Total hepatectomy and liver transplant for hepatocellular adenomatosis and focal nodular hyperplasia

Abstract

Extensive hepatocellular adenomatosis (HA) and focal nodular hyperplasia (FNH) represent a proliferation of hepatic cells that occurs most frequently in women. These lesions are uncommon in the pediatric age group, accounting for 2% of pediatric hepatic tumors, and are extremely rare in males. The etiology of HA and FNH has been correlated with the use of oral contraceptives. We report to the best of our knowledge the first series of patients treated with OLTx for HA and FNH (five cases). All these patients had lesions involving at least 90% of the hepatic parenchyma and all underwent major hepatic surgery before OLTx because of life threatening complications. One patient died in the immediate postoperative period following retransplantation for primary non-function of the first OLTx. Four out of five patients are currently alive from 4.1 to 9.6 years after OLTx. Our results justify the use of OLTx for symptomatic patients with HA and FNH who cannot be treated with conventional hepatic resections.

Fig. 1

Pre OLTx CT scan, case no. 1. Multiple hepatic masses occupying almost all the liver parenchyma

Fig. 2

Case no. 1, recipient liver (a portion has been removed for biochemical study). Multiple nodules distorting the surface are clearly discernible

Fig. 3

Pre OLTx CT scan, case no. 3. Surgical clips (related to previous left and right segmental resection) can be noted in the liver. Inhomogenicity with multiple low density areas can be noted throughout all lobes of the liver

Fig. 4

Pre OLTx CT scan, case no. 4. Multiple hepatic mass lesions of various sizes are spread throughout the entire liver

Fig. 5

Case no. 4. Cross-section of the recipient liver. The parenchyma is in most part replaced by circumscribed, irregular nodules ranging from 0.2 to 7.0 cm

Fig. 6

Pre OLTx CT scan, case no. 5. A large hypodense lesion is demonstrated involving the entire left hepatic lobe with extension into the anterior segment of the right lobe. There is an additional smaller round lesion in the posterior segment of the right lobe