Abstract
Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant systemic vascular disease. In this report, we present a case of HHT accompanied by focal nodular hyperplasia (FNH) with long-term follow-up. The patient was a woman in her 60s with no chief complaints, but she had recurrent nosebleeds since childhood. She was referred to our hospital in 2006 after an intrahepatic mass was suspected at another hospital. She had a family history of epistaxis. On admission, hepatitis virus and tumor markers were negative. Abdominal ultrasonography (US) revealed a prominent portal vein, dilated hepatic vein, coiled hepatic duct dilatation, multiple shunts, and multiple masses throughout the liver. Upon performing color Doppler imaging, the largest tumor (S8) showed a spoke-wheel-pattern signal. Dynamic computed tomography also revealed similar vascular abnormalities. The tumor was enhanced in the early phase, but no washout was observed. A biopsy of the tumor confirmed FNH. In 2007, Sonazoid-enhanced US of the largest mass showed enhancement in the early phase, but no defect was noted in the late phase. On annual examinations, vascular lesions remained unchanged, but the largest mass showed regression on US and gadolinium-ethoxybenzyl-diethylenetriamine pentaacetic acid-enhanced magnetic resonance imaging (EOB-MRI) and disappeared in 2015. After its disappearance, EOB-MRI detected emergence and regression of other masses, but not US. However, a new mass was confirmed by US in 2022. Sonazoid contrast-enhanced US was performed using a full-focus device, and a mass that could not be observed previously on B-mode US could now be observed simultaneously. We performed long-term follow-up of HHT accompanied by multiple FNH. Sonazoid-enhanced US and EOB-MRI are useful imaging modalities for multiple evolving tumors.
