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      Sertoliform endometrioid carcinoma of the right ovary

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          Abstract

          Summary

          Endometrioid carcinomas of the ovary are a subtype of epithelial ovarian tumors, with sertoliform endometrioid carcinomas being a rare variant. We report a case of a previously healthy premenopausal woman presenting with androgenic symptoms in the form of hirsutism and male pattern alopecia. On further testing, she was found to have high levels of luteinizing hormone and total testosterone levels, and imaging revealed a large pelvic abdominal mass in the right ovary. She underwent total hysterectomy with bilateral salpingo-oophorectomy. Microscopy and histopathology confirmed the diagnosis of sertoliform endometrioid carcinoma. Her symptoms improved significantly on follow-up. Androgenic tumors might not be common in premenopausal women; however, it is important to maintain a high level of suspicion in patients presenting with virilizing symptoms especially of rapid progression.

          Learning points
          • Our 47-year-old patient presented with virilizing symptoms that were rapidly progressing, which raises the suspicion of an underlying androgen secreting neoplasm.

          • Sertoliform endometrioid carcinoma (SEC) is an extremely rare variant of endometrioid carcinomas and tend to present at an earlier stage as compared to most endometrioid carcinomas of the ovary.

          • Recognition of SEC in virilizing patients is important as it is a well-differentiated, low-grade malignancy with a good prognosis when confined to the ovary.

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          Most cited references12

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          O-RADS US Risk Stratification and Management System: A Consensus Guideline from the ACR Ovarian-Adnexal Reporting and Data System Committee

          The Ovarian-Adnexal Reporting and Data System (O-RADS) US risk stratification and management system is designed to provide consistent interpretations, to decrease or eliminate ambiguity in US reports resulting in a higher probability of accuracy in assigning risk of malignancy to ovarian and other adnexal masses, and to provide a management recommendation for each risk category. It was developed by an international multidisciplinary committee sponsored by the American College of Radiology and applies the standardized reporting tool for US based on the 2018 published lexicon of the O-RADS US working group. For risk stratification, the O-RADS US system recommends six categories (O-RADS 0-5), incorporating the range of normal to high risk of malignancy. This unique system represents a collaboration between the pattern-based approach commonly used in North America and the widely used, European-based, algorithmic-style International Ovarian Tumor Analysis (IOTA) Assessment of Different Neoplasias in the Adnexa model system, a risk prediction model that has undergone successful prospective and external validation. The pattern approach relies on a subgroup of the most predictive descriptors in the lexicon based on a retrospective review of evidence prospectively obtained in the IOTA phase 1-3 prospective studies and other supporting studies that assist in differentiating management schemes in a variety of almost certainly benign lesions. With O-RADS US working group consensus, guidelines for management in the different risk categories are proposed. Both systems have been stratified to reach the same risk categories and management strategies regardless of which is initially used. At this time, O-RADS US is the only lexicon and classification system that encompasses all risk categories with their associated management schemes.
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            Epidemiology, diagnosis and management of hirsutism: a consensus statement by the Androgen Excess and Polycystic Ovary Syndrome Society.

            Hirsutism, defined by the presence of excessive terminal hair in androgen-sensitive areas of the female body, is one of the most common disorders in women during reproductive age. We conducted a systematic review and critical assessment of the available evidence pertaining to the epidemiology, pathophysiology, diagnosis and management of hirsutism. The prevalence of hirsutism is ~10% in most populations, with the important exception of Far-East Asian women who present hirsutism less frequently. Although usually caused by relatively benign functional conditions, with the polycystic ovary syndrome leading the list of the most frequent etiologies, hirsutism may be the presenting symptom of a life-threatening tumor requiring immediate intervention. Following evidence-based diagnostic and treatment strategies that address not only the amelioration of hirsutism but also the treatment of the underlying etiology is essential for the proper management of affected women, especially considering that hirsutism is, in most cases, a chronic disorder needing long-term follow-up. Accordingly, we provide evidence-based guidelines for the etiological diagnosis and for the management of this frequent medical complaint.
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              Ovarian Sertoli-Leydig cell tumors. A clinicopathological analysis of 207 cases.

              The clinical and pathological features of 207 ovarian Sertoli-Leydig cell tumors from our consultation and hospital files were reviewed. The patients ranged in age from 2 to 75 (average 25) years. Seventy-five percent of them were 30 years of age or younger and less than 10% were over 50 years of age. One-third of the patients presented because of unequivocal evidence of androgen excess, and an additional 10% had a history suggesting androgen excess; most of the remaining patients complained of abdominal swelling or pain. At operation, 97.5% of the tumors were Stage I, 1.5% were Stage II, and 1% were Stage III. Both ovaries were involved in 1.5% of the cases. The tumors ranged from microscopic to 51 cm in diameter (average 13.5 cm); 15% of them were ruptured. Thirty-eight percent of the tumors were solid, 58% were solid and cystic, and 4% were cystic. The solid tissue was typically lobulated and yellow. On microscopic examination, 11% of the tumors were well differentiated, 54% were of intermediate differentiation, 13% were poorly differentiated, and 22% contained heterologous elements according to the criteria of the World Health Organization; a prominent retiform pattern was present in 15% of them. Follow-up was obtained for 164 patients. The tumor was clinically malignant in 18% of them. The prognosis correlated most meaningfully with the stage and degree of differentiation of the tumor. The high-stage tumors were all clinically malignant. All the well-differentiated tumors were benign, but 11% of those of intermediate differentiation, 59% of the poorly differentiated tumors, and 19% of those with heterologous elements were malignant. In a few cases radiation therapy, chemotherapy, or a combination of the two, in addition to surgical excision, was of benefit in the management of the malignant tumors.

                Author and article information

                Journal
                Endocrinol Diabetes Metab Case Rep
                Endocrinol Diabetes Metab Case Rep
                EDM
                Endocrinology, Diabetes & Metabolism Case Reports
                Bioscientifica Ltd (Bristol )
                2052-0573
                06 September 2023
                01 July 2023
                : 2023
                : 3
                : 23-0046
                Affiliations
                [1 ]Kuwait Board of Internal Medicine , Kuwait City, Safat, Kuwait
                [2 ]Department of Internal Medicine , Adan Hospital, Kuwait City, Kuwait
                Author notes
                Correspondence should be addressed to M Alali; Email: mjalali@ 123456moh.gov.kw
                Author information
                http://orcid.org/0009-0002-8363-297X
                http://orcid.org/0000-0002-8609-2190
                Article
                EDM230046
                10.1530/EDM-23-0046
                10563599
                37767716
                7132af52-f2b5-4f57-8712-b79d7997b2a8
                © the author(s)

                This work is licensed under a Creative Commons Attribution 4.0 International License.

                History
                : 18 April 2023
                : 06 September 2023
                Categories
                Adult
                Female
                Other
                Kuwait
                Ovaries
                Developmental Endocrinology
                Endocrine-Related Cancer
                Gynaecological Endocrinology
                Endometrial Cancer
                Unique/Unexpected Symptoms or Presentations of a Disease
                Unique/Unexpected Symptoms or Presentations of a Disease

                adult,female,other,kuwait,ovaries,developmental endocrinology,endocrine-related cancer,gynaecological endocrinology,endometrial cancer,unique/unexpected symptoms or presentations of a disease,september,2023

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