Familial Cushing's syndrome with primary adrenocortical microadenomatosis (primary adrenocortical nodular dysplasia)

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Abstract
Cushing''s syndrome was found in 2 of 4 siblings all of which also had other malformations. After bilateral adrenalectomy no hyperpigmentation was noted. Morphologically the adrenals of both siblings with Cushing''s syndrome showed the typical lesions of the so-called microadenomatosis of primary adrenocortical nodular dysplasia with foci or eosinophilic giant cells. This is a new form of Cushing''s syndrome due to an inborn error of the adrenals and no hypothalamic-pituitary dysfunction.

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