Endocrine Abstracts

Background: Adreno cortical carcinoma (ACC) is a rare malignancy associated with aggressive biological behavior and poor outcome. The reported incidence in literature is about approximately two cases per million population’s.These tumors might be functional or non-functional depending on their ability to secrete various adrenocortical hormones. ACC occurring in children and adults show distinct characteristics and there is not much literature regarding the differences. Since it is a rare disease large sample size is a difficulty.

Aim: The aim of this study was to study Clinico-pathologic profile and outcome of ACC occurring in children and adults.

Methods: This study (January 1990–June 2017) was carried out in a tertiary referral centre, included 60 patients with Adreno cortical carcinoma. Patients aged 18 years or more were classified as adults and rest as children. Demographics, clinical profile, hormonal profile, details of surgical procedures, histology and/ or cytology reports and follow up findings were noted. Survival analysis was performed using Kaplan Meir method and significance of various factors was calculated by Cox regression analysis. Various factors and outcome were compared. A P value of less than 0.05 was considered significant.

Results: There were 20 children and 40 adults. Mean Age was 8±5.7 (M: F=1: 2.1) and 44.4±15 years (M: F=1:1.1). Prevalence of functioning tumors was significantly high in children (85 vs 40% P=0.001), and Incidentaloma in adults (6.3 vs 51.7% P=0.05). Tumor stage distribution at presentation was comparable in both groups. 85% children and 62.5% of adults were operated. The mean tumor size and weight were 10.9 vs 13.7 cm (P=0.08), and 392.9 vs 892.9 gm (P=0.24) respectively. Adults had better five year overall survival (OS) than children. On univariate analysis stage of disease (P=0.008), surgical intervention (P=0.004), Weiss score (P=0.006) 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, there was not much difference in clinicpathologic attributes. Larger studies and multicentre studies are needed so that guidelines can be framed and diverse management can be avoided.