An 88-Year-Old Woman with a 33-Year History of Idiopathic Portal Hypertension Presenting with Hepatocellular Carcinoma Treated with Carbon-Ion Radiotherapy

Abstract

BACKGROUND Idiopathic portal hypertension (IPH) is a rare disease of unknown etiology that causes hypersplenism, splenomegaly, and portal hypertension. There have been rare reports of hepatocellular carcinoma (HCC) in patients with IPH, but no causal relationship has been confirmed. This report details the case of an 88-year-old Japanese woman who developed HCC after a 30-year history of IPH and was treated with carbon-ion radiotherapy. CASE REPORT An 88-year-old Japanese woman had presented to our hospital 33 years earlier with bleeding from esophageal varices. Liver function test results were normal. Computed tomography (CT) showed marked splenomegaly. She had no known causative factors for liver disease, and IPH was suspected. Endoscopic injection sclerotherapy was performed repeatedly for episodes of bleeding from esophageal varices until 4 years after presentation, when she underwent Hassab's procedure. A liver biopsy showed preserved lobular architecture and moderate fibrous enlargement of the portal area without necro-inflammatory reaction. She had a stroke 18 years later and was started on clopidogrel. Nine years later, CT revealed a 24-mm HCC in S8, and portal vein thrombosis (PVT). Carbon-ion radiotherapy was administered, followed by edoxaban. Three months later, CT showed shrinkage of the HCC and complete resolution of the PVT. Almost 3 years later, CT showed no recurrence of HCC or PVT. CONCLUSIONS We report a rare case of IPH and HCC co-existing in a patient followed up for more than 30 years. Although there is no recognized association between IPH and HCC, this report highlights the importance of continued clinical follow-up of patients with chronic liver disease.

Figure 1. Histological features of a liver specimen obtained 26 years before the onset of hepatocellular carcinoma

(A) Photomicrograph showing a rounded portal tract expanded by dense stromal fibrosis (arrow: portal vein) (Masson trichrome staining). (B) Photomicrograph showing focal dilatation of the sinusoids with poor evidence of hepatocellular inflammation in the portal region or parenchyma (hematoxylin and eosin).

Figure 2. Computed tomography (CT) images obtained at the onset of hepatocellular carcinoma (HCC)

CT image showing a round, slightly irregular, 24-mm-diameter nodule (arrow) in segment 8 just below the hepatic dome that is (A) enhanced in the arterial phase and (B) washed out in the equilibrium phase. These findings are consistent with HCC. (C) No thrombus is evident in the portal vein in the portal venous phase.

Figure 3. Computed tomography images obtained 4 months after the diagnosis of hepatocellular carcinoma (HCC)

The HCC increased to 30 mm in diameter (arrow) in (A) the arterial phase and (B) the equilibrium phase. (C) A thrombus is evident in the main trunk of the portal vein (arrow) in the portal venous phase.

Figure 4. Computed tomography images obtained 3 years 10 months after carbon-ion radiation therapy

The liver is atrophied and deformed, but no tumor is detected in or near the area where the tumor had previously been located in (A, C) the arterial phase and (B, D) the equilibrium phase. (E) The portal vein thrombus has completely resolved in the portal venous phase.