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Paper 4
Published in Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw, The Final FRCR, 2020
Amanda Rabone, Benedict Thomson, Nicky Dineen, Vincent Helyar, Aidan Shaw
Fibrolamellar HCC is an uncommon variant of HCC affecting young patients with no risk factors. The tumour is typically large at diagnosis. The central scar is usually hyperechoic on ultrasound and hypointense on MRI sequences, which helps to differentiate it from the T2 hyperintense central scar in focal nodular hyperplasia (FNH). The central scar generally does not enhance in fibrolamellar HCC but does in FNH.
Pathology and Epidemiology
Published in John T. Kemshead, Pediatric Tumors: Immunological and Molecular Markers, 2020
The identification of tumor subtypes with a better prognosis than the rest of their class has also been seen with another form of liver tumor, namely hepatocellular carcinoma. A morphologically clearly distinguishable form of hepatocellular carcinoma, the fibrolamellar carcinoma,13 has been identified as being particularly prone to develop in older children and young adults. Unlike other forms of hepatocellular carcinoma, this tumor occurs in livers not predisposed to tumor formation by factors such as cirrhosis. Fibrolamellar carcinomas are also, on the whole, more amenable to complete surgical resection and are commonly associated with considerably longer patient survival times.
Primary tumours of the liver and biliary tract
Published in Anju Sahdev, Sarah J. Vinnicombe, Husband & Reznek's Imaging in Oncology, 2020
Hero K Hussain, Mohammad Abu Shattal
On imaging, non-cirrhotic HCC has features similar to classic HCC without the background of cirrhosis. Non-cirrhotic HCC often presents as a large solitary mass or a dominant mass with satellite lesions. Tumours can be encapsulated or poorly circumscribed. Variable degrees of necrosis, haemorrhage, macroscopic fat, and upstream intrahepatic biliary dilatation can be seen (134). Direct extrahepatic extension of HCC or metastasis is more common in non-cirrhotic HCC, with abdominal nodal enlargement seen in up to 21% of cases (135). Specifically, the fibrolamellar subtype tends to develop nodal and peritoneal metastasis (135,136). Tumour thrombus can occasionally be seen in the portal or less commonly in the hepatic veins (137).
Pathologic and Immunophenotypic Characterization of Syncytial Giant Cell Variant of Pediatric Hepatocellular Carcinoma. A Distinct Subtype
Published in Fetal and Pediatric Pathology, 2023
Mukul Vij, Jagadeesh Menon, Komalavalli Subbiah, Lexmi Priya Raju, Gowripriya Gowrisankar, Naresh Shanmugum, Ilankumaran Kaliamoorthy, Ashwin Rammohan, Mohamed Rela
HCCs predominately occur in adults. The frequency of pHCC is only 0.5% to 1% and a majority occur in older children (10 to 14 years of age) [9]. The outcomes of inoperable pHCC remain dismal with a five-year event-free survival of only 8% [10, 11]. Complete surgical resection or LT remain the treatment of choice and are associated with a significant improvement in survival (>50% at 5 y) [12]. Several histological subtypes of HCC have been reported in adults [13]. In children, only a few HCC subtypes have been described. The recent WHO classification of pediatric liver tumors distinguishes fibrolamellar and non-fibrolamellar HCC (including conventional, clear cell variants) [14, 15]. Fibrolamellar HCC is a distinct clinical and histological variant representing 25% of all HCCs diagnosed in children [16]. Conventional growth pattern is reported in 73%, and clear cell variant in 2% of HCC cases in children [16]. Scirrhous proliferation pattern has been reported [17]. Our review of pHCC identified conventional HCC with trabecular architecture and clear cell subtype of HCC [2, 6, 18]. Only two cases of pHCC with syncytial giant cells have been reported (“vide infra”) [19, 20]. Tumors which demonstrate pathological features of both hepatoblastoma and HCC are now considered a variant of hepatoblastoma, and are very aggressive [21].
Pediatric fibrolamellar hepatocellular carcinoma: case report and review of the literature
Published in Acta Chirurgica Belgica, 2021
Laura Depauw, Glenn De Weerdt, Ben Gys, Sofie Demeulenaere, Wouter Mebis, Dirk Ysebaert
Macroscopically FL-HCC usually presents as a single, scirrhous, white-brownish, slow-growing and well-circumscribed mass (Figure 2(a)). Microscopically it shows polygonal cells with large nucleoli and eosinophilic cytoplasm, which contains intracytoplasmic pale bodies. Thick fibrous collagen bands encircle the neoplastic hepatocytes (Figure 2(b)) [11]. The neoplastic cells in the fibrolamellar variant do not produce α-fetoprotein (AFP). They do commonly express biliary markers, including cytokeratin CK7 and CK19, and molecular markers such as epithelial membrane antigen (EMA), monoclonal carcinoembryonic antigen (mCEA), cancer antigen 19 (CA19) and epithelial cell adhesion molecule (EpCAM). This suggests that FL-HCC could be derived from a precursor cell with the ability to differentiate into hepatocytes and biliary cells and that the underlying carcinogenetic process differs from that in conventional HCCs, where serum AFP is increased and the biliary-type cytokeratin CK7 is only rarely expressed [13,14].
A classification based on tumor budding and immune score for patients with hepatocellular carcinoma
Published in OncoImmunology, 2020
Li Wei, Zhang Delin, Yuan Kefei, Wu Hong, Huang Jiwei, Zhang Yange
Clinical features including age, sex, AFP level, tumor stage, background liver cirrhosis, and MVI showed no significant differences between high and low-grade tumor budding groups (Table S4). In Figure S2 and Figure 3(a), we showed the representative HE images of different pathologic patterns including tumor architectural pattern, stromal features, and EMT-related markers. As expected, high-grade tumor budding was significantly associated with high vimentin (P = .002) and low E-cadherin (P = .005) expressions (Figure 3(b)). Interestingly, the rate of high-grade tumor budding was significantly higher in tumors with immature stroma (P < .001) and strong α-SMA expression (P = .005) (Figure 3(c)). In addition, high-grade tumor budding was most frequently identified in non-steatotic tumors (P < .001) (Figure 3(d)). Fibrolamellar-HCC exhibited less frequent high-grade tumor budding than other tumors (P < .001) (Figure 3(d)). However, tumor budding was not related to tumor differentiation (P = .344), pseudoglandular (P = .954) and trabecular pathologic patterns (P = .799) (Figure 3(d)).