Royal Australasian College of Surgeons
HOW I DO IT
How to do: En bloc nephro-adrenalectomy with right hepatectomy and caval thrombectomy for adrenocortical carcinoma causing budd chiari syndrome
Background
Adrenocortical carcinomas are rare lesions and R0 surgical re- section remain their only chance for cure. They can be diagnosed because of their secretion but also on symptoms in relation with bulky tumours. Large lesion with caval thrombus can be surgically challenging, especially, as in the present case, when hepatic outflow obstruction leads to Budd Chiari Syndrome. The present video shows a nephro-adrenalectomy with en bloc right hepatectomy and caval thrombectomy for adrenocortical carcinoma in a patient with Budd Chiari Syndrome.
Case and procedure presentation
We here present a 22-year-old woman with no past medical history. In 2017, during her pregnancy a 5 cm adrenal mass was discovered, with no additional workup. Five years after, because of increasing abdominal pain and abdominal tenderness, a CT scan was per- formed showing a large adrenal mass. Clinically the mass was pal- pable and was associated with Cushing’s syndrome with high
blood pressure, abdominal obesity and reddish stretch marks, with an increased urinary free cortisol. With no distant metastasis on CT and 18FDG-Pet CT scan, the tumour was associated with liver involvement and caval thrombus causing Budd Chiari syn- drome (Fig. 1).
As showed in the video, En bloc resection was proposed1,2 associating:
· Merced’s incision to have full access to liver, adrenal and kidney.
· Cattell - Braasch manoeuvre to access to the inferior vena cava and the duodenum.
· Section of the ureter and the gonadic vessels,
· Control the infra renal vena cava.
· Control and ligation of the right renal vein and then the right venal artery to slowly devascularised the tumour.
· The right adrenal vein was controlled and cut.
· Pringle manoeuvres to perform en bloc right hepatectomy.
· Intraoperative ultrasonography shows the tumoral thrombus with hepatic venous outflow obstruction causing Budd Chiari syndrome and the hepatomegaly.
Liver involvement
thrombus
ANZ J Surg 95 (2025) 260-261
@ 2025 The Author(s).
ANZ Journal of Surgery published by John Wiley & Sons Australia, Ltd on behalf of Royal Australasian College of Surgeons.
· Control of the right portal vein, the right hepatic artery that is stapled and cut in order to devascularized the right liver.
· Short phrenotomy to control the superior vena cava and start after Pringle manoeuvre the right hepatectomy with anterior approach.
· Control of the right hepatic vein
· Under caval exclusion, cavotomy and thrombus resection. The vena cava is closed longitudinally.
· Omentoplasty
Operative time was 390 min, with 7000 mL blood loss during hepatectomy on Budd Chiari syndrome. Perioperative transfusion was 14 packed red blood cells, 4 fresh frozen plasma and 6 platelets. In view of the need for massive transfusion, total vascular exclusion was tried but with a poor hemodynamic tolerance. This surgery needs experienced surgeon both in adrenal and HPB surgery and a collaborative work between surgeon and anesthesiologists. The only postoperative complication was chylous ascites treated medi- cally with the need of prolonged drainage and fat-free alimentation; and pneumonia treated by antibiotics. She was discharged home on postoperative day 13.
Pathological examination showed a RO resection of 19 cm, 4439 g adrenocortical carcinoma, with 2 liver metastases and tumoral caval thrombus (pT4N0M1). The Weiss score was 8 and the Ki 67 was 80%. Adjuvant treatment with cisplatine-etoposide and OP’DDD was recommended.
AUTHOR CONTRIBUTIONS
C. Bouygues, C. Lim, F. Menegaux, S. Gaujoux: writing, reviewing, assembly, and study design.
References
1. Fassnacht M, Dekkers OM, Else T et al. European Society of Endocri- nology Clinical Practice Guidelines on the management of adrenocortical
carcinoma in adults, in collaboration with the European network for the study of adrenal tumors. Eur. J. Endocrinol. 2018; 179: G1-G46.
2. Gaujoux S, Mihai R, Joint Working Group of ESES and ENSAT. European Society of Endocrine Surgeons (ESES) and European network for the study of adrenal Tumours (ENSAT) recommendations for the sur- gical management of adrenocortical carcinoma. Br. J. Surg. 2017; 104: 358-76.
Supporting information
Additional Supporting Information may be found in the online ver- sion of this article at the publisher’s web-site:
Video S1. Nephro-adrenalectomy with right hepatectomy and caval thrombectomy for adrenocortical carcinoma.
Constance Bouygues,* MD Chétana Lim, ¡ MD, PhD Fabrice Menegaux, ** MD, PhD Sébastien Gaujoux, ** # MD, PhD D
*Department of Digestive and Endocrine Surgery, Sorbonne Université, Pitié-Salpêtrière Hospital, Paris, France, ¿Department of HPB Surgery, Sorbonne Université, Pitié-Salpêtrière Hospital, Paris, France and Sorbonne University, Paris, France
Correspondence
Pr Sébastien Gaujoux, Department of Pancreatic, General, Visceral, and Endocrine Surgery, AP-HP, Sorbonne Université, Pitié- Salpêtrière Hospital, Paris, 47-83 Avenue de l’Hôpital, 75013 Paris, France. Email: sebastien.gaujoux@aphp.fr and sebastien. gaujoux@gmail.com
doi: 10.1111/ans.19350