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      A clinically functioning gonadotroph adenoma presenting with abdominal pain, ovarian hyperstimulation and fibromatosis

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          Summary

          Pituitary adenomas can be classified as functioning or non-functioning adenomas. Approximately 64% of clinically non-functioning pituitary adenomas are found to be gonadotroph adenomas on immunohistochemistry. There are reported cases of gonadotroph adenomas causing clinical symptoms, but this is unusual. We present the case of a 36-year-old female with abdominal pain. Multiple large ovarian cysts were identified on ultrasound requiring bilateral cystectomy. Despite this, the cysts recurred resulting in further abdominal pain, ovarian torsion and right oophorectomy and salpingectomy. On her 3rd admission with abdominal pain, she was found to have a rectus sheath mass which was resected and histologically confirmed to be fibromatosis. Endocrine investigations revealed elevated oestradiol, follicle-stimulating hormone (FSH) at the upper limit of the normal range and a suppressed luteinising hormone (LH). Prolactin was mildly elevated. A diagnosis of an FSH-secreting pituitary adenoma was considered and a pituitary MRI revealed a 1.5 cm macroadenoma. She underwent transphenoidal surgery which led to resolution of her symptoms and normalisation of her biochemistry. Subsequent pelvic ultrasound showed normal ovarian follicular development. Clinically functioning gonadotroph adenomas are rare, but should be considered in women presenting with menstrual irregularities, large or recurrent ovarian cysts, ovarian hyperstimulation syndrome and fibromatosis. Transphenoidal surgery is the first-line treatment with the aim of achieving complete remission.

          Learning points:
          • Pituitary gonadotroph adenomas are usually clinically non-functioning, but in rare cases can cause clinical symptoms.

          • A diagnosis of a functioning gonadotroph adenoma should be considered in women presenting with un-explained ovarian hyperstimulation and/or fibromatosis.

          • In women with functioning gonadotroph adenomas, the main biochemical finding is elevated oestradiol levels. Serum FSH levels can be normal or mildly elevated. Serum LH levels are usually suppressed.

          • Transphenoidal surgery is the first-line treatment for patients with functioning gonadotroph adenomas, with the aim of achieving complete remission.

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          Most cited references9

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          The prevalence of pituitary adenomas: a systematic review.

          Pituitary adenomas display an array of hormonal and proliferative activity. Once primarily classified according to size (microadenomas, or = 1 cm), these tumors are now further classified according to immunohistochemistry and functional status. With these additional classifications in mind, the goals of the current study were to determine the prevalence of pituitary adenomas and to explore the clinical relevance of the findings. The authors conducted a metaanalysis of all existing English-language articles in MEDLINE. They used the search string (pituitary adenoma or pituitary tumor) and prevalence and selected relevant autopsy and imaging evaluation studies for inclusion. The authors found an overall estimated prevalence of pituitary adenomas of 16.7% (14.4% in autopsy studies and 22.5% in radiologic studies). Given the high frequency of pituitary adenomas and their potential for causing clinical pathologies, the findings of the current study suggest that early diagnosis and treatment of pituitary adenomas should have far-reaching benefits.
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            The enigma of desmoid tumors.

            To analyze patients with recurrent extremity desmoids, in whom the surgical therapeutic option was either major amputation or observation. The biology and natural history of desmoid tumors are an enigma. These tumors invade surrounding structures and recur locally but do not metastasize. The morbidity of treating these tumors in the context of their relatively benign biology is uncertain. Between July 1982 and June 1998, the authors treated and prospectively followed 206 patients with extremity desmoid tumors. All patients underwent standardized surgical resection, the surgical goal always being complete resection with negative margins. When tumors recurred, they were evaluated for reresection. Amputation was considered when resection was not possible because of neurovascular or major bone involvement, or in the presence of a functionless, painful extremity. During this period, 22 patients had disease that was not resectable without amputation. This was out of a total of 115 patients with primary disease and 91 patients with recurrent disease. All recurrences were local; in no patient did metastasis develop. In this group of 22 patients with unresectable disease, 7 underwent amputation and 15 did not. These 15 patients were followed, alive with disease, having no surgical resection. Four patients received systemic treatment with tamoxifen and nonsteroidal antiinflammatories, three received systemic cytotoxic chemotherapy, and two received both tamoxifen and chemotherapy. Six patients received no systemic treatment. The range of follow-up was 25 to 92 months. In all patients, there was no or insignificant tumor progression; in three patients who underwent observation alone, there was some regression of tumor. During follow-up, no patient has required subsequent amputation, and no patient has died from disease. In desmoid tumors, aggressive attempts at achieving negative resection margins may result in unnecessary morbidity. Function- and structure-preserving procedures should be the primary goal. In select patients, whose only option is amputation, it may be prudent to observe them with their limb and tumor intact.
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              Clinical review: Functioning gonadotroph adenomas.

              Functioning gonadotroph adenomas (FGAs) are pituitary tumors secreting biologically active gonadotropins. The published literature includes only small case series or individual case reports. This review summarizes the published data on this rare entity and, based on them, suggests guidance on the follow-up of these patients.
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                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
                11 December 2018
                2018
                : 2018
                : 18-0123
                Affiliations
                [1]Southmead Hospital , North Bristol NHS Trust, Westbury-on-Trym, Bristol, UK
                Author notes
                Correspondence should be addressed to C Broughton; Email: chloebroughton@ 123456nhs.net
                Article
                EDM180123
                10.1530/EDM-18-0123
                6300858
                30532999
                99e94aa9-a063-49f7-b2ea-710fc6946cfd
                © 2018 The authors

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

                History
                : 13 November 2018
                : 22 November 2018
                Categories
                New Disease or Syndrome: Presentations/Diagnosis/Management

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