Adrenal incidentaloma and patients with homozygous or heterozygous congenital adrenal hyperplasia.

Adrenal tumors are being detected more frequently in consequence of the wider application of increasingly sensitive radiological investigation techniques. According to the working hypothesis that more silent adenomas could develop from hyperplastic tissue areas under increased stimulation of the adrenal cortex, heterozygous and homozygous patients with congenital adrenal hyperplasia (CAH) were studied. A high incidence of adrenal masses, nearly 82% in homozygous and 45% in heterozygous patients, was found. There was no correlation between tumor size and serum 17-hydroxyprogesterone concentrations. These tumors are, therefore, probably silent adenomas. On the basis of these results, CAH should always be ruled out in the case of incidentally detected adrenal masses. Since CAH is a relatively frequent disease, and the adrenal carcinoma belongs to the rarest malignant tumors, a malignant transformation of these tumors seems to be unlikely.

[Adrenogenital syndrome--today a radiologic diagnosis?]. / Adrenogenitales Syndrom--heute eine radiologische Diagnose?

The following case report shows the course of disease of a 37 year old man, who showed the classical symptoms of an untreated congenital adrenal hyperplasia. But only when an adrenal tumor accidentally was detected, the endocrinological tests and the diagnosis of the congenital adrenal hyperplasia were made. By this diagnosis the planned adrenalectomy could be avoided now.

[Silent adrenal gland tumors in patients with adrenogenital syndrome]. / Stumme Nebennierentumoren bei Patienten mit Adrenogenital Syndrom.

Adrenal tumors accidently detected by CT scan are increasingly seen in patients without clinical signs of adrenal diseases. We studied whether enhanced adrenal stimulation is of importance in the development of adrenal tumors. For this purpose 22 patients with adrenogenital syndrome (AGS) were studied by CT scan. One of these patients suffered from C-11 beta-hydroxylase-, one from C-3 beta-hydroxy steroid dehydrogenase-, and 20 from C-21-hydroxylase deficiency. The average adrenal size of these patients was 506 +/- 79 mm2 as compared to 132 +/- 8 mm2 in the controls (P less than 0.001). Only two patients with the late onset form revealed adrenal glands of normal size. There was a significant correlation between adrenal size and patients' age (P less than 0.01). Females with the simple virilizing form revealed adrenal glands larger than those of the late onset form (640 +/- 169 vs 308 +/- 56 mm2). Eighteen patients with AGS exhibited one (n = 11) or several (n = 7) adrenal tumors, the size of which was 5-9 mm in diameter in 9, 10-20 mm in 7, and more than 50 mm in 2 patients. There was a significant correlation between adrenal hyperplasia and tumor diameter (P less than 0.001). No correlation was found between tumor size and plasma concentrations of testosterone or 17-hydroxyprogesterone, patients' age at the time of diagnosis, or clinical signs of androgenization. Again, tumors were larger in females suffering from the simple virilizing form of AGS than in those with the late onset form (14.8 +/- 5.5 vs 7.7 +/- 0.8 mm).(ABSTRACT TRUNCATED AT 250 WORDS)