Key result
In a patient with ACTH-independent bilateral adrenocortical macronodular hyperplasia, P450c17 immunoreactivity was predominantly observed in small compact cortical cells, while 3βHSD was observed exclusively in large clear cortical cells.
Case Report (n=1)
The unique differential expression of steroidogenic enzymes between clear and compact cells in AIMAH may explain the relatively mild hypercortisolism despite markedly enlarged adrenal glands.
Suggests cell-specific enzyme expression may modulate hypercortisolism severity in AIMAH; hypothesis-generating and should not change practice.
A 55-year-old woman developed Cushing's syndrome due to ACTH-independent bilateral adrenocortical macronodular hyperplasia. Plasma ACTH was undetectable, and was not stimulated by administration of metyrapone, CRH, or insulin. Hypercortisolism was not suppressed by a high dose of dexamethasone, but was responsive to ACTH. Both adrenal glands were enlarged with a total weight of 200 g, and contained multiple nodules composed of two cell types (large clear cells and small compact cells). In immunohistochemical studies, P450c17 immunoreactivity was predominantly observed in small compact cortical cells, while that of 3 beta HSD was observed exclusively in large clear cortical cells. This pattern of expression of steroidogenic enzymes as well as histological and clinical features is considered to be unique to ACTH-independent bilateral adrenocortical macronodular hyperplasia.
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Koizumi et al. (1994) conducted a case report in Cushing's syndrome due to ACTH-independent bilateral adrenocortical macronodular hyperplasia (AIMAH) (n=1). ACTH-independent bilateral adrenocortical macronodular hyperplasia (AIMAH) was evaluated on Immunolocalization of steroidogenic enzymes (P450c17 and 3βHSD). In a patient with ACTH-independent bilateral adrenocortical macronodular hyperplasia, P450c17 immunoreactivity was predominantly observed in small compact cortical cells, while 3βHSD was observed exclusively in large clear cortical cells.
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