Clinical and Histopathological Variables and Prognostic Factors of Adrenocortical Carcinoma.

Sara Sohail,Ahmed Siddiqui,Umal Azmat,Sajid Mushtaq,Madiha Syed,Muhammad Azam,Usman Hassan,Syed Abbas Raza,Khurram Mir,Waqas Shafiq,Shehryar Khawaja

doi:10.7759/cureus.15721

Abstract

Adrenocortical carcinoma (ACC) is a rare and highly aggressive tumor with a poor prognosis. The literature on prognosis from low-income or low-middle-income countries is limited and scarce. This study aimed to determine the clinical and histopathological characteristics, recurrence-free survival (RFS), overall survival (OS), and the factors affecting ACC's prognosis.This was a retrospective study of patients that presented with ACC to the Shaukat Khanum Memorial Cancer & Research Center, Lahore, Pakistan, between January 2011 and May 2018. Information regarding demographics and clinical and histopathological variables were extracted and analyzed. Of the 25 subjects, 16 (64%) were female. The median age of the sample was 35 years (range; 21 - 72 years).Statistically significant associations were found between RFS and functional status of the tumor (p = 0.014), cortisol overproduction (p = 0.02), androgen excess (testosterone [p = 0.03] and dehydroepiandrosterone sulfate [DHEA SO4] [p = 0.004]), Ki-67 score (p = 0.03), mitotic rate (p = 0.02), stratified mitotic rate (p = 0.01), and composite variable of disease (p = 0.004). The OS was found to have statistical associations with cortisol hypersecretion (p = 0.02), DHEA SO4 excess (p = 0.01), Modified Weis Score (p < 0.001), mitotic rate (p = 0.02), stratified mitotic rate (p = 0.003), and composite variable of disease (p = 0.001). Linear regression (forward-type) analysis suggested that the functional status of the tumor and the disease recurrence index statistically predicted the variance in RFS and OS, respectively.Multiple clinical and histopathological variables appear to affect the prognosis of ACC. However, based on multivariable analysis, it appears that the functional status of the tumor and the composite variable of disease recurrence are predictors of RFS and OS, respectively.

Highlights

Adrenocortical carcinoma (ACC) is a rare, highly aggressive malignancy with an incidence of 0.72 million persons per year [1]
Based on multivariable analysis, it appears that the functional status of the tumor and the composite variable of disease recurrence are predictors of recurrence-free survival (RFS) and overall survival (OS), respectively
The OS was significantly associated with clinical cushingoid appearance (p ≤ 0.01), cortisol excess (p = 0.02), DHEA SO4 overproduction (p = 0.01), Modified Weiss score (p ≤ 0.01), mitotic rate (p = 0.02), stratified mitotic rate (p ≤ 0.01), composite variable of disease (p ≤ 0.01)

Summary

Introduction

Adrenocortical carcinoma (ACC) is a rare, highly aggressive malignancy with an incidence of 0.72 million persons per year [1]. It may present at any age; there is a bimodal age distribution. Within the secretory ACC sub-type, the majority of the tumors produce cortisol excess. The second most commonly produced hormones in patients with ACC are adrenal androgens, causing rapid-onset male pattern baldness, hirsutism, virilization, and menstrual irregularities in women. Radical open surgical excision of the primary tumor is the only curative therapy for ACC [5]. Mitotane and radiation therapy have been used as adjuvant modalities [3,6]

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