The authors have declared that no competing interests exist.
Steroid cell tumors of the ovary are particularly rare, secreting sex hormones, characterized by steroid cell proliferation and represent only 0.1% of all ovarian tumors. They are classified into three subtypes according to their cell of origin: stromal luteoma, Leydig cell tumors and a third subtype of unknown lineage corresponds to a not specified steroid cell tumor (SCT-NOS). This third subtype accounts for 60% of steroid cell tumors. The clinical manifestations of SCT-NOS can take many forms, including pain, abdominal distension, but perhaps the most visible presentations are those related to hormonal activity and virilization of the tumor. We present a rare case of a 48-year-old woman with vaginal bleeding and a history of trunk obesity, hirsutism for 2 years and hypothyroidism with hormone replacement therapy. Clinical examination revealed a characteristic of obesity, virilization. Serum testosterone was 3.62 µg / L and CA-125 was 40.67. Magnetic resonance imaging identified a left ovarian solid mass and histopathology confirmed a steroid cell tumor not specific. The patient underwent exploratory laparotomy and left salpingoophorectomy. Macroscopically, the mass is well circumscribed, solid, homogeneous and yellowish. Microscopically, the tumor is mainly composed of eosinophilic or vacuolar granular cytoplasm. Immunohistochemistry showed that the tumor cells were strongly positive for inhibin. The postoperative period was uneventful. Through this rare observation, we will discuss the aspects that characterize this type of tumor and present some guidelines to be used in the differential diagnosis, as well as the difficulties encountered in the clinical, radiological and therapeutic fields.
Steroid cell tumors of the ovary are particularly rare, accounting for 0.1% of all ovarian tumors. Steroid cell tumors a not otherwise specified (SCT-NOS) are a rarely subtype stromal tumors of the ovarian sex cord, which have a difficult clinical and radiological presentation. A few cases of SCT-NOS have been described in the literature. Appropriate evaluation is recommended for patients with symptoms of virilization such as hirsutism, amenorrhea, and ovarian mass. The histological, clinical, and radiological features of SCT-NOS are briefly summarized. A discussion of the differential pathologic, radiologic diagnostic criteria, guidelines, and treatment options appropriate for this tumor are presented in this article along with a review of the literature.
A 48-year-old premenopausal woman in her 48th year had hair growth on her face and back over the past 2 years, bleeding from her vagina for 3 weeks and lower abdominal pain for 1 week. She had a history of recent weight gain, voice changes and medication use. There was a history of hypertension and hypothyroidism with hormone replacement therapy. There was no history of gynecological or breast tumors in the family. The examination revealed a patient with no objective clinical signs. Her BMI was 34.81 kg/m2 with a weight of 91 kg and a height of 167 cm. Systemic examination was normal. The patient was evaluated for bleeding and androgen excess characteristics. Pelvic ultrasound showed a normal uterus and a left ovarian mass of 5 cm. The DHEAS was 117 µg / dL and serum testosterone were elevated to 6.3 ng / mL (normal 0.1 - 1.2 ng / mL). CA-125 was 40.67 IU/mL, LH was 0.07 MIU/mL (normal 15.9-54 MIU/mL) and FSH was 0.37 MIU/mL (normal 23.0-116.3 MIU/mL). The dexamethasone suppression test suppressive cortisol by 19.29-0.81 µg/mL
Une IRM realized without and with contrast Gadolinium, in sequence T1,T2, DIFFUSION and confirmed one left masse ovarian
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A stepwise laparotomy was performed, peritoneal fluid was sent for cytological analysis. There was a 4x4 cm friable yellow mass on the left ovary (
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Histology shows no necrosis or intra-tumor hemorrhage. Less than one mitosis for every 10 W.H.P.'s. Nuclear atypia is moderate.
Crystals of Reinke were not seen. The staining of Oil-red-O has revealed numerous intracellular lipid droplets. No mitotic figures were identified. No hemorrhagic or necrotic contents were noted.
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Ovarian steroid cell tumors are a rare tumor that secrete sex hormones and are characterized by a proliferation of steroid cells. They account for less than 0.1% of all ovarian tumors and are grouped into three subtypes according to their cells of origin: stromal luteoma, Leydig cell tumor (or hilus) and steroid cell tumor not otherwise specified (SCT-NOS)
The first description and term of steroid cell tumor not otherwise specified was given in 1979 by Scully
They are mostly benign, diagnosed early at stage I at presentation with good prognosis, however malignant development with metastasis occurs in 25 to 40% of cases
This tumor produces several steroids, particularly testosterone, and are associated with androgen changes in 55-75% of cases
However, 25% of patients with SCT- NOS, are asymptomatic, non-functional tumor
To make a definite diagnosis of an endocrine tumor of ovarian origin, hormonal tests will be required. Elevated testosterone levels with normal levels of DHEA, LH, FSH and 17-hydroxyprogesterone are indicative of a virilizing tumor of the ovary.
There are three main markers of ovarian cancer: CA 125, CA 19-9 and ACE
MR imaging and color flow Doppler ultrasound are two excellent imaging modalities that may demonstrate characteristic radiologic features and characterization differential diagnosis for these tumors.
Sonography is generally used as the first-line imaging technique for the evaluation of ovarian pathologic abnormalities. It makes it possible to specify the tumor ovarian origin; its structure, dimensions, solid and/or cystic component, its vascularization, either unitary or bilateral, also show its extension to adjacent organs. The SCT-NOS ovarian tumor varies in size from 1.5 cm to 40 cm and is bilateral in 6% of cases
MR imaging is useful for identifying these tumors in young and postmenopausal women
Laparoscopy is indicated when the ultrasound scan is not formal and when there is doubt about the organic nature of the lesion. It eliminates non-ovarian lesions, determines the ovarian tumor and makes an etiological diagnosis by cytological punctures of the mass.
Macroscopically, these tumors are typically very limited, well circumscribed and unilateral, yellow to orange, red or brown in color depending on the lipid content. It varies in size from 1 cm to 45 cm, with a generally solid consistency, but a combination of solid and cystic forms can be observed. In our case, the tumor was entirely solid, with no cystic area. Its cross-sectional area was generally yellow and lobulated
Histological examination allows the definitive diagnosis and is confirmed by immunohistochemistry. Cells with abundant granular or vacuolar eosinophilic cytoplasm, positive for fat spots and negative for Reinke's crystals and stromal hyperthecosis were found
SCT-NOS steroid cell tumors should be differentiated from other types of steroid cell tumors, in which the development of steroid hormone-producing cells occurs secondarily.
This includes granulosa cell tumors, fibroids and thecomas, stromal luteoma and Leydig cell tumor.
1) Granulosa cell tumors are the most common malignant stromal tumors and are often revealed at stage 1 at diagnosis. They constitute the main group of hormone-secreting ovarian tumors and are subdivided into two different clinicopathological subtypes: adult and juvenile, depending on age, and are distinguished by size and solid or cystic type
2) Fibroma, fibro-thecomas and thecomas are benign ovarian tumors, accounting for 4%-6% of all ovarian tumors and affect pre- and post-menopausal women. Thecomas are stromal tumors composed of theca-type lipids, as well as lutein cells and fibroblasts
Fibromas are composed of a non-functional stroma with no estrogenic activity, formed exclusively of collagen-producing spindle cells containing only small amounts of cytoplasmic lipids
3) Sclerosing stromal tumors (SSTs) were first described by Chalvardijaian and Scully in 1973
4) Sertoli-Leydig cell tumors are very rare and benign and occur in young women with clinical androgenic symptoms and account for less than 0.5% of ovarian tumors
the dominant hilar form, consisting of a small benign unilateral nodule. Leydig cells are well limited with Reinke's crystalloids in 50% of cases
the non-hilar form consists of tumors of the ovarian stroma, located in the ovarian parenchyma and containing 100% Reinke's crystalloids.
Anatomopathological, they are small reddish-brown lesions with eosinophilic cells with low cytoplasmic lipid content, presenting mainly intracytoplasmic Reinke crystals. Their nuclei are hyperchromatic and have only one small nucleolus. Mitoses are rare and always benign
The basic therapy for steroid-induced ovarian tumors is total abdominal hysterectomy with bilateral salpingo-oophorectomy and complete surgical staging. However, for women who wish to maintain their fertility, conservative surgical treatment with unilateral oophorectomy may be accepted.
Surgical management of SCT-NOS should be prompt, imperative, based on two essential elements: histological type and patient desires.
In the first instance, satisfactory resection of the tumor must be carried out for any benign or malignant case with multiple variants
-Total hysterectomy with bilateral salpingectomy for elderly women who do not wish to preserve the uterus.
For young women, before the age of 40, conservative treatment is applied in the case of benign tumors, with a conservative cystectomy to preserve ovarian function.
Uni-lateral salpingo-ovarectomy in women of child-bearing age with a benign tumor.
Unilateral salpingo-ovarectomy with malignant tumor and preservation of the uterus for future fertility, but close monitoring with staging is essential.
Metastatic malignant tumour, requires post-operative adjuvant chemotherapy BEP, Bleomycin, Etoposide and Cisplatin. The best adjuvant chemotherapy is still unknown
In our case, the patient is young and eager to have children, and has had a successful unilateral sapling-oophorectomy.
For this, it is important to establish a close correlation between various clinical, pathological parameters and the undesirable behavior of the tumor, such as the age of patients in the clinical stage, tumor size greater than 7, 0 cm or more (78% malignant tumors), pituitary field mitoses greater than 2 mitoses (92% malignant tumors), grade 2-3 nuclear atypia (64% malignant tumors), necrosis (86% malignant tumors) and hemorrhage (77% malignant tumors)
Regular monitoring with measurement of serum testosterone levels is mandatory, and since little is known about the behavior of these tumors, it is not known for how long.
In our case, the prognosis is favorable since the size of the tumor is less than 7 cm, without mitosis, necrosis or hemorrhage. Clinically malignant tumours occur in 25 to 43% of cases, whereas clinically malignant cases are histologically benign
S. No | Tumor | Typical Tumor Morphology | Key Imaging Features |
1 | GCT | ||
Juvenile | Multicyclic | Hemorrhage, pseudoprecocious puberty | |
Adult (polyps, earcinoma) | Solid or multicystic and solid | Hemorrhage, spongelike cysts in tumor, endometrial abnormalities | |
2 | The coma | Solid | Nonspecific |
3 | Fibroma | Solid | Sound attenuation at US, low signal at T2 weighted MR imaging |
4 | Sertoli-Leydig tumor | Solid | Nonspecific |
5 | Steroid cell tumor | Solid | Nonspecific |
SCT-NOS are particularly rare stromal tumors of the ovarian sex cord, which are difficult and misleading to diagnose clinically and radiologically, as this observation demonstrates. For early diagnosis, careful physical examination, supplemented by laboratory values and imaging, is useful in patients with symptoms of virilization and amenorrhea. In most cases, typical histopathological analyses confirm the diagnosis. In case of difficulties, immunohistochemistry is an important diagnostic tool. Treatment depends on the histology, surgical stage and the desire to preserve fertility. The treatment of malignant tumors should combine surgery and chemotherapy.
We thank Dr Aida Darouichi for professional advice and support during this study.