A review of 218 pediatric cases of hepatocellular carcinoma

Allan, Bassan J.; Wang, Bo; Davis, James S.; Parikh, Punam P.; Pérez, Eduardo A.; Neville, Holly L.; Sola, Juan E.

doi:10.1016/j.jpedsurg.2013.09.050

Cited by 73 publications

(100 citation statements)

References 32 publications

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“…It was first described as a unique subtype of HCC by Edmondson in 1956 and was named FL‐HCC by Craig in 1980 . It accounts for less than 1% of all primary liver cancers, but represents the majority of HCCs in patients younger than 30 years of age . Due to the rarity of FL‐HCC and a lack of representative experimental systems, the molecular basis for carcinogenesis in these tumors has been elusive and difficult to study.…”

Section: Introductionmentioning

confidence: 99%

Fibrolamellar Hepatocellular Carcinoma: Mechanistic Distinction From Adult Hepatocellular Carcinoma

Riggle

Turnham

Scott

et al. 2016

Pediatric Blood & Cancer

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Fibrolamellar hepatocellular carcinoma (FL-HCC) has historically been classified as a rare subtype of HCC. However, unlike ‘classic’ HCC, it occurs in children and young adults without underlying liver disease. The recent discovery of a deletion mutation in all FL-HCCs represented a major advancement in understanding the pathogenesis of this disease. This deletion results in the fusion of the genes encoding a heat shock protein (DNAJB1) and the catalytic subunit of protein kinase A (PRKACA), and results in overexpression of PRKACA and enhanced cAMP-dependent PKA activity. This review summarizes recent advancements in FL-HCC pathogenesis and characteristics of the HSP40-PKA C protein.

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Section: Introductionmentioning

confidence: 99%

Fibrolamellar Hepatocellular Carcinoma: Mechanistic Distinction From Adult Hepatocellular Carcinoma

Riggle

Turnham

Scott

et al. 2016

Pediatric Blood & Cancer

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“…A study using the Surveillance, Epidemiology and End Results (SEER) database examined a cohort of pediatric patients with HCC from 1973 to 2009, and found an overall 5-year survival of 24% and 20-year survival of 8%4. The HCC tumor response in the pediatric population to the commonly used regimen of cisplatin/doxorubicin (PLADO) or cisplatin/5-fluorouracil/vincristine is only about 50%567.…”

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confidence: 99%

YAP Subcellular Localization and Hippo Pathway Transcriptome Analysis in Pediatric Hepatocellular Carcinoma

LaQuaglia

Grijalva

Mueller

et al. 2016

Sci Rep

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Pediatric hepatocellular carcinoma (HCC) is a rare tumor which is associated with an extremely high mortality rate due to lack of effective chemotherapy. Recently, the Hippo pathway and its transcriptional co-activator Yes-associated protein (YAP) have been shown to play a role in hepatocyte proliferation and development of HCC in animal models. Therefore, we sought to examine the activity of YAP and the expression of Hippo pathway components in tumor and non-neoplastic liver tissue from 7 pediatric patients with moderately differentiated HCC. None of the patients had underlying cirrhosis or viral hepatitis, which is commonly seen in adults with HCC. This highlights a major difference in the pathogenesis of HCC between children and adults. We found a statistically significant increase in YAP nuclear localization in 100% of tumors. YAP target gene (CCNE1, CTGF, Cyr61) mRNA expression was also increased in the tumors that had the most significant increase in YAP nuclear localization. Based on Ki67 co-localization studies YAP nuclear localization was not simply a marker of proliferation. Our results demonstrate a clear increase in YAP activity in moderately differentiated pediatric HCC, providing evidence that it may play an important role in tumor survival and propagation.

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“…FLHCC, a distinctive subtype of HCC, observed in this patient has a median age of diagnosis of 33 y compared to 66 y for the typical variant [4]. Despite its younger age of onset, the most common form in children and young adults is still typical variant.…”

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confidence: 75%