Pathological and Genetic Stratification for Management of Adrenocortical Carcinoma.
J Clin Endocrinol Metab
; 107(4): 1159-1169, 2022 03 24.
Article
in En
| MEDLINE
| ID: mdl-34850906
ABSTRACT
CONTEXT Adrenocortical carcinoma (ACC) is a rare endocrine malignancy that affects patients across the age spectrum. Although the overall survival in patients with ACC is poor, there is significant heterogeneity in terms of outcomes, presentation, and underlying genetic drivers. EVIDENCE ACQUISITION This review is based on the evidence collected from primary research studies, expert reviews, and published guidelines. The studies were identified through PubMed search with key words "adrenocortical carcinoma," "prognosis," "pathology," and "genetics." The PubMed search was complemented by authors' expertise, research, and clinical experience in the field of ACC. EVIDENCE SYNTHESIS:
Identification of biomarkers has been critical to gain better insight into tumor behavior and to guide therapeutic approach to patients. Tumor stage, resection status, and Ki67 are pathological tumor characteristics that have been identified as prognosticators in patients with ACC. Cortisol excess also correlates with worse prognosis. Clinical and histopathological characteristics help stratify patient outcomes, yet still up to 25% of patients have a different outcome than predicted. To bridge this gap, comprehensive genomic profiling studies have characterized additional profiles that correlate with clinical outcomes. In addition, studies of clinically applicable molecular markers are under way to further stratify outcomes in patients with ACC tumors.CONCLUSIONS:
Clinical predictors in combination with pathological markers play a critical role in the approach to patients with ACC. Recent advances in genetic prognosticators will help extend the stratification of these tumors and contribute to a personalized therapeutic approach to patients with ACC.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Adrenal Cortex Neoplasms
/
Adrenocortical Carcinoma
Type of study:
Guideline
/
Prognostic_studies
/
Systematic_reviews
Limits:
Humans
Language:
En
Journal:
J Clin Endocrinol Metab
Year:
2022
Document type:
Article
Affiliation country:
United States
Publication country:
EEUU
/
ESTADOS UNIDOS
/
ESTADOS UNIDOS DA AMERICA
/
EUA
/
UNITED STATES
/
UNITED STATES OF AMERICA
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US
/
USA