Definition
Malignant epithelial neoplasm of adrenal cortical cells.
Clinical Features
Morbidity is mainly related to hormone overproduction by the tumor, with glucocorticoid excess and virilization being the most common syndromes. Patients may also experience an abdominal mass and flank pain as invasion of adjacent organs or spontaneous rupture of the neoplasm ensue. Incidentally detected nonfunctioning adrenocortical carcinomas are not infrequent. In rare instances, distant metastases to liver, lung, retroperitoneal lymph nodes, and bone may appear as the first clinical manifestations.
Incidence
Adrenocortical carcinomas are rare, with an annual incidence of 1–2 cases per million (Else et al. 2014). They make up approximately 0.2% of all cancers and 3% of endocrine neoplasms.
Age
A bimodal age distribution is observed, the main peak occurring in the seventh decade of life and the secondary peak in childhood.
Sex
There is a prevalence in women with a female-to-male ratio as high as...
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References and Further Reading
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de Krijger, R. R., & Papathomas, T. G. (2012). Adrenocortical neoplasia: Evolving concepts in tumorigenesis with an emphasis on adrenal cortical carcinoma variants. Virchows Archiv, 460, 9–18.
Duregon, E., Fassina, A., Volante, M., Nesi, G., Santi, R., Gatti, G., Cappellesso, R., Dalino Ciaramella, P., Ventura, L., Gambacorta, M., Dei Tos, A. P., Loli, P., Mannelli, M., Mantero, F., Berruti, A., Terzolo, M., & Papotti, M. (2013). The reticulin algorithm for adrenocortical tumor diagnosis: A multicentric validation study on 245 unpublished cases. American Journal of Surgical Pathology, 37, 1433–1440.
Else, T., Kim, A. C., Sabolch, A., Raymond, V. M., Kandathil, A., Caoili, E. M., Jolly, S., Miller, B. S., Giordano, T. J., & Hammer, G. D. (2014). Adrenocortical carcinoma. Endocrine Reviews, 35, 282–326.
Erickson, L. A. (2018). Challenges in surgical pathology of adrenocortical tumours. Histopathology, 72, 82–96.
Lau, S. K., & Weiss, L. M. (2009). The Weiss system for evaluating adrenocortical neoplasms: 25 years later. Human Pathology, 40, 757–768.
Papathomas, T. G., Duregon, E., Korpershoek, E., Restuccia, D. F., van Marion, R., Cappellesso, R., Sturm, N., Rossi, G., Coli, A., Zucchini, N., Stoop, H., Oosterhuis, W., Ventura, L., Volante, M., Fassina, A., Dinjens, W. N., Papotti, M., & de Krijger, R. R. (2016). Sarcomatoid adrenocortical carcinoma: A comprehensive pathological, immunohistochemical, and targeted next-generation sequencing analysis. Human Pathology, 58, 113–122.
Papotti, M., Volante, M., Duregon, E., Delsedime, L., Terzolo, M., Berruti, A., & Rosai, J. (2010). Adrenocortical tumors with myxoid features: A distinct morphologic and phenotypical variant exhibiting malignant behavior. American Journal of Surgical Pathology, 34, 973–983.
Poli, G., Ruggiero, C., Cantini, G., Canu, L., Baroni, G., Armignacco, R., Jouinot, A., Santi, R., Ercolino, T., Ragazzon, B., Assié, G., Mannelli, M., Nesi, G., Lalli, E., & Luconi, M. (2019). Fascin-1 is a novel prognostic biomarker associated with tumor invasiveness in adrenocortical carcinoma. Journal of Clinical Endocrinology and Metabolism, 104, 1712–1724.
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Nesi, G., Santi, R. (2020). Adrenocortical Carcinoma. In: van Krieken, J. (eds) Encyclopedia of Pathology. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-28845-1_5237-1
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DOI: https://doi.org/10.1007/978-3-319-28845-1_5237-1
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