How to Differentiate Benign from Malignant Adrenocortical Tumors?

Adrenocortical carcinoma (ACC) is a rare cancer with a poor prognosis. Adrenal incidentalomas are, however, commonly identified in clinical practice. Discrimination between benign and malignant adrenal tumors is of great importance considering the large differences in clinical behavior requiring dif...

Full description

Bibliographic Details
Main Authors: Charlotte L. Viëtor, Sara G. Creemers, Folkert J. van Kemenade, Tessa M. van Ginhoven, Leo J. Hofland, Richard A. Feelders
Format: Article
Language:English
Published: MDPI AG 2021-08-01
Series:Cancers
Subjects:
Online Access:https://www.mdpi.com/2072-6694/13/17/4383
_version_ 1797521593482608640
author Charlotte L. Viëtor
Sara G. Creemers
Folkert J. van Kemenade
Tessa M. van Ginhoven
Leo J. Hofland
Richard A. Feelders
author_facet Charlotte L. Viëtor
Sara G. Creemers
Folkert J. van Kemenade
Tessa M. van Ginhoven
Leo J. Hofland
Richard A. Feelders
author_sort Charlotte L. Viëtor
collection DOAJ
description Adrenocortical carcinoma (ACC) is a rare cancer with a poor prognosis. Adrenal incidentalomas are, however, commonly identified in clinical practice. Discrimination between benign and malignant adrenal tumors is of great importance considering the large differences in clinical behavior requiring different strategies. Diagnosis of ACC starts with a thorough physical examination, biochemical evaluation, and imaging. Computed tomography is the first-level imaging modality in adrenal tumors, with tumor size and Hounsfield units being important features for determining malignancy. New developments include the use of urine metabolomics, also enabling discrimination of ACC from adenomas preoperatively. Postoperatively, the Weiss score is used for diagnosis of ACC, consisting of nine histopathological criteria. Due to known limitations as interobserver variability and lack of accuracy in borderline cases, much effort has been put into new tools to diagnose ACC. Novel developments vary from immunohistochemical markers and pathological scores, to markers at the level of DNA, methylome, chromosome, or microRNA. Molecular studies have provided insights into the most promising and most frequent alterations in ACC. The use of liquid biopsies for diagnosis of ACC is studied, although in a small number of patients, requiring further investigation. In this review, current diagnostic modalities and challenges in ACC will be addressed.
first_indexed 2024-03-10T08:14:42Z
format Article
id doaj.art-880590c5875644fd91ade073a8ab4780
institution Directory Open Access Journal
issn 2072-6694
language English
last_indexed 2024-03-10T08:14:42Z
publishDate 2021-08-01
publisher MDPI AG
record_format Article
series Cancers
spelling doaj.art-880590c5875644fd91ade073a8ab47802023-11-22T10:26:37ZengMDPI AGCancers2072-66942021-08-011317438310.3390/cancers13174383How to Differentiate Benign from Malignant Adrenocortical Tumors?Charlotte L. Viëtor0Sara G. Creemers1Folkert J. van Kemenade2Tessa M. van Ginhoven3Leo J. Hofland4Richard A. Feelders5Department of Surgical Oncology and Gastrointestinal Surgery, Erasmus MC Cancer Institute, 3015GD Rotterdam, The NetherlandsDepartment of Internal Medicine, Division of Endocrinology, Erasmus MC University Medical Center, 3015GD Rotterdam, The NetherlandsDepartment of Pathology, Erasmus MC University Medical Center, 3015GD Rotterdam, The NetherlandsDepartment of Surgical Oncology and Gastrointestinal Surgery, Erasmus MC Cancer Institute, 3015GD Rotterdam, The NetherlandsDepartment of Internal Medicine, Division of Endocrinology, Erasmus MC University Medical Center, 3015GD Rotterdam, The NetherlandsDepartment of Internal Medicine, Division of Endocrinology, Erasmus MC University Medical Center, 3015GD Rotterdam, The NetherlandsAdrenocortical carcinoma (ACC) is a rare cancer with a poor prognosis. Adrenal incidentalomas are, however, commonly identified in clinical practice. Discrimination between benign and malignant adrenal tumors is of great importance considering the large differences in clinical behavior requiring different strategies. Diagnosis of ACC starts with a thorough physical examination, biochemical evaluation, and imaging. Computed tomography is the first-level imaging modality in adrenal tumors, with tumor size and Hounsfield units being important features for determining malignancy. New developments include the use of urine metabolomics, also enabling discrimination of ACC from adenomas preoperatively. Postoperatively, the Weiss score is used for diagnosis of ACC, consisting of nine histopathological criteria. Due to known limitations as interobserver variability and lack of accuracy in borderline cases, much effort has been put into new tools to diagnose ACC. Novel developments vary from immunohistochemical markers and pathological scores, to markers at the level of DNA, methylome, chromosome, or microRNA. Molecular studies have provided insights into the most promising and most frequent alterations in ACC. The use of liquid biopsies for diagnosis of ACC is studied, although in a small number of patients, requiring further investigation. In this review, current diagnostic modalities and challenges in ACC will be addressed.https://www.mdpi.com/2072-6694/13/17/4383adrenal tumorsadrenocortical carcinomadiagnosticsmolecular markers
spellingShingle Charlotte L. Viëtor
Sara G. Creemers
Folkert J. van Kemenade
Tessa M. van Ginhoven
Leo J. Hofland
Richard A. Feelders
How to Differentiate Benign from Malignant Adrenocortical Tumors?
Cancers
adrenal tumors
adrenocortical carcinoma
diagnostics
molecular markers
title How to Differentiate Benign from Malignant Adrenocortical Tumors?
title_full How to Differentiate Benign from Malignant Adrenocortical Tumors?
title_fullStr How to Differentiate Benign from Malignant Adrenocortical Tumors?
title_full_unstemmed How to Differentiate Benign from Malignant Adrenocortical Tumors?
title_short How to Differentiate Benign from Malignant Adrenocortical Tumors?
title_sort how to differentiate benign from malignant adrenocortical tumors
topic adrenal tumors
adrenocortical carcinoma
diagnostics
molecular markers
url https://www.mdpi.com/2072-6694/13/17/4383
work_keys_str_mv AT charlottelvietor howtodifferentiatebenignfrommalignantadrenocorticaltumors
AT saragcreemers howtodifferentiatebenignfrommalignantadrenocorticaltumors
AT folkertjvankemenade howtodifferentiatebenignfrommalignantadrenocorticaltumors
AT tessamvanginhoven howtodifferentiatebenignfrommalignantadrenocorticaltumors
AT leojhofland howtodifferentiatebenignfrommalignantadrenocorticaltumors
AT richardafeelders howtodifferentiatebenignfrommalignantadrenocorticaltumors