Adrenocortical carcinoma in children and adults: Two decades experience in a single institution.
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Date
2016-04
Authors
Sabaretnam, M
Mishra, A
Agarwal, G
Agarwal, A
Verma, AK
Mishra, SK
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Journal ISSN
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Abstract
CONTEXT: Adrenocortical carcinoma (ACC) occurring in children and adults show distinct characteristics. However, due to rarity of the disease no large
series addressing this issue has been published. AIMS: The aim of this study was to study clinico‑pathologic profile and outcome of ACC in children
and adults. SETTINGS AND DESIGN: Tertiary referral center. Retrospective study (January 1990‑June 2011). SUBJECTS AND METHODS: Forty‑five
patients with ACC were included; 16children (aged < 18 years) and 29 adults. Clinical details, hormonal profile, operation records, pathology reports and
follow‑up findings were noted and compared. Survival analysis was performed using Kaplan‑Meier method. Log rank test and Cox regressionan alysis
were performed. RESULTS: Mean age was 8 ± 5.7 (M: F = 1:2.1) in children and 44.4 ± 15 years (M: F = 1:1.1) in adult groups. Prevalence of functioning
tumors was significantly high in children (87.5 vs. 31% P = 0.001), while prevalence of incidentalomas was high in adults (6.3 vs. 51.7% P = 0.05).
Tumor stage distribution at presentation, mean diameter (10.9 vs. 13.7 cm), and weight (392.9 vs. 892.9 g) didn’t differ significantly in two groups. Adults
had better albeit non‑significant 5 year overall survival (OS) than children (0 vs. 13%). On univariate analysis stage of disease (P = 0.008), surgical
intervention (P = 0.004), Weiss score (P = 0.04) and hormonal secretion (P = 0.04) were significantly associated with OS in adults but not in children.
No factor was found significant on multivariate analysis. CONCLUSIONS: Except for high prevalence of functioning tumors in children, clinico‑pathologic
attributes and outcome of ACC in the two groups didn’t differ significantly.
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Keywords
Adrenal tumors, Cushing’s syndrome, malignant conns’, virilizingtumors
Citation
Sabaretnam M, Mishra A, Agarwal G, Agarwal A, Verma AK, Mishra SK. Adrenocortical carcinoma in children and adults: Two decades experience in a single institution. Indian Journal of Cancer. 2016 Apr-June; 53(2): 317-321.