Abstract
BackgroundFibrolamellar hepatocellular carcinoma (FL-HCC) is a liver tumor that occurs almost exclusively in young adults without underlying liver disease. In spite of its distinct clinical characteristics and specific imaging findings, preoperative diagnosis is often difficult due to the extremely low incidence of the tumor. Although FL-HCC shows particular morphological features on H&E-stained tissue sections, differential diagnosis from ordinary HCC, especially the scirrhous variant of HCC, and intrahepatic cholangiocarcinoma needs additional immunohistochemical (IHC) analyses and/or molecular genetic testing.Case presentationA 21-year-old male patient was referred to our hospital for further evaluation of a large liver mass. Abdominal ultrasound examination, contrast-enhanced computed tomography, and magnetic resonance imaging revealed a well-defined hypervascular lobulated liver mass, 11 × 11 cm in diameter, with a central scar and calcification, in segments 5/8. Under the diagnosis of FL-HCC, we carried out extended anterior sectorectomy, including a part of segment 4. On microscopic examination, the tumor was composed of proliferating polygonal cells with abundant eosinophilic granular cytoplasm containing nuclei with vesicular chromatin and enlarged nucleoli, in an abundant stroma. Collagen fibers arranged in a parallel lamellar pattern were seen in the tumor stroma. These findings, together with the results of subsequent IHC analyses using HAS, CK7, and CD 67, we made the diagnosis of FL-HCC, which was further confirmed by detection of the DNAJB1-PRKACA fusion gene in the tumor cells by RT-PCR.ConclusionFL-HCC shows distinct imaging appearances. Although it also has characteristic morphological features, combined use of IHC and/or molecular genetic studies are necessary for the final diagnosis.
Highlights
Fibrolamellar hepatocellular carcinoma (FL-HCC) is a liver tumor that occurs almost exclusively in young adults without underlying liver disease
FL-HCC shows distinct imaging appearances. It has characteristic morphological features, combined use of IHC and/or molecular genetic studies are necessary for the final diagnosis
We present the case of a 21-year-old man, who we diagnosed preoperatively as having FL-HCC based on the findings of abdominal ultrasound (US), computed tomography (CT), and magnetic resonance imaging (MRI)
Summary
Fibrolamellar hepatocellular carcinoma (FL-HCC) was first described in 1956 by Edmondson [1], and named as FL-HCC by Craig in 1980 [2]; it was not until. Despite its distinct clinical characteristics, i.e., predominant occurrence in adolescents and young adults (ages between 5 and 40 years) who do not have underlying liver disease [4], preoperative diagnosis of FL-HCC is often difficult because of its extremely low incidence, with an estimated age-adjusted incidence rate of 0.02 per 100,000 in the United States [5]. This aspect becomes further emphasized in countries including. He has been under follow-up at our outpatient clinic, without showing evidence of recurrence until now, 17 months since the surgery
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