Abstract
Fibrolamellar hepatocellular carcinoma (FL-HCC) is generally a fairly rare event in routine pathology practice. This variant of hepatocellular carcinoma (HCC) is peculiarly intriguing and,in addition, poorly understood. Young people or children are often the target individuals with this type of cancer. Previously, I highlighted some pathology aspects of FL-HCC, but in this review, the distinctive clinico-pathologic features of FL-HCC and the diagnostic pathologic criteria of FL-HCC are fractionally reviewed and expanded upon. Further, molecular genetics update data with reference to this specific tumor are particularly highlighted as a primer for general pathologists and pediatric histopathologists. FL-HCC may present with metastases, and regional lymph nodes may be sites of metastatic spread. However, peritoneal and pulmonary metastatic foci have also been reported. To the best of our knowledge, FL-HCC was initially considered having an indolent course, but survival outcomes have recently been updated reconsidering the prognosis of this tumor. Patients seem to respond well to surgical resection, but recurrences are common. Thus, alternative therapies, such as chemotherapy and radiation, are ongoing. Overall, it seems that this aspect has not been well-studied for this variant of HCC and should be considered as target for future clinical trials. Remarkably, FL-HCC data seem to point to a liver neoplasm of uncertain origin and unveiled outcome. A functional chimeric transcript incorporating DNAJB1 and PRKACA was recently added to FL-HCC. This sensational result may give remarkable insights into the understanding of this rare disease and potentially provide the basis for its specific diagnostic marker. Detection of DNAJB1-PRKACA seems to be, indeed, a very sensitive and specific finding in supporting the diagnosis of FL-HCC. In a quite diffuse opinion, prognosis of this tumor should be reconsidered following the potentially mandatory application of new molecular biological tools.
Highlights
Fibrolamellar hepatocellular carcinoma (FLC/FL-HCC) is usually a fairly rare variant of hepatocellular carcinoma (HCC)
A functional chimeric transcript incorporating DNAJB1 and PRKACA was recently added to FL-HCC
Radiological experience and databases demonstrate that the FL-HCC scar is often calcified, an important hint, which is uncommonly to be observed with focal nodular hyperplasia (FNH)
Summary
Fibrolamellar hepatocellular carcinoma (FLC/FL-HCC) is usually a fairly rare variant of hepatocellular carcinoma (HCC). The target population is not limited to youth only, but childhood and, commonly, adolescents are affected by this tumor The epidemiology of this type of cancer shows that both genders are involved and Diagnostics 2016, 6, 3; doi:10.3390/diagnostics6010003 www.mdpi.com/journal/diagnostics. FL-HCC has, a peculiar lamellar fibrosis, i.e., a fibrosis having a substantial thickness, which is considered remarkable for this tumor [1] This kind of fibrosis occurs without the patients presenting liver cirrhosis or underlying liver disease. Vitamin B12 binding capacity (characteristically labelled and known as transcobalamin), and plasmatic γ-carboxy-prothrombin are more often considered laboratory markers for FL-HCC. These markers have been associated with disease burden [8,9,10,11,12]. Radiological experience and databases demonstrate that the FL-HCC scar is often calcified, an important hint, which is uncommonly to be observed with FNH
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