Abstract
Adrenal cortical carcinomas (ACCs) are rare; account for 3 % of endocrine tumors; affect people of all ages, including children; are more common in females; and have an incidence of one per one million patient-years. Up to 49 % of ACCs are functional, with androgen secretion (virilization) and glucocorticoid secretion (Cushing syndrome) common; functional tumors are more frequent in females. ACCs may vary in size (1–30 cm), but usually are large (10–13 cm) and may show necrosis. Rarely, ACCs may be cystic. Differentiating ACC from adenoma may be very difficult; ACCs usually are larger than adenomas. Tumors ≥100 g are particularly worrisome for malignancy; however, tumors less than 100 g have metastasized, and adenomas may weigh more than 100 g. ACCs recapitulate normal cortex but show more diffuse growth and eosinophilic cytoplasm and lack the zonation of normal adrenal cortex. Histologic features of malignancy are vascular invasion, necrosis, fibrous bands, capsular invasion, increased mitotic rate, atypical mitoses, and nuclear pleomorphism.
This is a preview of subscription content, log in via an institution.
Buying options
Tax calculation will be finalised at checkout
Purchases are for personal use only
Learn about institutional subscriptionsReferences
Erickson LA et al. Pathologic features and expression of insulin-like growth factor-2 in adrenocortical neoplasms. Endocr Pathol. 2001;12(4):429–35.
Bodie B et al. The Cleveland Clinic experience with adrenal cortical carcinoma. J Urol. 1989;141(2):257–60.
Crucitti F et al. The Italian Registry for Adrenal Cortical Carcinoma: analysis of a multiinstitutional series of 129 patients. The ACC Italian Registry Study Group. Surgery. 1996;119(2):161–70.
Didolkar MS et al. Natural history of adrenal cortical carcinoma: a clinicopathologic study of 42 patients. Cancer. 1981;47(9):2153–61.
Erickson LA et al. Cystic adrenal neoplasms. Cancer. 2004;101(7):1537–44.
Weiss LM. Comparative histologic study of 43 metastasizing and nonmetastasizing adrenocortical tumors. Am J Surg Pathol. 1984;8(3):163–9.
Aubert S et al. Weiss system revisited: a clinicopathologic and immunohistochemical study of 49 adrenocortical tumors. Am J Surg Pathol. 2002;26(12):1612–9.
Wieneke JA, Thompson LD, Heffess CS. Adrenal cortical neoplasms in the pediatric population: a clinicopathologic and immunophenotypic analysis of 83 patients. Am J Surg Pathol. 2003;27(7):867–81.
Assie G, Giordano TJ, Bertherat J. Gene expression profiling in adrenocortical neoplasia. Mol Cell Endocrinol. 2012;351(1):111–7.
Giordano TJ. Adrenocortical tumors: an integrated clinical, pathologic, and molecular approach at the University of Michigan. Arch Pathol Lab Med. 2010;134(10):1440–3.
Gicquel C et al. Rearrangements at the 11p15 locus and overexpression of insulin-like growth factor-II gene in sporadic adrenocortical tumors. J Clin Endocrinol Metab. 1994;78(6):1444–53.
Sasano H, Suzuki T, Moriya T. Adrenal cortex. In: Lloyd RV, editor. Endocrine pathology: differential diagnosis and molecular advances. Totowa: Humana Press; 2004. p. 211–26.
Gandour MJ, Grizzle WE. A small adrenocortical carcinoma with aggressive behavior. An evaluation of criteria for malignancy. Arch Pathol Lab Med. 1986;110(11):1076–9.
Weiss LM, Medeiros LJ, Vickery Jr AL. Pathologic features of prognostic significance in adrenocortical carcinoma. Am J Surg Pathol. 1989;13(3):202–6.
Brown FM et al. Myxoid neoplasms of the adrenal cortex: a rare histologic variant. Am J Surg Pathol. 2000;24(3):396–401.
Coli A et al. Sarcomatoid carcinoma of the adrenal gland: a case report and review of literature. Pathol Res Pract. 2010;206(1):59–65.
Collina G et al. Primary sarcomatoid carcinoma of the adrenal gland. First case report. Virchows Arch A Pathol Anat Histopathol. 1989;415(2):161–7.
Mearini L et al. Adrenal oncocytic neoplasm: a systematic review. Urol Int. 2013;91(2):125–33.
Bisceglia M et al. Adrenocortical oncocytic tumors: report of 10 cases and review of the literature. Int J Surg Pathol. 2004;12(3):231–43.
el-Naggar AK, Evans DB, Mackay B. Oncocytic adrenal cortical carcinoma. Ultrastruct Pathol. 1991;15(4–5):549–56.
Author information
Authors and Affiliations
Rights and permissions
Copyright information
© 2014 Springer Science+Business Media New York
About this chapter
Cite this chapter
Erickson, L.A. (2014). Adrenal Cortical Carcinoma. In: Atlas of Endocrine Pathology. Atlas of Anatomic Pathology. Springer, New York, NY. https://doi.org/10.1007/978-1-4939-0443-3_24
Download citation
DOI: https://doi.org/10.1007/978-1-4939-0443-3_24
Published:
Publisher Name: Springer, New York, NY
Print ISBN: 978-1-4939-0442-6
Online ISBN: 978-1-4939-0443-3
eBook Packages: MedicineMedicine (R0)