Ovarian steroid cell tumors account for less than 0.1% of all ovarian tumors. They are grouped under sex-chord stromal tumors and are usually benign, unilateral and characterized by a steroid cell proliferation [1
They are further divided into three subtypes: stromal leuteoma, Leydig cell tumor, and steroid cell tumor [1
], the latter being the most common of the three subtypes accounting for approximately 60% of cases.
Steroid cell tumors are associated with androgenic changes with variable frequency, ranging from 12% to over 50 % [1
], and they are usually of many years duration[1
]. This case is unique because of its acute presentation, with sudden onset of amenorrhea and signs of virilization, over a four month period, following resumption of menses after a normal delivery.
Historically these tumors have been variously referred to as lipoid cell tumors, lipid cell tumors, adrenal-like tumors, masculinovoblastomas, leutomas, hypernephroid tumors, and adrenal rest tumors, indicating the infidelity of their supposed lineage [1
]. However, it is postulated that these are of adrenal cortical rest origin, residing near the ovary, on the basis of the presence of mRNA for steroidogenic p450c11 and p450c21, which are expressed only in tissues of adrenal origin [7
]. However this theory is still disputed [1
The majority of steroid cell tumors have a benign or low-grade behavior. Interestingly, pathologically-benign tumors can behave in a clinically malignant fashion [8
]. About 20% patients develop metastatic lesions usually within the peritoneal cavity, and rarely at distant sites [2
]. In a series of 63 cases from the Massachusetts General Hospital, 94% of the tumors were found to be unilateral, and 28.6% cases had features suggestive of malignant nature [2
Hayes and Scully [2
] have identified five pathologic features that are highly associated with malignancy: More than two mitoses per high-power field; necrosis; size of 7 cm or larger; hemorrhage; and Grade 2 or 3 nuclear atypia. The final pathologic diagnosis is based on the characteristic histological appearance, including polygonal to round cells with distinct cell borders and an abundant cytoplasm and the absence of Reinke's crystals, and a characteristic pattern of immunohistochemistry staining for inhibin and vimentin [75%], anti human cytokeratin [46%], and AE1/AE3 [37%] [1
The primary treatment is surgical extirpation of the primary lesion, and there are no reports of effective radiation or chemotherapy [10
]. In a young patient with stage IA disease, a unilateral salpingo-oophorectomy is adequate since the frequency of bilateral occurrence is only 6% ( [1
The main reason for poor understanding of the therapeutic value of chemotherapy and radiotherapy in the treatment of these tumors is due to the rarity of this tumor. In recent years attempts have been made to describe the use of Gonadotrophin Releasing Hormone [GnRH] analogues to induce a suppression of secretions and an apoptosis leading to a non-surgical cure [11
]. Pascale, Pugeat and Roberts suggested that androgen secretion by ovarian virilizing tumor is gonadotrophin dependent [11
]. They concluded that various ovarian androgen secreting tumors are not autonomous but apparently depend on continuous gonadotrophin stimulation.
Imai A, Lida K, Tamaya T suggested the various relationships between GnRH receptor and tumor growth. Increased Fas ligand level within GnRH receptor-bearing tumors might promote apoptotic cell death through an attack on intra-tumoral Fas positive cells that could, at least in part, account for the anti-proliferative action of the hormone [12
]. The coupling of GnRH receptor to the Gi protein subfamily may be a signaling pathway by which GnRH acts in peripheral tumor [12
So far these approaches have been tried primarily in non-operable cases or recurrent disease. Therefore, at best, these treatments can be considered experimental in the present times. Further insight into the nature, biology and behavior of these tumors may change the gold standard of treatment from surgical extirpation to medical management over time.