AJR 2003; 180:1288-1290
© American Roentgen Ray Society
Imaging of Ovarian Fibromatosis
Marc Bazot1,
Christine Salem1,
Annie Cortez2,
Jean-Marie Antoine3 and
Emile Daraï3
1 Department of Radiology, Hôpital Tenon, Assistance
Publique-Hôpitaux de Paris, 4 rue de la Chine, 75020 Paris,
France.
2 Department of Pathology, Hôpital Tenon, Assistance
Publique-Hôpitaux de Paris, 75020 Paris, France.
3 Department of Obstetrics and Gynecology, Hôpital Tenon, Assistance
Publique-Hôpitaux de Paris, 75020 Paris, France.
Received July 8, 2002;
accepted after revision August 29, 2002.
Address correspondence to M. Bazot.
Introduction
Tumorlike ovarian enlargement due to diffuse ovarian fibrosis is referred
to as ovarian fibromatosis [1].
Ovarian fibromatosis is a benign disorder that differs from other pelvic
fibrotic processes such as desmoid tumors, ovarian fibroma, Brenner tumors,
and Krukenberg's tumors. To our knowledge, the radiologic features of ovarian
fibromatosis have never been described. We report the imaging features of a
case of histologically confirmed ovarian fibromatosis.
Case Report
A 25-year-old woman presented with metrorrhagia and menstrual
irregularities. Physical findings and the CA 125 serum level were normal.
Transabdominal sonography showed well-defined bilateral heterogeneously
echogenic masses (Fig. 1A).
Transvaginal sonography showed a normal right ovary adjacent to a
heterogeneous mass and a left ovarian mass with dense posterior wall
attenuation. Doppler sonography revealed little flow in the two masses. The
uterus was normal, and a small amount of fluid was seen in the pouch of
Douglas. CT showed bilateral solid homogeneous ovarian masses with no fat
component. The right and left masses measured 7 and 6 cm, respectively, and
were hyperattenuating compared with the adjacent myometrium on unenhanced CT
(Fig. 1B). Dynamic and delayed
CT showed little enhancement of the masses relative to the adjacent myometrium
(Fig. 1C). No hypertrophic
lymph nodes were found. Both ovarian masses showed homogeneous low signal
intensity on T1- and T2-weighted MR images (Figs.
1D and
1E). The right mass infiltrated
the posterior border of the ovary, but normal ovarian tissue persisted. In
contrast, no normal ovarian tissue was found on the left side. Small cystic
structures were observed in both masses. No vessels and no significant uptake
of contrast material were noted in either mass during dynamic MR imaging. The
tumors showed little enhancement relative to myometrium, and no abnormal
peritoneal enhancement was noted on delayed fat-suppressed T1-weighted spoiled
gradient-echo fast low-angle shot imaging. Free peritoneal fluid was present
in the pouch of Douglas.
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Fig. 1B. —25-year-old woman who presented with ovarian fibromatosis.
Unenhanced CT scan shows bilateral well-defined solid ovarian masses. Both
masses (asterisks) are slightly hyperattenuating relative to adjacent
myometrium (arrow). Note small calcification (arrowhead) in
left mass.
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Fig. 1D. —25-year-old woman who presented with ovarian fibromatosis.
Axial breath-hold T1-weighted spoiled gradient-echo fast low-angle shot MR
image shows low signal intensity of both ovarian tumors.
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Fig. 1E. —25-year-old woman who presented with ovarian fibromatosis.
Axial T2-weighted turbo spin-echo MR image shows bilateral adnexal masses with
low signal intensity. Small cystic structures (arrowheads) are
observed in both tumors. Right-sided tumor infiltrates posterior border of
ovary (arrow), which is partially intact. Note presence of fluid in
pouch of Douglas and air in rectum (asterisk).
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Surgery consisted of left oophorectomy and partial resection of the right
ovary.
The left-sided mass measured 7 cm in diameter; the right-sided mass was
composed of three portions measuring 3, 4, and 8 cm. Macroscopically, two
distinct patterns were found: a firm well-defined nodular tumor with a dense
white fasciculated cut surface, and diffuse infiltration by a white
multimicronodular structure with a lobulated margin surrounding multiple
microcystic structures (Fig.
1F). Microscopic examination showed proliferation of spindle cells
separated by dense collagen. The adjacent ovarian stroma was infiltrated but
cystic follicles were spared. Few vessels were present. All the specimens
contained focal areas of edema. No lutein or sex-cord cell proliferation was
found. The final pathologic diagnosis was ovarian fibromatosis.
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Fig. 1F. —25-year-old woman who presented with ovarian fibromatosis.
Photograph shows pathologic specimen of left ovarian mass. Note firm,
well-defined nodular tumor and diffuse infiltration by white multimicronodular
structure (arrows) with lobulated margin surrounding multiple
microcystic structures (arrowheads).
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Postoperatively, the patient presented climacteric symptoms related to
altered ovarian function and confirmed by an elevated serum level of
follicle-stimulating hormone (54 mIU/mL; normal, < 25 mIU/mL). Hormonal
replacement therapy was started, but the patient discontinued this treatment
after 4 months. Normal menstrual cycles resumed spontaneously. No tumor
recurrence has occurred 4 years after surgery.
Discussion
Ovarian fibromatosis is a rare benign disorder first described by Young and
Scully [1] in young patients
(mean age, 25 years) who present with menstrual abnormalities, abdominal and
pelvic pain, and occasionally hirsutism or virilization. Ovarian fibromatosis
is closely related to massive ovarian edema (residual normal ovarian
structures are present) but differs by the presence of abnormal fibrous tissue
in the former and edema in the latter. Ovarian fibrous tissue is most often
found in neoplastic diseases such as fibromas and Brenner, Krukenberg's, and
desmoid tumors. Because of the benign nature of ovarian fibromatosis and its
occurrence in young women, correct pretreatment diagnosis is crucial to
determine a laparotomic or laparoscopic approach and to allow conservative
treatment [1]. After
conservative surgery, Young and Scully
[1] reported normalization of
menses and regression of masculinization, and some patients became
pregnant.
Heterogeneously echogenic adnexal masses that show posterior wall
attenuation are usually ovarian fibromas, dermoids, or degenerative leiomyomas
[2]. Low flow on Doppler
sonography and poor enhancement on dynamic and delayed CT are suggestive of
ovarian fibromas [3]. In our
patient, both masses had homogeneously low signal intensity on T1- and
T2-weighted MR imaging, although these features can also be seen in ovarian
fibromas; fibrothecomas; and Brenner, Krukenberg's, and desmoid tumors
[4]. However, Brenner tumors
contain extensive amorphous calcifications in more than 80% of their solid
portion on CT and MR imaging
[5]. Krukenberg's tumors are
usually bilateral and have sharp margins and an oval shape
[6]; they also usually show
strong contrast enhancement on CT and MR imaging
[7]. Pelvic desmoid tumors are
rare locally invasive, nonmetastasizing neoplasms that typically occur in the
second or third decade [8].
Their radiologic features, and particularly their MR imaging signal, may
resemble those of ovarian fibromatosis. However, ovarian fibromatosis appears
relatively well limited, with no involvement of adjacent pelvic structures. In
our patient, the absence of significant enhancement during the parenchymal
phase of dynamic MR imaging, and the mild enhancement on delayed MR images,
were related to the fibrous component of the masses. T2-weighted MR imaging
showed a hypointense signal, corresponding to fibrous infiltration of the
right ovary, and remnants of normal ovarian parenchyma, which are unusual in
ovarian fibroma. Ovarian fibromas and fibrothecomas occur at all ages, but
most frequently during the fifth or sixth decade of life, and are often
unilateral and asymptomatic. The occurrence of bilateral ovarian tumors in a
young woman with hormonal disturbances is unusual but fibroma cannot be ruled
out. However, the combination of a partially intact right ovary, follicle
enlargement, and multiple hyperintense foci on T2-weighted MR imaging could be
suggestive of ovarian fibromatosis.
In conclusion, ovarian fibromatosis should be considered in women with
solid ovarian tumors that have a predominant fibrous component. The presence
of ovarian infiltration, with partial sparing of normal ovarian structures,
which is particularly visible on MR imaging, can help to distinguish
fibromatosis from ovarian fibroma.
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Introduction
Case Report
