Primary adrenocortical micronodular dysplasia: enzyme histochemical and ultrastructural studies of two cases with a review of the literature

Abstract

The adrenal glands from two patients with primary adrenocortical micronodular dysplasia (PAMD) were studied (no. 1, a 23-year-old man with cardiac myxomas and sarcoidosis; no. 2, a 16-year-old girl). The PAMD cells showed intense activity of 3 beta-hydroxysteroid dehydrogenase (3 beta DH), succinate DH, glucose-6-phosphate DH, alkaline phosphatase (AlPase), and other DHs and lysosomal hydrolases, giving a characteristic staining pattern. The staining patterns correlated well with ultrastructural findings. The larger adrenals (no. 1: 16.6 g) were associated with larger black nodules and internodular cortex (INC) with fairly well-retained enzyme activities, and contained cell clusters and single cells with intense AlPase activity with the appearance of PAMD nodules in the buds. The smaller adrenals (no. 2: 5.4 g) were associated with smaller black/yellow nodules, and had INC with weaker enzyme activity. Analysis of descriptions of INC in 25 previously reported cases revealed that the larger adrenals (more than 10 g) had less atrophic INC than the smaller ones. The characteristic enzyme pattern in PAMD cells explains the paradox that PAMD adrenals smaller than normal can cause Cushing's syndrome, and may be useful for investigating neoplastic and non-neoplastic counterparts in other adrenal lesions. The relationship between PAMD cells and INC is not simply one of autonomy versus atrophy, and both cell types may be stimulated by a certain common trophic factor. Thus, PAMD belongs to the category of hyperplasia.