Haak H R, Fleuren G J
Department of Endocrinology, University Hospital Leiden, The Netherlands.
Cancer. 1995 Feb 1;75(3):860-4. doi: 10.1002/1097-0142(19950201)75:3<860::aid-cncr2820750318>3.0.co;2-g.
The syndrome of multiple endocrine neoplasia type 1 frequently involves the adrenal cortex. The relationship between the diffuse neuroendocrine system and the adrenal cortex is not clear however, particularly as the adrenal cortex is not considered to be an intrinsic part of the diffuse neuroendocrine system.
The possible relationship between the adrenal cortex and the diffuse neuroendocrine system was investigated in a study of the immunohistochemical characteristics of ten normal adrenal glands, four adrenal adenomas, and 18 adrenocortical carcinomas using the indirect peroxidase method of staining intermediate filaments and neuroendocrine proteins.
With synaptophysin, 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 adenomas.
Adrenocortical tumors may originate from neuroendocrine foci. The findings illuminate the pathogenesis of adrenocortical carcinoma, and may carry significant implications about the choice of treatment of patients with this malignancy and other related tumors.
1型多发性内分泌肿瘤综合征常累及肾上腺皮质。然而，弥漫性神经内分泌系统与肾上腺皮质之间的关系尚不清楚，尤其是因为肾上腺皮质不被认为是弥漫性神经内分泌系统的固有组成部分。
采用间接过氧化物酶法对10个正常肾上腺、4个肾上腺腺瘤和18个肾上腺皮质癌的中间丝和神经内分泌蛋白进行免疫组织化学染色，研究肾上腺皮质与弥漫性神经内分泌系统之间的可能关系。
突触素、神经元特异性烯醇化酶和波形蛋白在正常肾上腺皮质仅少数球状带细胞中有局灶性染色，而肾上腺皮质癌和腺瘤对这些蛋白广泛呈阳性。角蛋白免疫反应性在100%的正常皮质中存在，在仅一半的癌中可检测到，在所有腺瘤中均不存在。
肾上腺皮质肿瘤可能起源于神经内分泌灶。这些发现阐明了肾上腺皮质癌的发病机制，可能对这种恶性肿瘤及其他相关肿瘤患者的治疗选择具有重要意义。
