Feminizing Adrenocortical Carcinoma with Selective Suppression of Follicle-Stimulating Hormone Secretion and Disorganized Steroidogenesis: A Case Report and Literature Review

Saito, Takatoshi; Tojo, Katsuyoshi; Furuta, Nozomu; Ono, Koji; Sasano, Hironobu; Utsunomiya, Kazunori

doi:10.2169/internalmedicine.50.4968

Cited by 6 publications

(6 citation statements)

References 26 publications

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“…Feminizing adrenal tumors (FAT) are defined as adrenal neoplasms with estrogens (estrone and estradiol) overproduction with or without other adrenal hormones. [ 5 7 10 ] These exceedingly rare tumors are deemed to have a dire prognosis. [ 1 ] They are mainly observed in men or in children, and are exceptional in premenopausal and menopausal women.…”

Section: Efinition P Revalence mentioning

confidence: 99%

“…The last condition is very rare and the prognosis is deemed to be the worst. In literature only single cases have been reported in adults[ 1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 19 21 22 23 24 25 25 ] and in pediatric population. [ 19 26 27 28 29 30 31 32 33 34 35 36 37 38 39 40 41 ] The largest published series does not exceed two[ 7 25 ] and three cases.…”

Section: Introductionmentioning

confidence: 99%

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Feminizing adrenocortical tumors: Literature review

Chentli

Bekkaye

Azzoug

2015

Indian J Endocr Metab

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Feminizing adrenal tumors (FAT) are extremely rare tumors prevailing in males. Clinical manifestations are gynecomastia and/or other hypogonadism features in adults. They are rarer in pediatric population and their main manifestation is peripheral sexual precocity.In women genital bleeding, uterus hypertrophy, high blood pressure and/or abdomen mass may be the only manifestations.On the biological point, estrogen overproduction with or without increase in other adrenal hormones are the main abnormalities. Radiological examination usually shows the tumor, describes its limits and its eventual metastases. Adrenal and endocrine origins are confirmed by biochemical assessments and histology, but that one is unable to distinguish between benign and malignant tumors, except if metastases are already present. Immunostaining using anti-aromatase antibodies is the only tool that distinguishes FAT from other adrenocortical tumors. Abdominal surgery is the best and the first line treatment. For large tumors (≥10 cm), an open access is preferred to coeliosurgery, but for the small ones, or when the surgeon is experienced, endoscopic surgery seems to give excellent results. Surgery can be preceded by adrenolytic agents such as ortho paraprime dichloro diphenyl dichloroethane (Mitotane), ketoconazole or by aromatase inhibitors, but till now there is not any controlled study to compare the benefit of different drugs. New anti-estrogens can be used too, but their results need to be confirmed in malignant tumors resistant to classical chemotherapy and to conventional radiotherapy. Targeted therapy can be used too, as in other adrenocortical tumors, but the results need to be confirmed.

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Section: Efinition P Revalence mentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Feminizing adrenocortical tumors: Literature review

Chentli

Bekkaye

Azzoug

2015

Indian J Endocr Metab

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“…[15] 21 OHase, 3 beta- ol-deshydrogenase, and 11beta OHase-acquired deficits are responsible of an increase in some precursors such as androstenedione, 17OHP, and DHEA S that are converted to estrogens via an increase in aromatase activity. [16] As a consequence of estradiol excess, there is also an inhibition of pituitary function[17] especially for FSH. [6716] Gonadotropins inhibition may also be increased by inhibin production by the tumor.…”

Section: Discussionmentioning

confidence: 99%

Feminizing adrenal tumors: Our experience about three cases

Chentli

Bekkaye

Yahiaoui

et al. 2013

Indian J Endocr Metab

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Feminizing adrenal tumors (FATs) are very rare as they account for less than 2% of all the adrenal neoplasms. Their prognosis is deemed to be very poor. We aimed to present a mono centre (adult and pediatric) experience over a long period of time (January 1980 to Jun 2012). During the study period, we observed only three cases in men aged 22 (2 cases) and 45 (1 case). They all consulted for a painful gynecomastia, decreased libido and impotency. Estradiol was high in two cases at presentation, and after a relapsing tumor in the third one. All had big adrenal tumors (5.9, 6, and 17 cm), and a mixed secretion composed by high estradiol and cortisol. The pathological study argued for malignancy in two cases. But, only one had diffuse metastasis and died 4 years after diagnosis; the others diagnosed one and three years ago are still alive without any metastasis or relapsing.

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“…Previous studies have shown aromatase overexpression at the mRNA and protein levels in estrogen-secreting ACCs. [3][4][5][6] This may contribute to ectopic estrogen production in ACCs. However, performing detailed analyses of the steroidogenesis pathway by immunohistochemical analysis is difficult due to its low sensitivity and tumor heterogeneity.…”

Section: Introductionmentioning

confidence: 99%

A case of estrogen‐secreting adrenocortical carcinoma: Comprehensive immunohistochemical analysis of disorganized steroid genesis

Yokoyama¹,

Kijima²,

Takada‐Owada

et al. 2021

IJU Case Reports

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Introduction: Adrenocortical carcinoma rarely secretes estrogens, and little is known regarding the mechanism of intra-tumor estrogen production. We report an estrogensecreting adrenocortical carcinoma in a postmenopausal woman, where comprehensive immunohistochemical analyses of the resected tumor revealed disorganized steroidogenesis. Case presentation: A 68-year-old woman presented with postmenopausal vaginal bleeding and was found to have a left adrenal tumor. Serum estradiol and testosterone were elevated but they normalized after resection of the tumor, suggestive of adrenocortical carcinoma with disorganized steroidogenesis. Immunohistochemical analyses revealed that the tumor expressed aromatase which converts androgens into estrogens. Furthermore, the tumor lacked 17bHSD2, which converts estradiol to estrone, suggesting that estradiol accumulated as the final product of the tumor's steroidogenic pathway. Conclusion:The capability of adrenocortical carcinoma to produce estrogen can be demonstrated by comprehensive immunohistochemical analyses of steroidogenic enzymes, such as those reported here.

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Feminizing Adrenocortical Carcinoma with Selective Suppression of Follicle-Stimulating Hormone Secretion and Disorganized Steroidogenesis: A Case Report and Literature Review

Cited by 6 publications

References 26 publications

Feminizing adrenocortical tumors: Literature review

Feminizing adrenocortical tumors: Literature review

Feminizing adrenal tumors: Our experience about three cases

A case of estrogen‐secreting adrenocortical carcinoma: Comprehensive immunohistochemical analysis of disorganized steroid genesis

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