]. Entrapped follicles be-

tween the fibrous tissue are the most important discrim-

inating feature of our case. Soft tissue fibromatosis of the

female pelvis involving the ovaries was also reported

characterized by obliteration and infiltration of the pe-

ripheral structures, in contrast to primary ovarian fibro-

matosis [4]. Massive ovarian edema (MOE), as described

by Young and Scully, is another nonneoplastic disease

where enlargement of the ovaries is due to cortical and

medullary edema. Microscopically, MOE may be associ-

ated with variable amounts of ovarian fibromatosis [2,5].

It is postulated that MOE is the result of recurrent

lymphatic obstructions due to the intermittent torsion of

the ovaries, and any pathologic process leading to in-

creased weight of the ovaries, including the ovarian

fibromatosis, can cause this entity.

There are some reports of the ovarian fibromatosis in

the literature showing an association with omental fibrosis,

idiopathic sclerosing peritonitis, and intraabdominal fibro-

matosis [3,6,7,8]. Ascites was also a common finding in

these reports. The possible mechanisms underlying these

pathologies are still not clear, but fibrogenic subtances or

asidic transudates released into the peritoneum can cause

fibrous proliferations in the soft tissues beneath the meso-

thelial cells [6]. Also reported are the cases with sex-cord

type cellular proliferations associated with ovarian fibro-

matosis by which the androgenic manifestations can be

explained [1,2].

Imaging studies with MRI may be helpful in the preop-

erative diagnosis of ovarian fibromatosis. MR imaging

features of our case have typical (hypointense appearance

on T1- and T2-weighted images) and atypical (strong

enhancement on postcontrast T1-weighted images) findings.

Also, multiple peripheral punctate high-intensity structures

were noted at the periphery of the right ovarian mass

consistent with cystic degeneration, which is suggestive of

ovarian fibromatosis according to Bazot et al. [9]. Presence

of strong enhancement in ovarian fibromatosis may be

explained by coexistence of MOE.

Conclusion

Ovarian fibromatosis commonly presents with ascites

and solid pelvic mass. Therefore, it can be misdiagnosed

as a malignant ovarian tumor. In young patients, clinicians

should consider ovarian fibromatosis in differential diagno-

sis of pelvic mass, especially in the presence of ascites

together with hirsutism. Preoperative MRI findings and

intraoperative frozen examination may avoid unnecessary

aggressive surgical management. As the number of the cases

increases in the literature, our understanding of this rare

disease will improve.

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