Surgical Aspects in the Treatment of Adrenocortical Carcinomas in Children: Data of the GPOH-MET 97 Trial

 

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Abstract

Background:

Adrenocortical carcinomas (ACCs) are a rare entity, with an incidence of 1.5 per million population per year. The prognosis of ACC is poor. Complete surgical resection is essential for a curative approach and significantly determines overall prognosis. Tumor resection is sophisticated and complicated by the vulnerability of the tumor and its invasive growth. Chemotherapy and Mitotane are additional therapeutic approaches that are combined with surgery in an interdisciplinary strategy.

Patients and methods:

In this study, 59 patients between 2 months and 18 years of age with histologically verified ACC were analyzed retrospectively with respect to oncosurgical aspects. Patients were registered in the GPOH-MET 97 trial of the Society of Pediatric Oncology and Haematology. Preoperative management, factors influencing surgical severity, and operative complications were assessed.

Results:

The gender ratio was 1:2 (m:f). A total of 58 patients showed increased hormonal activity and associated clinical signs of hormonal excess. Tumor volume was ≥ 300 mL in 25 patients. These patients showed an increased rate of operative complications and a poorer overall survival (OS) rate (p<0.01). A total of 14 patients showed metastatic spread, particularly to the lungs and lymph nodes. Biopsy of the tumor was performed in 12 patients. Tumor rupture occurred in 11 patients. Preoperative biopsy and/or experienced tumor rupture were associated with poorer OS rate. R2 resection only was achievable in 5 patients, and surgery was not feasible in 3 patients.

Conclusions:

In conclusion, since most of the pediatric ACC are hormone active and can be diagnosed clinically, the need of a tumor biopsy has to be discussed critically. Thorough pre- and perioperative management is essential for oncosurgical success.

Zusammenfassung

Hintergrund:

Das Nebennierenrindenkarzinom (ACC) ist eine seltene maligne Erkrankung mit einer Inzidenz von 1,5/1 000 000 Einwohner/Jahr und hat eine sehr ungünstige Prognose. Ein kurativer Ansatz ist nur durch die vollständige Resektion zu erreichen. Die Tumoren sind sehr vulnerabel und wachsen infiltrativ, so dass die Tumorresektion sehr anspruchsvoll ist. Chemotherapie und Mitotane sind weitere Bestandteile eines interdisziplinären Therapieansatzes.

Patienten und Methoden:

59 Patienten der GPOH-MET-97-Studie im Alter von 2 Monaten bis 18 Jahren mit histologisch gesichertem ACC wurden unter onkochirurgischen Aspekten retrospektiv analysiert. Besonderes Augenmerk lag auf dem präoperativen Management, Faktoren, die die Radikalität der Operation beeinflussten, und operativen Komplikationen.

Ergebnisse:

Das Geschlechtsverhältnis war 1:2 (m:w). Fast alle Patienten hatten eine gesteigerte hormonelle Aktivität und klinische Zeichen des Hormonexzeses. Bei 25 Patienten betrug die Tumorgröße ≥ 300 ml. Diese Patienten hatten ein schlechteres Gesamtüberleben (OS). Insgesamt 14 Patienten zeigten eine Metastasierung vor allem in die Lunge und Lymphknoten. Bei 12 Patienten wurde eine Tumorbiopsie durchgeführt, bei 11 Patienten kam es intraoperativ zu einer Tumorruptur. Beides war assoziiert mit einem schlechteren OS. Bei 5 Patienten konnte lediglich eine R2-Resektion erreicht werden, und in 3 Fällen war eine Operation nicht möglich.

Schlussfolgerung:

Die meisten ACC im Kindesalter sind hormonaktiv, so dass die Diagnose praktisch immer klinisch gestellt werden kann. Somit muss die Notwendigkeit einer Tumorbiopsie kritisch hinterfragt werden. Ein sorgfältiges prä- und perioperatives Management ist entscheidend für den chirurgischen Erfolg.

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