Background. The syndrome of multiple endocrine neoplasia type 1 freque
ntly involves the adrenal cortex. The relationship between the diffuse
neuroendocrine system and the adrenal cortex is not clear however, pa
rticularly as the adrenal cortex is not considered to be an intrinsic
part of the diffuse neuroendocrine system. Methods. The possible relat
ionship between the adrenal cortex and the diffuse neuroendocrine syst
em was investigated in a study of the immunohistochemical characterist
ics of ten normal adrenal glands, four adrenal adenomas, and 18 adreno
cortical carcinomas using the indirect peroxidase method of staining i
ntermediate filaments and neuroendocrine proteins. Results. With synap
tophysin, NSE, and vimentin there was focal staining in only few zona
glomerulosa cells in the normal adrenal cortex, whereas adrenocortical
carcinomas and adenomas were extensively positive for these proteins.
Keratin immunoreactivity, present in 100% of the normal cortices, was
demonstrable in only half of the carcinomas and absent in all adenoma
s. Conclusions. Adrenocortical tumors may originate from neuroendocrin
e foci. The findings illuminate the pathogenesis of adrenocortical car
cinoma, and may carry significant implications about the choice of tre
atment of patients with this malignancy and other related tumors.