RESUMO
The characteristic adrenal lesions of five patients with bilateral primary adrenocortical nodular dysplasia and Cushing's syndrome developing in adolescence are presented histopathologically, and in two cases ultrastructurally. Two of the patients were siblings with additional extra-adrenal lesions such as pigmented spots on the face, myxoid cutaneous tumours and in one case familial cardiac myxoma. The adrenals were small with an uneven surface due to numerous small brown to black pigmented nodules in the inner cortex. The nodules were composed of eosinophilic giant cells filled with lipofuscin granules. The familial cases in addition revealed a pronounced disorganization of the adrenocortical architecture with capsular disruption and infiltration of periadrenal fatty tissue. The early onset of Cushing's syndrome in the second decade, the bilateral involvement of adrenal glands despite ACTH independence and the familial occurrence in combination with other peculiar rare lesions are strongly suggestive of a malformative process of the zona reticularis of the adrenal glands.