Imaging liver nodules in tyrosinemia type-1: A retrospective review of 16 cases in a tertiary pediatric hospital. Issue 116 (July 2019)

• Record Type: Journal Article
• Authors: Ozcan, H. Nursun; Karcaaltincaba, Musturay; Pektas, Emine; Sivri, H. Serap; Oguz, Berna; Dursun, Ali; Tokatli, Aysegul; Coskun, Turgay; Haliloglu, Mithat
• Is Part Of: European journal of radiology. Issue 116(2019)
• Page Start: 41
• Page End: 46
• Publication Date: 2019-07
• Subjects: HT1 hereditary tyrosinemia type-1 -- AFP alpha feto protein -- FAH fumarylacetoacetate hydrolase -- SA succinylacetone -- NTBC 2-(2-nitro-4-trifluoromethylbenzoyl)-1, 3-cyclohexanedione -- HCC hepatocellular carcinoma -- CT computed tomography -- MRI magnetic resonance imaging; Liver -- Tyrosinemia type 1 -- Magnetic resonance imaging; Medical radiology -- Periodicals; Radiology -- Periodicals; Radiologie médicale -- Périodiques; Medical radiology; Periodicals; 616.075705
• DOI: 10.1016/j.ejrad.2019.04.016
• Languages: English
• ISSNs: 0720-048X

Abstract

Highlights: Fatty liver nodules might be observed in HT1 patients and they can display morphological changes during the follow up. Every liver nodule might not always/necessarily be HCC, close follow up -preferably with MR imaging- should be performed in addition to clinical and laboratory findings where HCC is suspected. Alpha-fetoprotein (AFP) values and liver imaging should be carried out periodically for early detection of HCC in HT1 patients. Abstract: Objective: To describe the liver imaging findings of Hereditary tyrosinemia type-1 (HT1) patients. Materials and methods: We report 16 patients (8 Female and 8 Male) with HT-1. Their demographic features, imaging findings and alpha feto protein (AFP) levels were recorded. Imaging features on CT and MR were evaluated for the following characteristics: contour of the liver and liver nodules. Liver nodules were categorized as; regenerative, dysplastic, fatty and malignant nodules (HCC). Results: Thirteen (81%) patients had multiple liver nodules (>20) on imaging studies. Five patients (31%) had regenerative nodules, six (38%) had dysplastic nodules and ten (63%) had fatty nodules. Dysplastic nodules were encountered in two patients with HCC and in four patients without a tumor. Four patients (25%) had HCC nodule on imaging studies. Those four patients had biopsy and all of them had HCC nodule on histopathology. In the follow-up period, in one patient fatty nodules had increased in size, in one patient regenerative nodules had disappeared and in one patient dysplastic nodules had disappeared. Conclusions: Multiple fatty nodules can be seen in HT1 patients and in some patients, the regenerative and dysplastic nodules can disappear during the follow-up period.