Cortisol Production by Testicular Tumors in Adrenogenital Syndrome

Fore, William W.

doi:10.1001/archinte.1972.03650010047009

Cited by 27 publications

(3 citation statements)

References 26 publications

(1 reference statement)

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“…There is controversy in the literature as to whether these tumors in congenital adrenal hyperplasia are of adrenal or of testicular origin (52)(53)(54)(55)(56). However, prolonged ACTH stimulation appears to lead to the development of these tumors (57), and whatever the cell of origin, they have the capacity to make cortisol or other steroid precursors (55)(56)(57). Although testicular nodule tissue from our patient was not incubated, these nodules appear stimulated by ACTH, as evidenced by a decrease in size, at least in the right testis, with increased glucocorticoid therapy.…”

Section: Resultsmentioning

confidence: 76%

Persistent testicular delta5-isomerase-3beta-hydroxysteroid dehydrogenase (delta5-3beta-HSD) deficiency in the delta5-3beta-HSD form of congenital adrenal hyperplasia.

Schneider¹,

Genel²,

Bongiovanni³

et al. 1975

J. Clin. Invest.

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A B S T R A C T A partial testicular defect in testosterone secretion has been documented in a pubertal male with a congenital adrenal hyperplasia due to hereditary deficiency of the A5-isomerase-3fi-hydroxysteroid dehydrogenase enzyme complex (AY-3j-HSD). Diagnosis of the enzymatic defect is based on the clinical picture of ambiguous genitalia and salt-losing crisis in infancy, together with high urinary A5-pregnenetriol and plasma dehydroepiandrosterone when the patient was taken off replacement corticoid treatment. No hormonal response to ACTH or salt deprivation was demonstrable. In addition, in vivo studies revealed a partial enzymatic defect in the testis. Although plasma testosterone was low-normal (250 ng/100 ml), plasma A5-androstenediol was markedly elevated and rose to a greater extent than testosterone after human chorionic gonadotropin administration. In vitro testicular incubation studies suggested a testicular A5-3%-HSD enzyme defect with less A4 products formed from A5 precursors than in a control testis. Histochemical studies of the testis were also consistent with this defect. Testicular biopsy revealed spermatoAn abstract of a portion of this work has appeared in Pediatr. Res.

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Section: Resultsmentioning

confidence: 76%

Persistent testicular delta5-isomerase-3beta-hydroxysteroid dehydrogenase (delta5-3beta-HSD) deficiency in the delta5-3beta-HSD form of congenital adrenal hyperplasia.

Schneider¹,

Genel²,

Bongiovanni³

et al. 1975

J. Clin. Invest.

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“…Such an unexpected expression of the steroidogenic P450 was also seen in reports of some human diseases. Testicular and ovarian tumours have been reported to synthesize and secrete cortisol by the activities of P450c21 and P45011b ectopically expressed in the tumours (Fore et al 1971). In addition, adrenal tumours were also reported to synthesize oestrogen as a result of the ectopic expression of P450arom (McKenna et al 1990).…”

Section: From the Adreno-genital Primordium To Mature Steroidogenic Tmentioning

confidence: 99%

The ontogenesis of the steroidogenic tissues

Morohashi

1997

Genes to Cells

104

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Based on the common function of steroid hormone-producing tissues and homologous regulation by the hypothalamo-pituitary axis, the adrenal cortex and the gonads have been suggested to have an intimate ontogenic relationship. This assumption is also supported by the findings of common transcription factors implicated in the differentiation of both types of tissue and further supported by concomitant defects in such tissues due to the disruption of a single gene. Similarly, simultaneous anomalies in those tissues are also observed in some diseases caused by mutations of the genes encoding those transcription factors. A recent immunohistochemical study with one of the transcription factors, Ad4BP/SF-1, definitely demonstrated the presence of a particular cell population designated the 'adreno-genital primordium (AGP)' which gives rise to both the adrenal cortex and the gonads. In the process of differentiation from the AGP to the mature adrenal cortex and the gonads of the two sexes, several interesting issues can be raised as to the next targets for the study. To address these issues it is important to elucidate the upstream regulatory mechanisms and downstream target genes of such transcription factors as WT1, SRY, SOX9 and DAX1, in addition to Ad4BP/SF-1, all of which are implicated in steroidogenic tissue differentiation.

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“…An enzymatic defect of the tissue has been found in testicular tumours in patients with congenital adrenal hyperplasia [6,14], The increases of dihydrotestosterone and oestradiol levels are due to peripheral conversion of testosterone, whereas the increase in prolactin is due to pressure of the hypophyseal stalk from the adenoma or to a direct effect of oestradiol on the lactotroph cells of the anterior hypophyseal lobe [15], Our patient had partial hypopituitarism, since FSH, LH, and GH did not respond normally, whereas PRL and TSH did in the combined insulin-TRH-LH-RH-metoclopramide test. The absent response of gonadotrophins is permanent and is due eith er to a destructive or to a compressive effect of the macroadenoma on the normal residual pituitary gland.…”

Section: Discussionmentioning

confidence: 99%

Paratesticular Tumours in a Patient with Nelson’s Syndrome

Ntalles¹,

Kostoglou-Athanassiou²,

Georgiou³

et al. 1996

Horm Res

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An extremely rare case of a 39-year-old man with Nelson’s syndrome and paratesticular tumours is presented. The patient had undergone bilateral adrenalectomy at the age of 23 years for Cushing’s syndrome. Later he developed skin and mucosal hyperpigmentation, Nelson’s syndrome was diagnosed, and he underwent transfrontal hypophysectomy. At age 29, the presence of paratesticular tumours was noted which were excised together with the left testis. The preoperative levels of testosterone, produced by the paratesticular tumours, were extremely high; they fell after surgery.

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Cortisol Production by Testicular Tumors in Adrenogenital Syndrome

Cited by 27 publications

References 26 publications

Persistent testicular delta5-isomerase-3beta-hydroxysteroid dehydrogenase (delta5-3beta-HSD) deficiency in the delta5-3beta-HSD form of congenital adrenal hyperplasia.

Persistent testicular delta5-isomerase-3beta-hydroxysteroid dehydrogenase (delta5-3beta-HSD) deficiency in the delta5-3beta-HSD form of congenital adrenal hyperplasia.

The ontogenesis of the steroidogenic tissues

Paratesticular Tumours in a Patient with Nelson’s Syndrome

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Cortisol Production by Testicular Tumors in Adrenogenital Syndrome

Cited by 27 publications

References 26 publications

Persistent testicular delta5-isomerase-3beta-hydroxysteroid dehydrogenase (delta5-3beta-HSD) deficiency in the delta5-3beta-HSD form of congenital adrenal hyperplasia.

Persistent testicular delta5-isomerase-3beta-hydroxysteroid dehydrogenase (delta5-3beta-HSD) deficiency in the delta5-3beta-HSD form of congenital adrenal hyperplasia.

The ontogenesis of the steroidogenic tissues

Paratesticular Tumours in a Patient with Nelson&rsquo;s Syndrome

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Paratesticular Tumours in a Patient with Nelson’s Syndrome