Histopathologic Features of Adrenal Cortical Carcinoma

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.

Reticular fibre structure in the differential diagnosis of parathyroid neoplasms

“…[11] In addition, the reticulin algorithm demonstrated high interobserver reproducibility among pathologists from different institutions in diagnosis of adrenocortical carcinoma. [12] The ve-year recurrence or metastasis rate of PTC is 40-82%. [13] Among the 36 PTC cases in this study, 19 were recurrent lesions and 6 were metastatic lesions.…”

“…Reticulin bre staining provides valuable support in con rming the diagnosis of adrenocortical carcinoma, and its assessment plays a fundamental role in the reticulin algorithm as a diagnostic tool [10]. RFS destruction is the basis of diagnosis of adrenocortical carcinoma.…”

The new histological system for the diagnosis of adrenocortical cancer

“…In recent decades, the criteria for detecting adrenocortical neoplasms have been improved, which has somewhat simplified the differential diagnosis of ACC and ACA, but diagnostic errors occur in 10-15% of cases ( 7 ). Despite the seemingly small percentage of errors, it has a high social significance: it is associated with exhausting regular monitoring and examination, material costs, and is actually unreasonable in the case of overdiagnosis.…”

Special Issue on Neuroendocrine Tumors: Updates on Classification Systems, Streamlining Diagnosis, and Emphasis on Molecular Signatures in Neuroendocrine Neoplasms

“…Gambella et al5 provide a detailed account of the histopathologic features of the uncommon, but aggressive, adrenocortical carcinoma. These tumors pose a unique challenge as there is no reliable way to clinically or radiologically differentiate an adrenal carcinoma from an adenoma; therefore, careful examination by a pathologist is essential.…”

“…This special issue of Advances in Anatomic Pathology highlights several hot topics in the realm of neuroendocrine neoplasia of the pituitary, thyroid, adrenal, parathyroid glands, and the pancreas; fundamental for the contemporary practicing pathologist's diagnostic arsenal. [1][2][3][4][5][6][7] These timely reviews will comprise current best practices, including algorithmic approaches to the diagnosis and grading of such entities, along with conveying essential advances at the molecular level. In addition, 1 review focuses solely on the newest updates in familial endocrine tumor syndromes.…”