In vitro Studies of Enzymatic Activities in Human Adrenal Tumors

Degenhart, H. J.; Drop, S. L. S.; Hoogerbrugge, Jos W.; Seters, A. P. van; Vries, Hd

doi:10.1159/000179488

Cited by 15 publications

(8 citation statements)

References 6 publications

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“…This is a consequence of multiple enzyme defi ciencies in tumor tissues. The tumor cells are capable of synthesizing a higher proportion of steroid hormone pre cursors [9][10][11][12],…”

Section: Introductionmentioning

confidence: 99%

Heterogeneity of Urinary Steroid Profiles in Children with Adrenocortical Tumors

Małunowicz¹,

Ginalska-Malinowska²,

Romer

et al. 1995

Horm Res

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The excretory patterns of urinary steroids determined by capillary gas chromatography in 11 children (aged 0.8-16.5 years) with adrenocortical tumors were established. In 8 patients the predominant clinical feature was virilization, in 3 others, Cushing’s syndrome. In 5 patients (3 carcinoma, 2 adenoma) very high excretion of 3β-hydroxy-5-ene steroids was observed. In 2 others (adenomas) only moderately elevated excretion of 11β-hydroxyandrosterone was found. In 1 patient (adenoma) pregnanediol dominated in the steroid profile, accompanied by moderately elevated 3β-hydroxy-5-ene steroids. Out of 3 Cushingoid patients (1 carcinoma, 2 adenomas), 1 presented an atypical urinary steroid pattern for hypercortisolemia, without 5α-reductase and 11β-hydroxysteroid dehydrogenase deficiencies. Neither the urinary steroid pattern nor tumor size alone were reliable indicators of tumor malignancy, as evaluated by a pathological examination and subsequent metastasis-free survival.

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“…This is a consequence of multiple enzyme defi ciencies in tumor tissues. The tumor cells are capable of synthesizing a higher proportion of steroid hormone pre cursors [9][10][11][12],…”

Section: Introductionmentioning

confidence: 99%

Heterogeneity of Urinary Steroid Profiles in Children with Adrenocortical Tumors

Małunowicz¹,

Ginalska-Malinowska²,

Romer

et al. 1995

Horm Res

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“…To our knowledge virilizing adrenal tumours mimicking congenital adrenal hyperplasia with P450c21-and P450cll deficiency have been described only in adults [12,13,15,21,26]. Such tumours, however, may occur in children and adults treated for congenital adrenal hyperplasia [7,8,17,18]. Abnormal steroid findings suggestive of congenital adrenal hyperplasia may also be produced by Leydig cell tumours as described by Solish et al [19] in a boy with sexual precocity.…”

Section: Discussionmentioning

confidence: 82%

“…In adrenal tumours of patients with congenital adrenal hyperplasia due to P450c21 (21-hydroxylase)-or P450cl 1 (11 ~-hydroxylase) deficiency, the enzyme deficiency may also be present [7,8,17,18]. Some adult patients with adrenocortical neoplasms, however, have been described with evidence of P450c21-or P450c 11 deficiency limited to the tumour tissue [12,13,15,21,26].…”

Section: Introductionmentioning

confidence: 99%

Virilizing adrenal tumour mimicking congenital adrenal hyperplasia with P450c11 (11β-hydroxylase) deficiency

Werder

Voutilainen

Zachmann³

1994

Eur J Pediatr

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In a girl aged 5 years with a virilizing adrenal adenoma the urinary and plasma steroid findings suggested the diagnosis of congenital adrenal hyperplasia due to P450c11 (11 beta-hydroxylase) deficiency. After removal of the tumour clinical signs receded and the hormonal values normalized. RNA analysis of the tumour tissue revealed low amounts of P450c11 mRNA which indicates that P450c11 deficiency of the adenoma caused the steroid abnormalities in this girl.

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“…11/8-hydroxylase activity was also in the range of normal controls in our previous report (Naganuma et al 1988). Other investigators documented the increased activity of various enzymes in Cushing's adenomas (Degenhart et al 1982;D'Agata et al 1987). Such increased enzyme activity was not observed in our study.…”

Section: Discussionmentioning

confidence: 97%

“…However, only a few reports (Degenhart et al 1982;D'Agata et al 1987) about steroidogenic enzyme activity in adenomas have been published. Therefore it is still difficult to determine its enzymic function from the histological investigation in each case.…”

mentioning

confidence: 99%

Steroid 21-hydroxylase and 17.ALPHA.-hydroxylase in microsomal fraction of functioning adrenocortical tumors.

Naganuma

Sasano

1989

Tohoku J. Exp. Med.

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In order to survey the enzymic activities of steroidogenesis in functioning adrenocortical tumors, we investigated the activities of steroid 21-hydroxylase and 17a-hydroxylase in microsomal fractions of 12 surgically resected adrenocortical tumors associated with Cushing's syndrome (5 adenomas and one carcinoma), primary aldosteronism (5 adenomas) and adrenogenital syndrome (AGS) (one carcinoma), and one adrenocortical hyperplasia resulting from Cushing's disease. Seven adrenal cortices from the patients with mammary carcinoma, renal cell carcinoma or pheochromocytoma were used for normal control. In normal controls 21-hydroxylase activities with progesterone as a substrate were 1.61+0.25 nmole/min/mg protein and those with 17a-hxdroxyprogesterone were 5.22 + 1.06 nmole/min/mg protein. The activity of 21-hydroxylase was higher in four cases of 5 aldosteronomas than in normal controls. Those activities in Cushing's adenomas were in the range of normal controls in this study. 17a-hydroxylase activities were much variable from case to case even though in normal controls (4.50+2.40 nmole/min/mg protein), and in most cases of adenomas 17a-hydroxylase activities were in the range of normal controls. Activities of both hydroxylase in carcinomas were lower than in normal controls. The present paper showed the abnormal steroidogenic enzyme activities in aldosteronomas and adrenocortical carcinomas.

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In vitro Studies of Enzymatic Activities in Human Adrenal Tumors

Cited by 15 publications

References 6 publications

Heterogeneity of Urinary Steroid Profiles in Children with Adrenocortical Tumors

Heterogeneity of Urinary Steroid Profiles in Children with Adrenocortical Tumors

Virilizing adrenal tumour mimicking congenital adrenal hyperplasia with P450c11 (11β-hydroxylase) deficiency

Steroid 21-hydroxylase and 17.ALPHA.-hydroxylase in microsomal fraction of functioning adrenocortical tumors.

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