AJR 2005; 185:207-215
© American Roentgen Ray Society
CT and MRI Findings of Sex Cord–Stromal Tumor of the Ovary
Seung Eun Jung1,
Sung Eun Rha1,
Jae Mun Lee1,
Soo Youn Park1,
Soon Nam Oh1,
Kyoung Sik Cho2,
Eun Ju Lee3,
Jae Young Byun1 and
Seong Tai Hahn1
1 Department of Radiology, St. Mary's Hospital, College of Medicine, The
Catholic University of Korea, #62, Youidodong, Youngdeungpo-gu, Seoul 150-713,
South Korea.
2 Department of Radiology, Asan Medical Center, University of Ulsan, Seoul
138-736, South Korea.
3 Department of Radiology, Ajou University, College of Medicine, Paldal-gu,
Suwon, Kyunggi-do, South Korea.
Received July 9, 2004;
accepted after revision September 22, 2004.
Address correspondence to S. E. Jung
(sejung{at}catholic.ac.kr).
Abstract
OBJECTIVE. The purpose of this article was to research the clinical
and imaging features of sex cord-stromal tumors of the ovary to help in
specific diagnosis of ovarian tumors. Sex cord-stromal tumors of the ovary are
rare ovarian neoplasms, which arise from stromal cells and primitive sex cords
in the ovary. The common types are granulosa cell tumors, fibrothecomas,
sclerosing stromal tumors, and Sertoli-Leydig cell tumors. They account for
most of the hormonally active ovarian tumors. They have characteristic imaging
features in each type of the tumor.
CONCLUSION. Clinical and radiologic clues are helpful in
differential diagnosis from the more common epithelial tumors; sex
cord-stromal tumors primarily are treated surgically and have generally good
prognosis.
Introduction
Sex cord-stromal tumors of the ovary are rare, making up
approximately 8% of all ovarian neoplasms. These tumors arise from two groups
of cells in the ovary: stromal cells and primitive sex cords. Stromal cells
contain fibroblasts, theca cells, and Leydig cells, and primitive sex cords
include granulosa cells in the normal ovary, Sertoli cells in the testis, and
Sertoli cells in ovarian tumors
[1]. They differ from the more
common epithelial neoplasms in clinical and radiologic aspects. Understanding
the clinical and imaging features of sex cord-stromal tumor of the ovary is
helpful in specific diagnosis of ovarian tumors.
Sex cord-stromal tumors of the ovary affect all age groups and account for
most of the hormonally active ovarian tumors that show estrogenic effects or
virilization. Unlike patients with common epithelial tumors, of which 75% are
considered to be at stage III or IV at diagnosis, approximately 70% of
patients with these tumors are classified as having stage I lesions at
presentation. Consequently, sex cord-stromal tumors are primarily treated
surgically and have a generally good prognosis. In addition, these tumors may
have characteristic imaging features
[1,
2]. In this article, we show CT
and MRI findings of relatively common sex cord-stromal tumors of the ovary and
discuss points that differentiate these tumors from more common ovarian
epithelial tumors.
Granulosa Cell Tumors
Granulosa cell tumors of the ovary account for less than 5% of all
malignant ovarian tumors but represent the most common malignant sex
cord-stromal tumor and the most common clinically estrogenic ovarian tumor.
Adult granulosa cell tumors appear more often than the juvenile type and occur
usually in postmenopausal women. They can present with abnormal vaginal
bleeding and can be associated with endometrial hyperplasia, polyps, and
carcinoma (3-25% of cases). Granulosa cell tumors have potential for
clinically malignant behavior. Prognosis correlates with stage and age at the
time of the diagnosis. Most patients with these tumors have an excellent
prognosis (> 90% having a 10-year survival rate). They have a tendency of
late recurrence, even 10-20 years after diagnosis
[1-3].
Adult granulosa cell tumors show a spectrum of imaging manifestations due
to various histologic appearances and various arrangements of tumor cells.
They can have the appearance of solid masses (Figs.
1A, and
1B), tumors with hemorrhagic or
fibrotic changes, multilocular cystic lesions (Figs.
2A,
2B,
3A,
3B, and
3C), or completely cystic
tumors. Heterogeneity within a solid tumor is caused by intratumoral bleeding,
infarct, fibrous degeneration, and irregularly arranged tumor cells (Figs.
3A,
3B, and
3C). Multilocular cystic
appearances are produced by a predominantly macrofollicular pattern of
granulosa cell tumor, and these multiple cystic spaces are filled with watery
fluid or hemorrhage. In contrast to the more common epithelial tumors,
granulosa cell tumors are confined to the ovary at the time of diagnosis with
less propensity for peritoneal seeding and are only rarely bilateral. They may
rupture and result in hemoperitoneum
[2-5].
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Fig. 2B —45-year-old woman with granulosa cell tumor. Axial
T1-weighted image obtained after gadolinium administration shows marked
enhancement of septa and solid portions with multiple small cystic areas
within tumor, resulting in spongy appearance.
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Fig. 3A —55-year-old woman with granulosa cell tumor. Axial
T1-weighted image shows large well-defined mass with multiple cystic areas of
extremely low signal intensity and tiny spots (arrows) with high
signal intensity that represent intratumoral hemorrhage.
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Fig. 3B —55-year-old woman with granulosa cell tumor. Axial
T2-weighted image shows large multicystic mass with some solid portion. This
is called bunch-of-grapes appearance of cystic tumor with multiple
chambers.
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Fibroma, Fibrothecoma, and Thecoma
Fibroma, fibrothecoma, and thecoma form a spectrum of benign tumors of the
ovary. These tumors constitute approximately 4% of all ovarian neoplasms and
occur in both pre- and postmenopausal women. Fibroma is the most common sex
cord tumor. Although fibroma arising from nonfunctioning stroma shows no
estrogenic activity, lipid-rich thecoma can show estrogenic activity. Fibromas
can be associated with Meigs' syndrome (ascites, an ovarian tumor, and a
right-sided pleural effusion). Fibrosarcomas are rare
[1,
2,
6].
Because fibromas have abundant collagen and fibrous contents, these tumors
show relatively diagnostic imaging findings. The mass appears as a homogeneous
solid tumor with delayed enhancement on CT (Figs.
4A, and
4B) and as a hypointense mass
on T1-weighted MRI with very low signal intensity on T2-weighted imaging
(Figs. 5A, and
5B). Dense calcifications are
often seen. Scattered high-signal-intensity areas in the mass indicate edema
or cystic degeneration [2]
(Figs. 6A,
6B, and
6C).
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Fig. 4B —34-year-old woman with exophytic fibroma. After contrast
administration, CT scan shows homogeneously enhanced anterior mass
(arrows) as pedunculated fibroma and posterior heterogeneous
structure as right ovary.
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Fig. 5A —28-year-old woman with bilateral fibromas. Upper pelvic level
(A) and lower pelvic level (B) multilobulated
low-signal-intensity masses are noted in both adnexal regions on T2-weighted
images. Masses have typical dark signal intensity. Ovarian follicles
(arrows) are noted in left adnexal area.
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Fig. 5B —28-year-old woman with bilateral fibromas. Upper pelvic level
(A) and lower pelvic level (B) multilobulated
low-signal-intensity masses are noted in both adnexal regions on T2-weighted
images. Masses have typical dark signal intensity. Ovarian follicles
(arrows) are noted in left adnexal area.
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Fig. 6C —35-year-old woman with fibrothecoma. Gadolinium-enhanced
fat-suppressed T1-weighted image reveals intense enhancement in most of mass.
Some areas of high signal intensity on T2-weighted image are not enhanced,
which represent cystic change and edema.
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Fibroma can be a cause of adnexal torsion. The CT and MRI findings in
adnexal torsion with fibroma include tube thickening, ascites, deviation to
the twisted side, hemorrhage in the thickened tube, and torsion knot. It is
difficult to diagnose hemorrhagic infarction after adnexal torsion because the
fibroma is a solid tumor. The finding of a high-signal-intensity area in the
periphery of the mass on T1-weighted imaging is helpful in diagnosis of
hemorrhagic infarction of fibroma
[7] (Figs.
7A,
7B,
7C, and
7D).
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Fig. 7A —60-year-old woman with torsion of left ovarian fibroma. She
presented with aggravation of back pain. Sagittal (A) and axial
(B) T2-weighted images show well-circumscribed solid mass with
heterogeneous dark signal intensity in left adnexal region. Increased signal
intensity area of anterior aspect of mass represents edema. Note whorled
structure (arrows) abutting anterior margin of ovarian mass, finding
that suggests twisted vascular pedicle.
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Fig. 7B —60-year-old woman with torsion of left ovarian fibroma. She
presented with aggravation of back pain. Sagittal (A) and axial
(B) T2-weighted images show well-circumscribed solid mass with
heterogeneous dark signal intensity in left adnexal region. Increased signal
intensity area of anterior aspect of mass represents edema. Note whorled
structure (arrows) abutting anterior margin of ovarian mass, finding
that suggests twisted vascular pedicle.
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Fig. 7D —60-year-old woman with torsion of left ovarian fibroma. She
presented with aggravation of back pain. After gadolinium administration, mass
is faintly enhanced in areas of low signal intensity on T2-weighted image.
Ovarian mass proved to be fibroma without any congestion or necrosis at
surgery. Torsion of pedicle was 270°.
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Sclerosing Stromal Tumor of Ovary
Sclerosing stromal tumors are rare benign ovarian tumors that occur
predominantly in young women. The most common clinical symptom is menstrual
irregularity. Ascites may be seen but is rare. Surgical removal of the tumor
is curative, and there is no local or distant recurrence
[2,
8].
Imaging findings include a large mass with hyperintense cystic components
(Figs. 8A,
8B, and
8C) or a heterogeneous solid
mass of intermediate to high signal intensity on T2-weighted MRI (Figs.
9A, and
9B). The thick peripheral
hypointense rim on T2-weighted imaging is a compressed ovarian cortex due to a
slow-growing tumor. There is striking contrast enhancement with internal small
cleft and cysts (Figs. 8A,
8B,
8C,
9A, and
9B). On dynamic
contrast-enhanced images, the tumors reveal early peripheral enhancement with
centripetal progression. Striking early enhancement reflects the cellular
areas with their prominent vascular networks, and an area of prolonged
enhancement in the inner portion of the mass represents the collagenous
hypocellular area. These findings can be useful in differentiating sclerosing
stromal tumor from fibroma because fibroma shows absence of early enhancement
and delayed accumulation of the contrast material
[8-10].
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Fig. 8A —34-year-old woman with sclerosing stromal tumor. She
complained of vaginal spotting during ovulation. Axial T1-weighted image shows
well-defined pelvic mass with slightly hyperintense peripheral portion
(arrows) and irregular central hypointense area.
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Fig. 8B —34-year-old woman with sclerosing stromal tumor. She
complained of vaginal spotting during ovulation. Axial T2-weighted image
revealed mass with marked hyperintense central area and slightly hyperintense
periphery (arrows).
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Fig. 8C —34-year-old woman with sclerosing stromal tumor. She
complained of vaginal spotting during ovulation. Gadolinium-enhanced
fat-suppressed T1-weighted image reveals very intense enhancement of periphery
of tumor.
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Fig. 9A —27-year-old woman with sclerosing stromal tumor. She had
menstrual irregularity and lower abdominal discomfort. Sagittal T2-weighted
image shows round mass (arrows) protruding from ovary. Mass has
homogeneous intermediate signal intensity. Flow voids are also seen between
mass and ovary.
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Fig. 9B —27-year-old woman with sclerosing stromal tumor. She had
menstrual irregularity and lower abdominal discomfort. Gadolinium-enhanced
T1-weighted sagittal images show very intense enhancement of mass. At surgery,
pedunculated sclerosing stromal tumor from ovary was confirmed.
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Sertoli-Leydig Cell Tumor
Sertoli-Leydig cell tumors are the most common virilizing ovarian tumor,
but they are very rare (< 0.5% of ovarian tumors). They occur usually in
young women. In 40-50% of patients, the presenting symptoms are related to
clinical signs of androgenic activity
[1]. The tumors are
microscopically ranged from well-differentiated to poorly differentiated.
Behavior correlates with degree of differentiation and stage. Most of these
tumors behave in a benign fashion
[11]. In contrast to granulosa
cell tumors, Sertoli-Leydig cell tumors tend to recur relatively soon after
initial diagnosis. They are almost always unilateral tumors that can be solid,
solid and cystic, and cystic—or even papillary. The mass appears as a
well-defined enhancing solid mass with intratumoral cysts on CT and as
hypointense with multiple variable-sized cystic areas on MRI (Figs.
10A,
10B,
10C, and
10D). Low signal intensity on
T2-weighted imaging depends on the extent of fibrous stroma. Multicystic areas
develop as a result of heterologous elements
[1,
2].
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Fig. 10B —56-year-old woman with poorly differentiated Sertoli-Leydig
cell tumor. Axial (B) and sagittal (C) T2-weighted images show
multilobulated mass with intermediate signal intensity with multiple tiny
high-signal cysts (arrows).
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Fig. 10C —56-year-old woman with poorly differentiated Sertoli-Leydig
cell tumor. Axial (B) and sagittal (C) T2-weighted images show
multilobulated mass with intermediate signal intensity with multiple tiny
high-signal cysts (arrows).
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Fig. 10D —56-year-old woman with poorly differentiated Sertoli-Leydig
cell tumor. After gadolinium administration, fat-suppressed T1-weighted image
shows that mass is intensely and heterogeneously enhanced.
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Steroid Cell Tumor
Steroid cell tumors are very rare ovarian tumors that affect patients from
a wide range of ages but usually those in the fifth or sixth decade of life.
Most steroid cell tumors are virilizing, and rare cases are associated with
Cushing's syndrome. Approximately one third of these tumors behave in a
clinically malignant fashion. Steroid cell tumors are usually small (< 3
cm) nodules and virtually always unilateral. The tumor manifests as a small
mass with hyperintense areas on T1-weighted images due to abundant
intracellular lipid and intense enhancement due to rich vascularity
[1,
12] (Figs.
11A,
11B, and
11C).
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Fig. 11A —41-year-old woman with steroid cell tumor. She presented with
amenorrhea and weight gain. Laboratory findings revealed elevated testosterone
level. Axial T1-weighted image shows small ( 3 cm) ovoid hypointense mass
(arrows) in left adnexal region. Ill-defined area of high signal
intensity is noted, indicating lipid content.
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Fig. 11B —41-year-old woman with steroid cell tumor. She presented with
amenorrhea and weight gain. Laboratory findings revealed elevated testosterone
level. On T2-weighted image, mass (arrows) is heterogeneously
hyperintense.
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Fig. 11C —41-year-old woman with steroid cell tumor. She presented with
amenorrhea and weight gain. Laboratory findings revealed elevated testosterone
level. After gadolinium administration, coronal fat-suppressed T1-weighted
image shows tumor (arrows) is very intensely enhanced.
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Summary
Sex cord-stromal tumors of the ovary are rare ovarian neoplasms that arise
from stromal cells and primitive sex cords in the ovary. These tumors may have
characteristic clinical and radiologic features. They affect all age groups
and account for most of the hormonally active ovarian tumors. Most of them are
stage I lesions at presentation with good prognosis. Granulosa cell tumors are
usually large multiloculated cystic masses with variable solid portions. The
tumors are associated with endometrial abnormalities. Fibromas and thecomas
are usually solid masses with dark signal intensity on T2-weighted MRI and
variable extent of calcification or degeneration. Sclerosing stromal tumors
show typical early peripheral enhancement with centripetal progression.
Sertoli-Leydig cell tumors appear as well-defined, enhancing solid masses with
variable-sized intratumoral cysts. Steroid cell tumors show a heterogeneous
solid mass with internal areas of intracellular lipid.
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Abstract
Introduction
