Stumme Nebennierentumoren bei Patienten mit Adrenogenital Syndrom

Summary 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-hydroxylase-, one from C-3-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<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<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<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). Our investigations show that adrenal tumors are found almost regularly (82%) in patients suffering from AGS. Incidence and size of these tumors appear to correlate to the degree as well as the duration of adrenal stimulation. As these tumors are benign, it is suggested that in case of accidental detection of silent adrenal tumors, an adrenogenital syndrome should be excluded by endocrinological tests.

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Abkürzungsverzeichnis ACTH Adrenocorticotropes Hormon - AGS Adrenogenital Syndrom - CT Computertomogramm - NN NebennierenUnterstützt durch: Ministerium für Wissenschaft und Forschung des Landes Nordrhein-Westfalen (IV B 5-400 021 86)