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Pathology of the Adrenal Cortex: a Reappraisal of the Past 25 Years Focusing on Adrenal Cortical Tumors

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Abstract

A reappraisal of the major advances in the diagnostic pathology of adrenal cortical lesions and tumors in the last 25 years is presented, with special reference to the definition of malignancy in primary adrenal cancer and its variants. Slightly more than 25 years ago, Weiss proposed his diagnostic scoring system for adrenal cortical carcinoma. This represented a milestone for adrenal pathologists and the starting point for further modifications of the system, either through minor changes in the scoring procedure itself or concentrating on some particular Weiss criterion such as mitotic index, integrated into alternative scoring schemes or algorithms that are currently under validation. Improvements in diagnostic immunohistochemistry have led to the identification of markers of cortical origin, such as Melan-A, alpha-inhibin, and SF-1 and of prognostic factors in carcinoma, such as the Ki-67 proliferation index and SF-1 itself. With regard to hyperplastic conditions, genetic investigations have allowed the association of the majority of cases of primary pigmented nodular adrenocortical disease (PPNAD) in Carney complex to mutations in the gene encoding the regulatory subunit 1A of protein kinase A (PRKAR1A). Other hereditary conditions are also associated with adrenal cortical tumors, including the Li–Fraumeni, Beckwith–Wiedemann, Gardner, multiple endocrine neoplasia type 1, and neurofibromatosis type 1 syndromes. Moreover, several advances have been made in the knowledge of the molecular background of sporadic tumors, and a number of molecules/genes are of particular interest as potential diagnostic and prognostic biomarkers.

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Acknowledgments

This work was partially supported by a grant from the Italian Association for Cancer Research (AIRC, Milan, grant no. IG/10795/2010 to MP) and University of Turin (ex-60 % grants to MV and MP).

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  1. Department of Oncology, University of Turin at San Luigi Hospital, Regione Gonzole 10, 10043, Orbassano, Torino, Italy

    Mauro Papotti, Eleonora Duregon & Marco Volante

  2. Molecular and Cellular Pathology, UQ Centre for Clinical Research, The University of Queensland, Brisbane, Australia

    Anne Marie McNicol

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  1. Mauro Papotti
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  2. Eleonora Duregon
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  4. Anne Marie McNicol
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Correspondence to Marco Volante.

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Papotti, M., Duregon, E., Volante, M. et al. Pathology of the Adrenal Cortex: a Reappraisal of the Past 25 Years Focusing on Adrenal Cortical Tumors. Endocr Pathol 25, 35–48 (2014). https://doi.org/10.1007/s12022-013-9291-6

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