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

Abstract

HighlightsSince 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.

Giant Adrenocortical Carcinoma: A Case Report and Review of the Relevant Literature

“…The clinical presentation of adrenal cortical tumors depends on their size and hormonal status. Many non-functioning adrenal tumors are incidental findings and not always related to the patient’s clinical presentation, and are therefore called incidentalomas [ 8 ].…”

“…Non-functioning tumors can present with unspecific symptoms like abdominal pain, malaise, hematuria, and weight loss or no symptoms at all (incidental findings). Functioning tumors have a varied presentation, including Cushing’s syndrome, virilization, and feminization [ 8 ]. Our patient had non-specific symptoms, none of which were associated with hormone production (abdominal pain, discomfort, palpable abdominal mass).…”

“…In cases of obvious endocrine symptomatology there are a number of measurements that indicate the functionality of the mass. Dexamethasone suppression test and 24-hour urine cortisol sample are used in the presence of Cushing’s syndrome, while plasma estradiol or estrone is measured when feminization is present [ 5 , 8 ]. When an adrenal lesion is incidentally found, appropriate laboratory tests to be performed are electrolyte levels measurement in the presence of hypertension to rule out hyperaldosteronism, urinary metanephrines and catecholamines levels to exclude pheochromocytoma, and estrogen or androgen levels where relevant symptoms exist [ 8 ].…”

“…Dexamethasone suppression test and 24-hour urine cortisol sample are used in the presence of Cushing’s syndrome, while plasma estradiol or estrone is measured when feminization is present [ 5 , 8 ]. When an adrenal lesion is incidentally found, appropriate laboratory tests to be performed are electrolyte levels measurement in the presence of hypertension to rule out hyperaldosteronism, urinary metanephrines and catecholamines levels to exclude pheochromocytoma, and estrogen or androgen levels where relevant symptoms exist [ 8 ]. Our patient underwent an abdominal CT and MRI scan, in which the mass was found, and a full blood biochemical work-up including cortisol, aldosterone, renin, metanephrines, normetaphrines, and dehydroepiandrosterone level measurement.…”