Summary
Background: Advanced adrenal carcinomas tend to infiltrate the adrenal vein and to grow intraluminally into the inferior vena cava, thereby diminishing or occluding the venous flow. Less often, tumour thrombi extend into the right atrium. Radical surgery requires caval or cavo-atrial thrombectomy while maintaining vascular control, complemented by multivisceral resections, if applicable.
Methods: A review of the literature — undertaken in orcer to describe surgical techniques as well as postoperative results in the treatment of organ-exceeding adrenal cancer — and own experiences are the basis of management recommendations.
Results: The extent of vascular involvement of adrenal neoplasms is classified into four different types, which require increasingly challenging surgical procedures. Low perioperative mortality and long tumour-free survival rates are reported in malignant phaeochromocytomas, adrenocortical malignancies, and other primary adrenal neoplasms such as leiomyosarcoma.
Conclusions: Nowadays, advanced disease does not preclude the patient from radical surgical therapy in adrenal malignancies. Advances in vascular surgery and extracorporeal bypass techniques have led to the development of safe techniques for the removal of tumour thrombi, even of the retrohepatic and subdiaphragmatic vena cava. The poor prognosis of patients without resection or with incomplete tumour resections justifies an aggressive surgical approach.
Zusammenfassung
Grundlagen: Fortgeschrittene Tumore der Nebenniere können die Nebennierenvene infiltrieren und per continuitatem in die V. cava inferior einwachsen, wo sie den venösen Blutfluß deutlich vermindern oder vollständig okkludieren. Seltener können intracavale Tumorthromben sogar bis in den rechten Vorhof reichen. In diesen Fällen erfordert eine radikale chirurgische Therapie eine cavale oder cavo-atriale Thrombektomie unter Wahrung der vaskulären Kontrolle, gegebenenfalls ergänzt durch multiviscerale Resektion.
Methodik: Zur Beschreibung von Resektionstechniken und postoperativen Ergebnissen in der Behandlung organ-überschreitender Nebennieren-Malignome wurde eine Literaturrecherche unternommen. Diese Resultate und eigene Erfahrungen bilden die Basis von Empfehlungen zum Management der genannten Tumore.
Ergebnisse: Die Ausdehnung der vaskulären Beteiligung von adrenalen Neoplasien läßt sich in vier Typen klassifizieren, welche unterschiedliche — zunehmend herausfordernde — Operationstechniken erfordern. Eine niedrige perioperative Mortalität und lange tumorfreie Überlebenszeiten wurden für das maligne Phäochromozytom, Nebennierenrinden-Karzinom und für andere primäre Tumoren der Nebenniere, wie z. B. das Leiomyosarkom, beschrieben.
Schlußfolgerungen: Heutzutage schließen auch fortgeschrittene Tumorstadien eines Nebennieren-Malignomes eine radikale chriurgische Therapie nicht aus. Fortschritte in der Gefäßchirurgie und der extrakorporalen Bypaß-Technik führten zur Entwicklung sicherer Verfahren zur Resektion von Tumorthromben aus der retrohepatischen und subdiaphragmalen V. cava. Die schlechte Prognose von Patienten ohne Tumorresektion oder nach inkompletter Resektion rechtfertigen einen aggressiven chirurgischen Therapieansatz.
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Musholt, T.J. Surgical management of advanced malignant adrenal tumours. Eur Surg 35, 97–101 (2003). https://doi.org/10.1046/j.1682-4016.2003.03043.x
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DOI: https://doi.org/10.1046/j.1682-4016.2003.03043.x