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 order 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.