Histopathologic Features of Adrenal Cortical Carcinoma
- 8 September 2022
- journal article
- review article
- Published by Ovid Technologies (Wolters Kluwer Health) in Advances in Anatomic Pathology
- Vol. 30 (1), 34-46
- https://doi.org/10.1097/pap.0000000000000363
Abstract
Adrenal cortical carcinoma (ACC) is a rare and aggressive malignancy that poses challenging issues regarding the diagnostic workup. Indeed, no presurgical technique or clinical parameters can reliably distinguish between adrenal cortical adenomas, which are more frequent and have a favorable outcome, and ACC, and the final diagnosis largely relies on histopathologic analysis of the surgical specimen. However, even the pathologic assessment of malignancy in an adrenal cortical lesion is not straightforward and requires a combined evaluation of multiple histopathologic features. Starting from the Weiss score, which was developed in 1984, several histopathologic scoring systems have been designed to tackle the difficulties of ACC diagnosis. Dealing with specific histopathologic variants (eg, Liss-Weiss-Bisceglia scoring system for oncocytic ACC) or patient characteristics (eg, Wieneke index in the pediatric setting), these scores remarkably improved the diagnostic workup of ACC and its subtypes. Nevertheless, cases with misleading features or discordant correlations between pathologic findings and clinical behavior still occur. Owing to multicentric collaborative studies integrating morphologic features with ancillary immunohistochemical markers and molecular analysis, ACC has eventually emerged as a multifaceted, heterogenous malignancy, and, while innovative and promising approaches are currently being tested, the future clinical management of patients with ACC will mainly rely on personalized medicine and target-therapy protocols. At the dawn of the new Fifth World Health Organization classification of endocrine tumors, this review will tackle ACC from the pathologist’s perspective, thus focusing on the main available diagnostic, prognostic, and predictive tissue-tethered features and biomarkers and providing relevant clinical and molecular correlates.Keywords
This publication has 182 references indexed in Scilit:
- Towards an understanding of the role of p53 in adrenocortical carcinogenesisMolecular and Cellular Endocrinology, 2012
- Pediatric adrenocortical tumors: morphological diagnostic criteria and immunohistochemical expression of matrix metalloproteinase type 2 and human leucocyte-associated antigen (HLA) class II antigens: Results from the Italian Pediatric Rare Tumor (TREP) Study projectHuman Pathology, 2012
- Adrenocortical neoplasia: evolving concepts in tumorigenesis with an emphasis on adrenal cortical carcinoma variantsVirchows Archiv, 2011
- The Weiss Score and Beyond—Histopathology for Adrenocortical CarcinomaHormones and Cancer, 2011
- Adrenocortical Tumors With Myxoid Features: A Distinct Morphologic and Phenotypical Variant Exhibiting Malignant BehaviorThe American Journal of Surgical Pathology, 2010
- Calculation of Risk of Colorectal and Endometrial Cancer Among Patients With Lynch SyndromeGastroenterology, 2009
- Beckwith–Wiedemann syndromeEuropean Journal of Human Genetics, 2009
- Adrenocortical Carcinomas and Adrenal Pheochromocytomas: Mass and Enhancement Loss Evaluation at Delayed Contrast-enhanced CTRadiology, 2005
- Adrenal Cortical Neoplasms in the Pediatric PopulationThe American Journal of Surgical Pathology, 2003
- An inherited p53 mutation that contributes in a tissue-specific manner to pediatric adrenal cortical carcinomaProceedings of the National Academy of Sciences of the United States of America, 2001