Abstract
Simple SummaryAdrenocortical carcinoma is a rare cancer with a poor prognosis. Adrenal tumors are, however, commonly identified in clinical practice. Discrimination between benign and malignant adrenal tumors is of great importance to determine the appropriate treatment and follow-up strategy. This review summarizes the current diagnostic strategies and challenges to distinguish benign from malignant adrenal lesions. We will focus both on radiological and biochemical assessments, enabling diagnosis of the adrenal lesion preoperatively, and on histopathological and a wide variety of molecular assessments that can be done after surgical removal of the adrenal lesion. Furthermore, new non-invasive strategies such as liquid biopsies, in which blood samples are used to study circulating tumor cells, tumor DNA and microRNA, will be addressed in this review.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.
Highlights
Adrenal lesions are commonly encountered in clinical practice and often incidentally discovered
At least one mutation could be detected in the tumors of eight patients, but mutations in the cell-free DNA (cfDNA) were only found in two out of these eight patients [167]. These findings indicate that cfDNA mutation detection can vary among Adrenocortical carcinoma (ACC) patients, which might be associated with tumor burden and prognosis
Accurate diagnosis of malignancy is crucial in order to guide the decision on adrenalectomy, for prognosis stratification, and to determine the intensity and duration of follow-up
Summary
Adrenal lesions are commonly encountered in clinical practice and often incidentally discovered. Several studies have reported the use of FDG-PET/CT for assessing malignant potential, it does not provide robust information on the origin of the adrenal mass (e.g., metastases, pheochromocytoma, primary adrenal) [25,26,27] This imaging modality should be considered as an additional imaging technique and is primarily useful in the case of suspected malignant disease [28]. With a sensitivity of 91% and a specificity of 97% for the assessment of malignancy of adrenal lesions, FDG-PET is thought to be especially helpful to rule out any diagnosis of ACC or metastatic disease [29]. Patients may present with symptoms or complaints as a result of local tumor growth or metastases, like abdominal discomfort, back pain or abdominal fullness [43]
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