Surgical management of adrenal tumours extending into the right atrium

Abstract

This paper discusses the surgical approach for the treatment of adrenal tumours extending into the right atrium (RA), using a cardio-pulmonary bypass (CPB) associated with deep hypothermic circulatory arrest (DHCA). Pre-operative planning and surgical steps are described in details. The association of CPB with hypothermic circulatory arrest (HCA) provides a bloodless operating field, direct intra-vascular vision, reduces the risk of embolization and allows extensive inferior vena cava (IVC) or RA repair in cases of infiltration of the vascular wall. Establishing a dedicated multidisciplinary team with experience in managing these challenging cases is fundamental to offer treatment to patients with advanced disease, who would otherwise risk being turned down for surgery. A close collaboration between general and cardiac surgeons and a deep understanding of the surgical procedure steps are fundamental to safely performing these procedures. We advocate centralising adrenal surgery in a small number of units with adequate multidisciplinary support.

Keywords: Adrenal gland tumour; adrenocortical carcinomas; deep hypothermic circulatory arrest (DHCA); inferior vena cava thrombus (IVC thrombus); right atrial mass.

Figure 1

Classification of tumour/thrombus in adrenal tumour [Ekici and Ciancio, adapted from (2)]. IVC, inferior vena cava; RA, right atrium.

Figure 2

Intra-operative trans-oesophageal echocardiogram. Thrombus in the IVC extending into the RA. IVC, inferior vena cava; RA, right atrium.

Figure 3

Schematic representation of cardio pulmonary bypass circuit and Ross basket for right atrium cannulation.

Figure 4

Radiological and intraoperative findings in a patient with level I tumour thrombus. Patient presenting with rapid progression of Cushing syndrome. During dissection of the tumour it became apparent that the right adrenal vein was invaded by tumour thrombus. After cross clamping of the IVC, a venotomy was performed, small volume tumour thrombus demonstrated in the lumen of the vein was fully removed and the IVC was sutured with 3.0 Prolene. IVC, inferior vena cava.

Figure 5

Radiological and intraoperative findings in a patient with level IIIa tumour thrombus. Patient with incidental finding of large nonsecreting ACC. Cardiopulmonary bypass allowed extraction of large volume tumour thrombus, repair of the IVC with a bovine pericardial patch and reanastomosis of left renal vein into the reconstructed IVC. Patient remains disease free 4 years after the operation. ACC, adrenocortical cancer; IVC, inferior vena cava.

Figure 6

Radiological findings of patients with level IV tumour thrombus. Phaeochromocytoma invading the IVC demonstrated on CT scan and confirmed on PET scan. IVC, inferior vena cava.