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

• Since its first description, by Dolan, in 1968, called adrenal dystopia, described as the union between the adrenal gland and the liver parenchyma, the adreno-hepatic fusion (AHF) counts on less than 90 articles in PubMed.
• In our experience, in spite of a little known entity, AHF may be a primary pathological entity that largely determines the surgical behavior, which in our experience results in terms of long-term and disease-free survival for the patient.
• The absence of capsulla between the liver and the adrenal gland means that the lesion must be resected as a whole, even with right hepatectomy, in order to extirpate all the microscopic intermingling between the two parenchymas.
• This case report underlines the importance of the detection of adrenohepatic fusion before the intervention, whose presence may compromise both resectability and survival.
• We present two consecutive cases of adrenal carcinoma with AHF treated in a reference unit of liver surgery, emphasizing the key points that helped improve the disease-free survival.

IntroductionAdrenohepatic 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 casesWe 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.DiscussionACC is a rare entity with poor prognosis. The major indicators of malignancy are tumour diameter over 6 cm, 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.ConclusionWe 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.