Juvenile granulosa cell tumor of the ovary - Applied Radiology Online

R A D I O L O G I C A L C A S E
Juvenile granulosa cell tumor
of the ovary
Jeremiah R. Long, MD, and David Danielson, DO
CASE SUMMARY
A 3-year-old girl presented to the
emergency department with an abdominal
mass, symptoms of bowel obstruction,
and evidence of precocious pu berty.
Imaging studies were ordered.
IMAGING FINDINGS
An abdominal radiograph was initially
obtained and raised suspicion for
a possible intussusception (Figure 1).
A subsequent abdominal ultrasound
found a large complex low anterior
abdominal mass abutting the uterine
fundus measuring 10 × 6.6 × 9.3 cm
(Figure 2). Laboratory analysis revealed
a dramatically elevated serum estradiol
level.
Axial computed tomography (CT) of
the abdomen and pelvis was performed
following the administration of oral and
intravenous contrast material. The enhancing
mass demonstrated both solid
and cystic components. There was free
fluid within the abdomen and pelvis, but
no lymphadenopathy. Prominent subareolar
breast tissue was noted on images
through the lower chest.
Based upon these findings, the patient
underwent laparotomy for tumor
FIGURE 1. This frontal abdominal radiograph
depicts the relative paucity of bowel
gas and the suggestion of a soft tissue
mass within the right lower quadrant.
FIGURE 2. These selected gray-scale ultrasound images of the abdomen show a sharply
marginated, complex cystic and solid mass within the low anterior abdomen at the midline.
The mass measured 10 × 6.6 × 9.3 cm and abutted the uterine fundus. The endometrial
stripe measured approximately 7 mm.
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R A D I O L O G I C A L C A S E
FIGURE 3. Selected CT images of the abdomen and pelvis following the administration of oral and intravenous contrast material depict the
well-circumscribed, heterogeneous, enhancing mass. The mass contained both cystic and solid components but no calcifications. There
was free fluid within the abdomen and pelvis. No lymphadenopathy was present. Prominent subareolar breast tissue was noted on images
through the lower chest.
resection and unilateral salpingooophorectomy.
DIAGNOSIS
Juvenile granulosa cell tumor of the
ovary
DISCUSSION
Ovarian cancer is the third most common
neoplasm of the female genital
tract, after carcinoma of the cervix and
endometrium. It accounts for 6% of all
cancers diagnosed in females, and stands
as the leading cause of gynecological
cancer death. This is primarily because
80% of women with ovarian cancer present
with advanced-stage disease. 1
Based upon their cell type of origin,
primary ovarian malignancies are classified
into surface epithelium, germ
cell, or sex cord tumors. Sex cord tumors
account for 1% to 2% of ovarian
malignancies. They may contain granulosa
cells, thecal cells, Sertoli cells, or
fibroblasts of gonadal stromal origin.
While approximately two thirds occur
in postmenopausal women, they can
occur at any age, with 5% occurring in
prepubescent patients. Approximately
70% of sex cord tumors are granulosa
46 ■ APPLIED RADIOLOGY © www.appliedradiology.com November 2008

cell tumors. These tumors are often hormonally
active. Thecal cells (luteinized
cells within the stroma) produce estrogen,
and if present in large enough
quantities within a granulosa cell tumor,
can cause elevated serum estradiol levels.
Additionally, these tumors may also
secrete inhibin. Ovarian granulosa cell
tumors may also express unique cell
surface markers that can be detected by
staining sample tissue with histological
markers. Surface expression of inhibin
appears to have the greatest pathologic
diagnostic potential for this tumor type. 2
Among the histologic subtypes of
granulosa cell tumors, juvenile granulosa
cell tumors are rare, and typically
present early. In a study of 125 patients
with juvenile granulosa cell tumors,
78% presented within the first 2 decades
of life. 3 Clinically, these patients typically
present with signs of hyperestrogenism—precocious
puberty in prepubertal
patients, menstrual irregularities
in women of reproductive age, and
abnormal uterine bleeding in postmenopausal
women. 3 In addition, patients
may complain of abdominal pain
or an abdominal mass.
Management of granulosa cell
tumors (including the juvenile subtype)
begins with surgery for definitive tissue
diagnosis, staging, and debulking. Surgical
tumor removal typically includes
ipsilateral salpingo-oophorectomy for
patients wishing to retain fertility, and
total hysterectomy for older patients or
patients who no longer desire fertility.
The role of chemotherapy or radiation
therapy in the treatment of granulosa
cell tumors remains uncertain because
of the lack of prospective randomized
trials supporting their roles as adjuvant
agents. 4 The paucity of extensive clinical
trials reflects the overall rarity of
these tumors. Prognosis depends upon
surgical stage at presentation. Fortunately,
most patients are diagnosed at
stage I, and enjoy a favorable prognosis.
Patients diagnosed at stages II through
IV tend to have poor clinical outcomes
with a higher rate of disease recurrence.
Granulosa cell tumors in premenarchal
girls appear to have a better outcome
than those occuring in adult women. 5
CONCLUSION
Juvenile granulosa cell tumors of the
ovary represent a small fraction of all
primary ovarian malignancies. They
typically present within the first 2 decades
of life with signs of hyperestrogenism,
and an abdominal mass. As the
majority of these tumors are diagnosed
at stage I, their treatment remains surgical.
The role of chemotherapy and/or
radiation therapy remains unclear.
Given the possibility for recurrence,
long-term follow-up and surveillance is
recommended for all patients.
REFERENCES
1. Funt SA, Hann LE. Detection and characterization
of adnexal masses. Radiol Clin North Am.
2002;40:591-608.
2. McCluggage WG. Recent advances in immunohistochemistry
in the diagnosis of ovarian neoplasms.
J Clin Pathol. 2000;53:327-334.
3. Young RH, Dickersin GR, Scully RE. Juvenile
granulosa cell tumor of the ovary. A clinicopathological
analysis of 125 cases. Am J Surg Pathol.
1984;8:575-596.
4. Schumer ST, Cannistra SA. Granulosa cell tumor
of the ovary. J Clin Oncol. 2003;21:1180-1189.
5. Lack EE, Perez-Atayde AR, Murthy AS, et al.
Granulosa theca cell tumors in premenarchal girls:
A clinical and pathologic study of ten cases. Cancer.
1981;48:1846-1854.
Prepared by Jeremiah R. Long, MD,
Walter Reed Army Medical Center
Department of Radiology, Washington,
DC, and David M. Danielson, DO,
Madigan Army Medical Center Department
of Radiology, Tacoma, WA.
The views expressed in this article are
those of the authors and do not reflect
the official policy of the Department of
Army, Department of Defense, or U.S.
Government.
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November 2008