[Clinical significance of urinary tetrahydro-11-deoxycortisol analysis in endocrine tests of the adrenal cortex].
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Primary pigmented nodular adrenocortical disease (PPNAD) is a rare cause of Cushing's syndrome, which is adrenocorticotropic hormone-independent and involves both adrenal glands. The characteristic pathological features include multiple pigmented cortical nodules and atrophy of the internodular cortex. The adrenal size ranges from small to normal or slightly enlarged. Review of the gross, light microscopic, and ultrastructural features of eight cases seen at the National Institutes of Health revealed several very unusual findings. We observed the following in our patients: macronodules up to 3.0 cm; microscopic foci of necrosis; mitoses; cortical hyperplasia rather than internodular atrophy; focal confluence of nodules giving a diffuse pattern of cortical involvement, and a focal trabecular growth pattern. The nodules were composed primarily of eosinophilic, lipid-poor cells similar to those of the zona reticularis. However, lipid-rich cells similar to those of the zona fasciculata were also demonstrated in the nodules by both light and electron microscopy. This suggests that the adrenal cortical nodules of PPNAD may be derived from not only the zona reticularis, but also the zona fasciculata.
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The article presents results of diagnostics and treatment of 26 children with tumors of the adrenals. The diversity of clinical symptoms was dependent on the zone of the adrenal cortex where the adrenocortical tumors were developing and the character of the hormones released. In diagnostics of the localization of adrenal tumors the authors give preference to pneumoretroperitoneography. A conclusion is made that the hormonal background must be studied in each patient with gynecomastia and pneumoretroperitoneography should be performed. Such a watchfulness is thought to be the only condition of the timely diagnostics of esteroma.
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