Results of surgical treatment of localized and locally advanced adrenocortical cancer in children

A S Temniy,S A Sardalova,S R Varfolomeeva,A P Kazantsev,N Yu Kalinchenko,P A Kerimov,M V Rubanskaya

doi:10.21682/2311-1267-2021-8-2-42-49

A S Temniy, S A Sardalova + Show 5 more

Open Access

https://doi.org/10.21682/2311-1267-2021-8-2-42-49

Copy DOI

Abstract

Introduction. Adrenal cortical carcinoma (ACC) is a rare cancer but is the most common primary cancer in the adrenal gland. Despite the low incidence of ACC the mortality rate ranges from 0.04 to 0.2 %, in the overall structure of cancer mortality. Treatment of ACC is mainly surgical and radical surgical excision is the treatment of choice for local disease stages.Aim of the study — to present our results of surgical treatment of localized and locally advanced ACC in children and to determine the risk factors of relapse.Materials and methods. Twenty-eight patients (median age of 47.8 (06—216) mo.) with localized and locally advanced ACC underwent a retrospectively analysis. Stage I, II, and III revealed in 12 (45 %), 7 (25 %), and 9 (30 %), respectively. In 19 (68 %) cases the secretion of one or more hormone observed. Macroscopically and microscopically complete resection were performed in 26 (93 %) and 23 (82 %) patients, respectively. The median tumor volume was 183 (3.6—1608) cm3 and the median tumor weight was 207.9 (48—710) g.Results. Five-year overall (OS) and relapse-free (RFS) survival were 71 % and 69 %, respectively. OS and RFS according to stage I, II, and III were 100 % vs. 71 % vs. 17 % and 100 % vs. 71 % vs. 14 % respectively. The radical surgical resection and the level of Ki-67 expression influenced significantly the rates of OS and RFS (p < 0.001).Conclusion. The main factor affecting the survival rate of ACC in children with stages I—III is the radical surgical resection. It should be taken into account when planning postoperative therapy. Some of biological characteristics of the tumor could also significantly affect the results of treatment.

Highlights

Adrenal cortical carcinoma (ACC) is a rare cancer but is the most common primary cancer in the adrenal gland
Радикальность хирургического вмешательства – основной фактор, влияющий на выживаемость при Адренокортикальный рак (АКР) у детей с I–III стадиями, что следует учитывать при планировании послеоперационной терапии наряду с рядом биологических характеристиками опухоли
Treatment of ACC is mainly surgical and radical surgical excision is the treatment of choice for local disease stages

Summary

Introduction

Adrenal cortical carcinoma (ACC) is a rare cancer but is the most common primary cancer in the adrenal gland. The main factor affecting the survival rate of ACC in children with stages I–III is the radical surgical resection. It should be taken into account when planning postoperative therapy. For citation: Temniy A.S., Kazantsev A.P., Kerimov P.A., Kalinchenko N.Yu., Rubanskaya M.V., Sardalova S.A., Varfolomeeva S.R. Results of surgical treatment of localized and locally advanced adrenocortical cancer in children. Материалы и методы В ретроспективный анализ включены 28 пациентов (15 девочек и 13 мальчиков) с гистологически доказанными локализованными и местно-распространенными формами АКР, получавших лечение или находящихся под наблюдением в НИИ ДОиГ ФГБУ «НМИЦ онкологии им. Распределение педиатрических пациентов с АКР по стадиям согласно классификации IPACTR/COG

Критерии Criteria

Presence of a microscopic or macroscopic tumor after surgery

Стадия по COG COG stage