Studies of the Diurnal Pattern of Plasma Corticosteroids and Gonadotropins in Two Cases of Feminizing Adrenal Carcinoma: Measurements of Estrogen and Corticosteroid Production

Boyar, Robert M.; Nogeire, Christopher; Fukushima, David K.; Hellman, Louis M.; Fishman, Jack

doi:10.1210/jcem-44-1-39

Cited by 11 publications

(5 citation statements)

References 23 publications

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“…As the patient had highly elevated E2 levels, we examined aromatase activity, aromatase protein, and tumor-specific aromatase gene promoter usage. To our knowledge, the patient was the youngest reported with an estrogen producing adrenal tumor [20][21][22][23][24][25][26][27][28][29][30][31][32][33][34][35], and constituted the second report describing the preferential usage of alternative exons 1 of aromatase gene in adrenal tumors (Table 3).…”

Section: Discussionmentioning

confidence: 99%

Estrogen Secreting Adrenal Adenocarcinoma in an 18-Month-old Boy. Aromatase Activity, Protein Expression, mRNA and Utilization of Gonadal Type Promoter.

et al. 2000

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Abstract.We examined clinical, endocrinological and molecular biological aspects of an estrogen-secreting adrenal carcinoma in an 18-month-old male to clarify the pathogenesis of this condition.An 18-month-old boy was referred for evaluation of progressive bilateral gynecomastia and appearance of pubic hair. The patient had elevated plasma estradiol (349 pg/ml) and testosterone (260 ng/dl) levels that completely suppressed FSH and LH levels, and was subsequently diagnosed with an adrenal tumor on the right side. After removal of a 300-g adenocarcinoma, gynecomastia regressed and essentially normal hormone levels were restored.Aromatase activity in the tumor tissue determined by the 3H-water method was 71.0-104.4 pmol/min/mg protein.High levels of aromatase protein and mRNA in the tumor tissue were also demonstrated, while neither aromatase activity nor protein was detected in normal adrenal glands. To investigate the regulation of aromatase expression in the adrenal carcinoma, we examined the usage of alternate promoters responsible for aromatase gene transcription.In the present case, the amounts of aromatase mRNA utilizing gonadal types of exon lc (I.3) and id (II) were significantly higher than those that using other exon is. This result suggested that the utilization of a gonadal-type exon 1 might be involved in the overproduction of aromatase in estrogen-secreting adrenal carcinoma.

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Section: Discussionmentioning

confidence: 99%

Estrogen Secreting Adrenal Adenocarcinoma in an 18-Month-old Boy. Aromatase Activity, Protein Expression, mRNA and Utilization of Gonadal Type Promoter.

et al. 2000

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“…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. [ 10 ] In our department we recorded five cases but only four have been published.…”

Section: Introductionmentioning

confidence: 99%

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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“…In oestrogen producing adrenal tumours, an elevation of urinary hCG levels was previously reported by several groups as well as non-suppressed serum pituitary gonadotrophin levels Rose et al, 1968;Boyer et al, 1977). In addition, increased serum levels and the post-operative normalization of pituitary gonadotrophins were reported in some patients with oestrogen producing adrenal tumours Rose et al, 1969).…”

mentioning

confidence: 74%

“…In addition, increased serum levels and the post-operative normalization of pituitary gonadotrophins were reported in some patients with oestrogen producing adrenal tumours Rose et al, 1969). Boyer et al (1977) suggested the possibility of hCG production and secretion from oestrogen producing adrenocortical neoplasms. In our case, serum hCG, LH and FSH levels were not elevated and immunoreactive hCG and LH were not detectable in the tumour tissue.…”

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confidence: 99%

Oestrogen producing adrenocortical adenoma: clinical, biochemical and immunohistochemical studies

Goto

Murakami²,

Sato³

et al. 1996

Clinical Endocrinology

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Oestrogen producing adrenocortical tumours are extremely rare. We report a 65-year-old woman who presented with abnormal vaginal bleeding, with no significant abnormalities in her uterus or ovaries, who was found to have a right adrenal mass by radiological examination. Excessive secretion of oestrogens from the tumour was demonstrated by adrenal venous sampling. Basal levels of corticosteroids were within normal limits. Adrenalectomy was performed and pathological examination revealed an adrenocortical adenoma measuring 5.5 cm in its greatest dimension, in which both clear and compact tumour cells were observed. Oestrogen levels normalized following the removal of the adrenal mass. Tissue concentrations of oestrone and oestradiol in the tumour were 6.9 (69.5 pmol/g wet tissue weight) and 34.6 (93.6 pmol/g wet tissue weight)-fold greater respectively than those of adjacent non-neoplastic adrenal cortex. Aromatase activity in the tumour tissue determined by the 3H-water method was 118.6 pmol/h/mg protein, equivalent to that of a full-term human placenta. Immunohistochemical analysis of aromatase demonstrated immunoreactivity in the tumour cells, especially in compact cells, but not in adjacent non-neoplastic adrenal cells. This is the first reported case of an oestrogen producing adrenocortical adenoma in which aromatase in the tumour cells was documented.

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Studies of the Diurnal Pattern of Plasma Corticosteroids and Gonadotropins in Two Cases of Feminizing Adrenal Carcinoma: Measurements of Estrogen and Corticosteroid Production

Cited by 11 publications

References 23 publications

Estrogen Secreting Adrenal Adenocarcinoma in an 18-Month-old Boy. Aromatase Activity, Protein Expression, mRNA and Utilization of Gonadal Type Promoter.

Estrogen Secreting Adrenal Adenocarcinoma in an 18-Month-old Boy. Aromatase Activity, Protein Expression, mRNA and Utilization of Gonadal Type Promoter.

Feminizing adrenocortical tumors: Literature review

Oestrogen producing adrenocortical adenoma: clinical, biochemical and immunohistochemical studies

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