THE OCCURRENCE AND SIGNIFICANCE OF LEYDIG CELL PROLIFERATION IN FAMILIAL ADRENAL CORTICAL HYPERPLASIA*†

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Abstract
The clinical features and gross and microscopic pathologic findings in 3 male infants with familial (congenital) adrenal cortical hyperplasia are reported. All 3 had nodular hyperplasia of cells, considered to be Leydig cells, in the region of the testicular hilum. These nodules differ from those of ectopic adrenal tissue by the nuclear and cytologic features of the cells, their lack of a capsule, the lack of arrangement of their cells into cords, and by their relation to nerve fibers. The anterior pituitaries of all 3 patients showed evidence of increased secretory activity of the basophils, supporting the conclusion that the basic cause of both the Leydig cell and the adrenal cortical hyperplasia is over-secretion of trophic hormone, possibly luteinizing hormone, by the anterior pituitary.