Peculiarities in the Occurrence of Adrenal Cortical Carcinoma

Abstract

Through clinical observations of peculiarities in the occurrence of certain childhood neoplasms, new understanding of cancer biology is being derived. The contribution from studies of adrenal cortical carcinoma (ACC) is far out of proportion to the frequency of the tumor, which develops in fewer than 15 children in the United States each year.¹ Etiologically interesting findings have been made in the patient or his family; eg, the spontaneous occurrence of second primary tumors, as in the two cases described by Levine in this issue of the Journal (see p 238). In 1967, findings from a relatively small series of 62 patients with ACC revealed that two had already developed astrocytomas,² as, subsequently, did a third patient.³ Previously, double primaries involving ACC had been reported with amelanotic melanoma of the cheek⁴ and with an apparent optic glioma in a child whose three maternal uncles had died of