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Recent progresses have been made in the pathology of adrenal cortex including the publication of World Health Organization 2017 Endocrine Tumor. In adrenocortical adenoma, some interesting developments have been recently made in the pathology of aldosterone-producing adenoma. The first was the presence of various somatic mutations of the genes encoding plasma membrane potassium or calcium channel. In particular, KCNJ5 mutations have been reported in more than half of the patients with aldosterone-producing adenoma, but their exact roles in tumorigenesis, development, or aldosterone overproduction have still remained unknown. The other development was to finally demonstrate the precise sites of aldosterone production in surgical pathology specimens using recently developed monoclonal antibody against CYP11B2, the rate-limiting step of aldosterone biosynthetic pathway. Immunolocalization of CYP11B2 did contribute to the novel classification of aldosterone-producing adrenocortical lesions, especially the small adrenocortical lesions, which could not be detected by computed tomography or other clinical imaging modalities, into microadenoma, multiple nodules, or diffuse hyperplasia.In adrenocortical carcinoma, the criterion standard of histological differentiation between adenoma and carcinoma is still the use of multiple scoring system, such as the criteria of Weiss, but some auxiliary methods to refine the individual histological factors such as Ki67 labeling index, silver staining, or others to define architectural abnormalities have been proposed. In addition, it is also important to note that there were several histological variants of adrenocortical carcinoma in which criteria of Weiss could not necessarily provide accurate prediction of biological behavior. These histological variants include pediatric, sarcomatoid, myxoid, and oncocytic adrenocortical carcinoma. In addition, Ki67 labeling index should be standardized in its methodology and interpretation as in other malignancies but could provide important information not only in diagnosis but also as to the postoperative clinical or biological behavior of the patients with adrenocortical carcinoma. Numerous molecular or gene profiling studies have been published on adrenocortical carcinoma, and even molecular-based classification has been proposed. Results of these studies are all interesting, but their clinical or biological value has still remained unknown.Adrenal metastases have been generally considered as advanced stage in cancer patients. However, in the cases of solitary adrenal metastasis, adrenalectomy or surgical resection of adrenals with metastasis has been reported to significantly improve overall clinical survival of the cancer patients, especially lung cancer. In addition, computed tomography or endoscopic ultrasound–guided core needle biopsy or fine-needle aspiration cytology could provide sufficient materials to pathologists in differential diagnosis between adrenocortical primary and secondary malignancies. Immunohistochemistry of steroidogenic factor 1 could play pivotal roles in this differential diagnosis.