Adrenohepatic fusion: Adhesion or invasion in primary virilizant giant adrenal carcinoma? Implications for surgical resection. Two case report and review of the literature

Abstract

Introduction: Adrenohepatic fusion means union between the adrenal gland and the liver, intermingling its parenchymas. It is not possible to identify this condition by image tests. Its presence implies radical and multidisciplinar approach.

Presentation of cases: We report two female cases of 45 and 50 years old with clinical virilization and palpable mass on the abdominal right upper quadrant corresponding to adrenocortical carcinoma with hepatic fusion. The contrast-enhanced tomography showed an indistinguishable mass involving the liver and the right adrenal gland. In the first case, the patient had a two-time operation, the former removing only the adrenal carcinoma, and the second performing a radical surgery after an early relapse. In the second case, a radical right en bloc adrenohepatectomy was performed. Both cases were pathologically reported as liver-infiltrating adrenal carcinoma. Only in the second case the surgery was radical effective as first intention to treat, with 3 years of disease-free survival.

Discussion: ACC is a rare entity with poor prognosis. The major indicators of malignancy are tumour diameter over 6cm, local invasion or metastasis, secretion of corticosteroids, virilization and hypertension and hypokalaemia. The parenchymal fusion of the adrenal cortical layer can be misdiagnosed as hepatocellular carcinoma with adhesion with the Glisson capsule. AHF in such cases may be misinterpreted during surgery, what may impair its resectability, and therefore the survival. The surgical treatment must be performed en bloc, often using liver vascular control. Postoperative treatment must be offered immediately after surgery.

Conclusion: We report two consecutive rare cases of adrenohepatic fusion in giant right adrenocortical carcinoma, not detectable by imaging, what has important implications for the surgical decision-making. As radical surgery is the best choice to offer a curative treatment, it has to be performed by a multidisciplinary well-assembled team, counting with endocrine and liver surgeons, and transplant surgeons in case of vena cava involvement, in order to maximize the disease-free survival.

Fig. 1

CT scan comparing the two initial image studies. Both patients showed virilization symptoms and right lobe liver mass. Diagnosis was adrenal carcinoma. 1: First case. 2: Second case.

Fig. 2

First case. Large adrenal mass compressing right liver lobe and displacing vena cava and right renal vein. No thrombus shown. Right: on portal equilibrium phase, large areas of low attenuation coefficient, attributed to tumoral necrosis, surrounded by a hyperattenuated capsule.

Fig. 3

Second case. Large mass displacing but also merging with the liver. It can be seen IVC and right renal vein stenosis. The mass pulls out the diaphragm and compresses the hepatic veins, as also contacts with the right atrium, without invading IVC.

Fig. 4

Second case. Macroscopic picture of the adrenohepatic resection en bloc specimen.

Fig. 5

Second case. Anatomopathological macroscopical aspect of the AHF detailing infiltration line (black arrow).