ABSTRACT
INTRODUCTION: Adrenocortical carcinoma (ACC) is diagnosed in paediatric patients at 5 months after symptom onset on average, and 38% die during the first 2.5 years of follow-up. This study aimed to compare the accuracy of Weiss, Van Slooten, and Wieneke histopathological ACC classifications for predicting follow-up prognosis in a paediatric population. METHODS: Data were retrieved from medical records of 57 patients aged <18 years who underwent surgical treatment for ACC with surgical follow-up over 6 months or death due to ACC. They were classified into either good (without recurrence/death due to ACC) or poor (with recurrence/death due to ACC) prognosis group. Two expert pathologists classified the ACC surgical specimens according to the Weiss, Van Slooten, and Wieneke criteria. RESULTS: The median follow-up duration was 126 (18-225) months in 38 males (66.7%) and 19 females (33.3%) (median age: 3 [1-6.5] years). The good prognosis group was younger than the poor prognosis group (median age: 3 [1.5-6.2] years vs. 5 [2-10] years). Seventeen (29.8%) patients in the poor prognosis group died due to ACC within the first 50 months of surgical follow-up; the earliest death occurred in the fourth follow-up month, and the majority of deaths occurred within 24 months of follow-up. The accuracies of Weiss, Van Slooten, and Wieneke classification systems were 40%, 47%, and 77%, respectively. DISCUSSION/CONCLUSION: The Wieneke classification showed the best accuracy but was not sufficiently precise to establish reliable prognosis for ACC in the paediatric population. The Wieneke classification had approximately 95% sensitivity and negative predictive value.
Subject(s)
Adrenal Cortex Neoplasms , Adrenocortical Carcinoma , Adrenal Cortex Neoplasms/diagnosis , Adrenocortical Carcinoma/diagnosis , Child , Child, Preschool , Female , Humans , Infant , Male , Prognosis , Retrospective StudiesABSTRACT
The nitric oxide/cyclic guanosine monophosphate (NO/cGMP) pathway has a significative influence in hemodynamic changes that occur in transplants. Classically, the ischemia-reperfusion syndrome (IRS) is characterized by hypotension and low vascular resistance, when cGMP and nitric oxide (NO) are increased, contributing to oxidative stress, within an inflammatory context. These mechanisms occur in several types of transplants, such as liver, heart, lungs, kidney, which are a therapeutic choice in several clinical conditions when conventional treatments failed. It is well known the significant relation between graft dysfunction or rejection and ischemia-reperfusion injury that is linked to inflammatory response and NO/cGMP pathway activation. This review aims to study the NO/cGMP pathway in solid organ transplants. Finally, we inquire whether physicians do not underestimate the NO/cGMP pathway.
