Androgen secreting steroid cell tumor of the ovary in a young lactating women with acute onset of severe hyperandrogenism: a case report and review of literature

Department of Surgical Oncology, Amrita Institute of Medical Sciences and Research Centre, Edapally, Kochi, Pin-682026, Kerela, India. .
Journal of Medical Case Reports 02/2007; 1(1):182. DOI: 10.1186/1752-1947-1-182
Source: PubMed


Steroid cell tumors of the ovary account for less than 0.1% of all ovarian tumors 1 and these tumours may present at any age in association with interesting presentations related to the hormonal activity and virilizing properties of tumor. Hayes and Scully 2 reported 63 cases in patients ranging from 2 to 80 years of age. The subtype, not otherwise specified, is associated with androgenic changes in approximately one half of patients with this tumour 1. In a series of 63 cases from Massachusetts General Hospital, 94% of the tumors were found to be unilateral and 28.6% were malignant 3. As most of these tumors are diagnosed at an early stage and do not recur or metastasize, little is known about their response to therapies such as chemotherapy or radiation 3.
We present the case of a 22-year old lactating woman who presented with four months of amenorrhea associated with signs of virilization. Clinical and diagnostic evaluation revealed a right adenexal mass and elevated serum levels of testosterone and she was diagnosed as having a stage 1A androgen secreting steroid cell tumor. In view of the early stage of the disease, she underwent right salpingo-oopherectomy. Histopathological examination and immunohistochemistry confirmed the diagnosis. Two months after surgery she regained normal menses and showed regression of the androgenic changes.
Surgery remains the mainstay of the treatment of gonadotrophin receptor positive steroid cell tumors although medical therapy using Gonadotrophin Releasing Hormone [GnRH analogues has been tried recently in recurrent or inoperable cases. There is no described effective chemotherapy or radiotherapy for this condition.

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    • "As in epithelial ovarian cancers, treatment of sex-cord tumours is surgery. There is no known chemotherapy agent or radiotherapy for sex-cord tumours, since these tumours are seen very rarely (Haji et al. 2007). "
    Journal of Obstetrics and Gynaecology 08/2014; 35(4):1-2. DOI:10.3109/01443615.2014.949227 · 0.55 Impact Factor
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    • "They are functional tumors and one of the ovarian virilizing tumors. They produce testosterone, leading to hyperandrogenism and virilization (hirsutism, voice deepening, clitoromegaly, increased muscle mass, et al) in woman who are mostly postmenopausal (7, 8, 11). "
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    ABSTRACT: Leydig stromal cell tumor is a rare ovarian tumor that belongs to the group of sex-cord stromal tumors. They produce testosterone leading to hyperandrogenism. We present a 41yr old woman with symptoms of virilization and a mass of right adenex via ultra Sonography, and a rise of total and free serum testosterone. An ovarian source of androgen was suspected and a surgery performed. A diagnosis of leydig-stromal cell tumor was confirmed. Our report is a reminder that although idiopathic hirsutism and other benign androgen excess disorder like Polycystic Ovarian Syndrome (PCOs) are common, ovarian mass should be considered in differential diagnosis.
    Medical journal of the Islamic Republic of Iran 11/2012; 26(4):185-188.
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    • "In an immunohistochemical study on 215 ovarian tumors, caleritinin was found to be a sensitive marker for sex cord tumors; however, it was a less specific marker than inhibin in differentiating these stromal tumors from fibrous neoplasms [10]. Most of the steroid cell tumors are positive for calretinin and inhibin [9,11,12]. However, some authors have also reported calretinin-negative steroid cell tumors [13,14]. "
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    ABSTRACT: Steroid cell tumors are microscopically characterized by abundant eosinophilic or vacuolated cytoplasm that is often positive for fat stains. These tumors could be of ovarian or ectopic adrenal origin. We present a rare case of a steroid cell tumor arising from the pelvic mesentery. A 31-year-old Asian woman was undergoing treatment for infertility and virilizing symptoms. She underwent laparoscopy followed by laprotomy for a suspected ovarian cyst/pelvic mass. During the laprotomy, a mass of 9 × 7 cm was detected in the pelvic mesentery.Microscopically, the tumor showed large cells arranged predominantly in sheets with abundant granular cytoplasm and large vesicular nuclei with prominent nucleoli. The tumor was seen infiltrating surrounding adipose tissue. Immunohistochemically, the tumor cells showed strong positivity for kertain, inhibin, vimentine, melan-A, neuron-specific enolase, chromogranin, and S-100 protein and focal positivity to epithelial membrane antigen. An MIB1 index showed 4% nuclear positivity. The tumor cells were negative for calretinin, desmin, and muscle actin. Considering the clinical findings, histomorphology, and immunohistochemistry, we made the diagnosis of extraovarian and extra-adrenal steroid cell tumor (not otherwise specified) of the pelvic mesentery. We report an extremely rare case of an extraovarian and extra-adrenal steroid cell tumor of the pelvic mesentery. The tumor was a cause of virilizing symptoms and infertility in the patient. Surgical removal of the tumor reverted the symptoms of virilization, and the patient subsequently conceived. Steroid cell tumors should be considered in differential diagnosis among women presenting with infertility and symptoms of virilization.
    Journal of Medical Case Reports 10/2011; 5:517. DOI:10.1186/1752-1947-5-517
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