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Ovarian steroid cell tumor, not otherwise specified, with

virilization symptoms Article Tools
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Yi-Jou Tai, Wen-Chun Chang, Kuan-Ting Kuo, Bor-Ching Sheu
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DOI: http://dx.doi.org/10.1016/j.tjog.2013.04.037 | Email Article

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Open access funded by Taiwan Association of Obstetrics & Gynecology
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Steroid cell tumors are rare sex cord tumors that account for fewer than 0.1% of all ovarian tumors. Most of
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these tumors produce steroids, particularly testosterone, and patients present clinically with symptoms of (100 minimum order)
virilization [[1], [2]]. The tumors can be categorized into three subtypes according to cell origin, stromal luteomas
arising from ovarian stroma, Leydig cell tumors arising from hilar Leydig cells, and steroid cell tumors, not
otherwise specified (NOS), when the origin is unknown. Steroid cell tumors, NOS, are often associated with
virilization symptoms [[1], [2]], although an association with estrogenic manifestations has also been reported.
We present a rare case of steroid cell tumor, NOS, with an initial presentation of infertility.

A 29-year-old nulliparous woman presented to our outpatient department for consultation regarding infertility.
She had been married for 4 years, but was not able to conceive. She had visited a local hospital 3 years
previously because of infertility; semen analysis of her spouse and hysterosalpingography showed no
abnormalities. Sonographic examination at that time revealed one 6-cm ovarian tumor, but the patient declined
further management.

The patient experienced intermittent vaginal bleeding and irregular menstruation the previous year but no
abdominal discomfort or change in bowel habits. She visited our infertility clinic and her endocrine profile was
checked as part of her infertility workup. The patient had an elevated testosterone level (1.35 ng/mL), but other
hormone levels (follicle-stimulating hormone, luteinizing hormone, prolactin, estradiol, progesterone) were within
normal limits. The patient was overweight (body mass index 28.9) and had facial acne and signs of hirsutism
(prominent on upper lip, arms, and thighs). Vaginal examination showed an enlarged clitoris and one fetal head-
sized, firm adnexal tumor. During the previous year, the patient experienced occasional intermenstrual vaginal
spotting and a change in the duration of menstruation from 1 week to 1 day. Detailed sonographic examination
at our clinic revealed one 10 cm 4 cm solid tumor with heterogenous echogenicity and prominent flow (Fig. 1).
The patient was then referred to our oncology clinic where tumor markers were checked for suspected
malignancy. Her serum cancer antigen-125 level was slightly elevated (84.8 /mL), with normal levels of alpha
fetoprotein (1.99 ng/mL), lactate dehydrogenase (260 U/L), and carcinoembryonic antigen (1.84 ng/mL).
Computed tomography revealed normal kidneys and adrenal glands, and one 10 cm 9 cm 6.5 cm solid
ovarian mass with a small amount of ascites (Fig. 2). A right ovarian tumor composed of multilobulated bright
yellowish nodules, with grossly diuse hemorrhage and gelatinous changes, was found on laparotomy (Fig. 3).
An immediate pathological frozen section revealed a steroid cell tumor; a right adnexectomy was performed. A
detailed microscopic pathological examination showed a well-encapsulated ovarian tumor composed of
uniformly ovoid or polygonal cells with small, centrally located nuclei, clumped chromatin, and eosinophilic
granular to clear vacuolated cytoplasm (Fig. 4). The tumor cells were dispersed into large lobules and small
nests by fibrous septae and a capillary network, respectively (Fig. 5). Foci of hemorrhage, focally prominent
vasculature, and degenerative changes were noted. There was little mitosis (less than 1 per 10 high-power
fields). The tumor cells reacted strongly positive to Sudan III and Oil Red fat stains (Fig. 6).
Immunohistochemically, the tumor cells were positive to alpha-inhibin and focally equivocally positive to S100,
but negative to cytokeratin (AE1 and AE3), MDM-2, and HMB-45. Steroid cell tumor, NOS was the final
diagnosis.The patients postoperative course was smooth and she was discharged from the hospital on the 4th
day after surgery. Two weeks postoperatively, her serum testosterone level had decreased to a normal level
(0.32 ng/mL). Her acne and facial hair also markedly diminished. She was regularly followed for 24 months at
our clinic, and her menstruation cycle and hormone profile were normal.
Fig. 1
A sonogram revealed one pelvic mass with heterogeneous content.

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Fig. 2
Computed tomographic scan showed one 10-cm hypervascular, heterogeneous mass in the pelvic cavity.

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Fig. 3
Cut surface of the tumor showed multilobulated, bright yellow nodules with foci of gelatinous change and
hemorrhage.

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Fig. 4
The tumor is composed of uniformly ovoid or polygonal cells with small, centrally located nuclei and
eosinophilic granular cells to clear vacuolated cytoplasm.

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Fig. 5
 The tumor cells are dissected into large lobules and small nests by fibrous septae and capillary network.

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Fig. 6
Tumor cells are strongly positive for Oil Red fat stains.

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Ovarian steroid cell tumors are rare sex cord stromal tumors, accounting for 0.1% of all ovarian tumors [[3], [4]].
These tumors are typically solid with a yellow or orange section surface because of intracytoplasmatic lipids.
Generally, steroid cell tumors can originate from either the ovaries or ectopic adrenal glands [[3], [4], [5]]. Steroid
cell tumors, NOS, account for approximately 60% of the three steroid cell tumor subtypes [6].

Steroid cell tumors, NOS, can be seen at all ages, and at a mean age of 43 years. They cause androgenic and
estrogenic manifestations in 50% and 10% of patients, respectively. The most common manifestations of the
hormonal eect from steroid cell tumor NOS, are caused by testosterone production. Approximately 5677% of
patients present with hirsutism and virilization symptoms, such as a husky voice, male pattern baldness, acne,
and increased facial and chest hair [7]. Estradiol secretion occurs in 623% of patients and results in irregular
menstruation or postmenopausal bleeding [8]. Approximately 25% of steroid cell tumors NOS, do not produce
hormones. An association with Cushing syndrome, which causes hypokalemia and hypertension, has also been
reported. In the current case, the levels of serum adrenocorticotropic hormone, cortisol, and electrolytes were
all within normal ranges.

In contrast to the benign behavior of stromal luteoma and Leydig cell tumors, steroid cell tumors NOS have a
guarded prognosis [9]. Although they are unilateral and amenable to unilateral oophorectomy when apparently
confined to one ovary, as many as 20% of cases have tumor cell seeding and/or extension beyond the ovary at
the time of diagnosis. For women who have completed childbearing, optimal staging with a total abdominal
hysterectomy and bilateral salpingooophorectomy is indicated. The predictive pathological characteristics of
malignancy proposed by Hayes and Scully [8] are two or more mitotic figures per 10 high-power fields (92%),
size exceeding 7 cm (78%), Grade 23 nuclear atypia (64%), necrosis (86%), and hemorrhage (77%) within the
tumors. In their series, 28.6% cases had features suggestive of malignancy. It is reported that 2543% of
steroid cell tumors, NOS, are malignant [8].

In a young patient with clinical stage IA disease, such as the patient in the current case, unilateral adnexectomy
is primarily considered because bilateral tumors only occur in approximately 6% of cases [8]. Adjuvant
chemotherapy or radiation should be considered based on histopathology and survival staging [8] but their
therapeutic eect is poorly understood. Because ovarian stromal tumors occur infrequently, chemotherapy
regimens have been conducted rarely in sporadically reported cases. Empirical chemotherapy with bleomycin,
etoposide, and cisplatin (BEP) for ovarian stromal tumors, such as granulosa cell or Sertoli-Leydig cell tumors,
has been reported to be eective for metastatic disease [[9], [10], [11]]. For malignant steroid cell tumors, NOS
and stromal tumors as well, BEP can be considered an eective and practical chemotherapy regimen. Our
patient had none of the aforementioned risk factors [8] except for a large tumor (exceeding 7 cm), and therefore
adjuvant therapy was not implemented. Empirical gonadotropin-releasing hormone analogue treatment has
been proposed for gonadotropin-dependent virilizing steroid cell tumors [12]. Regular monitoring of
postoperative steroid hormone levels is suggested for further adjuvant therapy.

References
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7. Wang, P.H., Chao, H.T., Lee, R.C., Lai, C.R., Lee, W.L., Kwok, C.F. et al. Steroid cell tumor of the ovary:
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8. Hayes, M.C. and Scully, R.E. Ovarian steroid cell tumors (not otherwise specified): a
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9. Homesley, H.D., Bundy, B.N., Hurteau, J.A., and Roth, L.M. Bleomycin, etoposide, and cisplatin
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Treatment of metastatic stromal tumors of the ovary with cisplatin, doxorubicin, and
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11. Surry, E.S., de Ziegler, D., Gambone, D.O., and Judd, H.L. Preoperative localization of androgen
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12. Pascale, M.M., Pugeat, M., and Roberts, M. Androgen suppresive eect of GnRH agonist in ovarian
hyperthecosis and virilizing tumors. Clin Endocrinol. 1994; 41: 571576
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2014 Published by Elsevier Inc.

< Previous Article June 2014 Volume 53, Issue 2, Pages 260262 Next Article >

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