Fibrolamellar hepatocellular carcinoma in Mexican patients

Arista-Nasr, Julián; Gutierrez-Villalobos, Lisa; Nuncio, Juan; Maldonaldo, Hector; Bornstein-Quevedo, Leticia

doi:10.1007/bf03033723

Cited by 30 publications

(18 citation statements)

References 21 publications

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“…Alkaline phosphatase levels may be elevated [10, 32, 34], occasionally with levels greater than 1000 IU/mL [23], findings that most likely reflect growth into the biliary tree or obstruction of the biliary tree. Alpha-fetoprotein (AFP) levels are typically normal [35].…”

Section: Clinical Findingsmentioning

confidence: 99%

Fibrolamellar Carcinoma: 2012 Update

Torbenson

2012

Scientifica

146

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Fibrolamellar carcinomas are a unique type of primary liver cancer. They occur most commonly in children and young adults. Their etiology remains a mystery, as they are not associated with chronic liver disease. Fibrolamellar carcinomas are not indolent tumors, but have an overall better prognosis than typical hepatocellular carcinomas, in part because of the younger age at presentation and the lack of cirrhosis. The most important prognostic feature is whether the tumor is resectable. Histologically, the tumor is made up of large cells that contain abundant mitochondria. The nuclei of the tumor cells have prominent nucleoli. The tumor cells induce the formation of extensive intratumoral fibrosis, which often grows in parallel, or lamellar bands. The tumor cells clearly show hepatocellular features but are also unique in showing both biliary and neuroendocrine differentiation. The uniqueness of fibrolamellar carcinoma extends to their molecular findings. While the genetic abnormalities that lead to fibrolamellar carcinomas are not yet known, studies have shown that they lack mutations in the genes most commonly mutated in typical hepatocellular carcinoma (TP53 and CTNNB1). In this paper, the clinical, pathological, and basic science literature on fibrolamellar carcinoma is comprehensively reviewed. Key areas of needed research are also discussed.

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Section: Clinical Findingsmentioning

confidence: 99%

Fibrolamellar Carcinoma: 2012 Update

Torbenson

2012

Scientifica

146

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“…FLC occurs in younger individuals, even in children in non-cirrhotic liver. Incidence varies by geographical regions between 1% and 5%, with the rate reported to be 1-2% of total HCC cases in the USA [7,8] and much higher in Mexico [9] and Poland [10].…”

Section: Introductionmentioning

confidence: 99%

Claudins and tricellulin in fibrolamellar hepatocellular carcinoma

et al. 2011

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Fibrolamellar hepatocellular carcinoma is a subtype of hepatocellular carcinoma occurring in non-cirrhotic liver at a younger age. The tumor expresses both hepatocellular and cholangiocellular markers. Previously, our group described overexpression of tight junction protein claudin 4 in cholangiocellular carcinoma in contrast to hepatocellular carcinoma. In the present study, tight junction protein expressions were studied to possibly clarify bipotential lineage of fibrolamellar hepatocellular carcinoma. Eleven fibrolamellar hepatocellular carcinomas were compared with seven "conventional" hepatocellular carcinomas, seven cholangiocellular carcinomas, and five normal liver samples. By immunohistochemistry, all fibrolamellar hepatocellular carcinomas were positive for HepPar1 and cytokeratins 7, 8, and 18, but negative for cytokeratin 19. Glypican-3 gave weak staining in two cases. Expression of claudin 1 was lower, while that of claudin 2 was higher in fibrolamellar hepatocellular carcinomas than in other tumors. Claudins 3, 4, and 7 were not detectable in fibrolamellar hepatocellular carcinomas as in the majority of "conventional" hepatocellular carcinomas, contrary to high expression observed in cholangiocellular carcinomas. Focal or diffuse claudin 5 expression was detected in nine of 11 fibrolamellar hepatocellular carcinomas contrary to other tumors. Tricellulin was significantly downregulated in all tumors compared with normal liver. Our findings showed claudins to exhibit specific expression patterns in fibrolamellar hepatocellular carcinomas not observed in other primary liver tumors, with unique claudin 5 expression and pattern features similar to common hepatocellular carcinoma, but different from cholangiocellular carcinoma. This is the first report describing the loss of tricellulin expression in human hepatic tumors.

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“…Abdominal pain and malaise are the most common complaints in FLH; hepatomegaly or an abdominal mass is sometimes noted, and jaundice is occasionally found. Many patients also suffer from multiple signs and symptoms such as fever, diarrhea, and weight loss [1,8]. FLH often exhibits aggressive behavior, invading the main portal vein, the proper hepatic artery, the central intrahepatic bile ducts, or other adjacent organs, and/or presenting with extrahepatic lymph node or distant metastases [2,3,9].…”

Section: Discussionmentioning

confidence: 99%

“…When the stomach is compressed by a malignant tumor, liver metastatic tumors and conventional HCC represent the most common causes of external compression of the anterior wall of the gastric body [11]. There are very few reports describing dyspepsia resulting from gastric compression in FLH [3,8]. However, clinicians should take into consideration the existence of this unusual presentation, which can mimic a functional disorder of the stomach, to facilitate prompt identification of the tumor.…”

Section: Discussionmentioning

confidence: 99%

An unusual cause of gastric extraluminal compression: fibrolamellar hepatocellular carcinoma

et al. 2013

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AbstractFibrolamellar hepatocellular carcinoma (FLH) is a rare variant of hepatocellular carcinoma that typically occurs in adolescents or young subjects in an otherwise normal liver. Correct recognition of FLH is extremely important, because a complete resection of the tumor can confer an excellent prognosis. FLHs can become large, exerting mass effects on adjacent organs before diagnosis. Unfortunately, there are very few imaging reports describing external compression of the stomach caused by a FLH. We report the case of a young female suffering from dyspepsia and abdominal pain caused by stomach compression resulting from a large FLH.

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Fibrolamellar hepatocellular carcinoma in Mexican patients

Abstract: fibrolamellar hepatocarcinoma is an uncommon, but not an exceptional neoplasm in our population and represents 5.8% of all hepatocarcinomas reviewed.

Cited by 30 publications

References 21 publications

Fibrolamellar Carcinoma: 2012 Update

Fibrolamellar Carcinoma: 2012 Update

Claudins and tricellulin in fibrolamellar hepatocellular carcinoma

An unusual cause of gastric extraluminal compression: fibrolamellar hepatocellular carcinoma

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