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      Steroid cell tumor: a rare cause of hirsutism in a female

      case-report

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          Summary

          Ovarian steroid cell tumors are very rare functioning sex-cord stromal tumors. They comprise <0.1% of all ovarian tumors. Previously designated as lipoid cell tumors, one-third of these tumors are considered malignant with the mean age of presentation at around 40 years. We present a case of a 28-year-old female with 2-year history of hirsutism, virilization, and amenorrhea. She was diagnosed with left ovarian tumor, for which she underwent left salpingo-oophorectomy. Histopathology revealed not otherwise specified subtype of steroid cell tumors. The patient resumed menses 2 months after the features of masculinization regressed. Within 1 year of surgery, the patient successfully conceived a full-term baby without any complications. In a young female, the neoplastic etiology of a rapid virilization or menses changing should always be kept in mind. Though commonly observed in adult females, steroid cell tumors have very good surgical outcomes if age at presentation is less and tumor is unilateral, and there are no evidences of bilateral malignancy. Bilateral salpingo-oophorectomy is not required.

          Learning points

          • In a case of severe rapid hirsutism and virilization with serum testosterone level more than 200 ng/dl or more than threefold of the normal range, neoplastic conditions should always be suspected.

          • Steroid cell tumor in young women without evidence of malignancy on histopathology has excellent surgical outcomes.

          • Unilateral salpingo-oophorectomy is the surgery of choice.

          • As the frequency of bilateralism is only 6%, prophylactic unaffected side oophorectomy need not be done.

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          Most cited references2

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          Ovarian steroid cell tumors (not otherwise specified). A clinicopathological analysis of 63 cases.

          The clinical and pathological features of 63 steroid cell tumors, not otherwise specified, were reviewed. The patients ranged in age from 2 1/2 to 80 years. The most common initial manifestation was virilization (41%); four patients had estrogenic manifestations, and four had hypercortisolemia with Cushing's syndrome. The tumors, 6% of which were bilateral, ranged from 1.2 to 45 cm in greatest dimension. Microscopic examination revealed two types of cells, which had overlapping features: those with abundant eosinophilic cytoplasm and those with vacuolated cytoplasm. Fat stains were positive in 75% of the 16 cases in which they were performed. Follow-up data ranging from 1 to 19 years (average 5.2 years) in duration were available for 50 patients. In 24 cases, the tumor was designated probably benign (no evidence of spread beyond the ovary within 3 or more years postoperatively). In 18 patients, the tumor was clinically malignant. The best pathological correlates of malignant behavior were: the presence of two or more mitotic figures per 10 high power fields (92% malignant); necrosis (86% malignant); a diameter of 7 cm or greater (78% malignant); hemorrhage (77% malignant); and grade 2 or 3 nuclear atypia (64% malignant).
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            Steroid Cell Tumor of the Ovary in an Adolescent: A Rare Case Report

            Steroid cell tumors (SCTs) of the ovary are a rare subgroup of sex cord tumors, account for less than 0.1% of all ovarian tumors, and also will present at any age. These tumors can produce steroids, especially testosterone, and may give symptoms like hirsutism, hair loss, amenorrhea, or oligomenorrhea. For the evaluation of androgen excess, testosterone and dehydroepiandrosterone sulfate (DHEA-S) are the first laboratory tests to be measured. A pelvic ultrasound and a magnetic resonance imaging are useful radiologic imaging techniques. Although steroid cell tumors are generally benign, there is a risk of malignant transformation and clinical malignant formation. Surgery is the most important and hallmark treatment.

              Author and article information

              Journal
              Endocrinol Diabetes Metab Case Rep
              Endocrinol Diabetes Metab Case Rep
              edm
              EDM Case Reports
              Endocrinology, Diabetes & Metabolism Case Reports
              Bioscientifica Ltd (Bristol )
              2052-0573
              16 September 2013
              2013
              : 2013
              : 130030
              Affiliations
              [1]Department of Endocrinology Institute of Medical Sciences, Benaras Hindu University Varanasi, 221005India
              Author notes
              Correspondence should be addressed to S Sharma Email: dr.sharma.shruti@ 123456gmail.com
              Article
              EDM130030
              10.1530/EDM-13-0030
              3922368
              24616767
              aa9e079a-a363-45a2-849c-68952f2874cc
              © 2013 The authors

              This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivs 3.0 Unported License.

              History
              : 18 August 2013
              : 23 August 2013
              Categories
              New Disease or Syndrome: Presentations/Diagnosis/Management

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