Fibrolamellar Hepatocellular Carcinoma
Department of Gastroenterology, The First Affiliated Hospital of China Medical University, Shenyang 110001, Liaoning Province, China. The American Journal of Gastroenterology
(Impact Factor: 10.76).
08/2009; 104(10):2617-24; quiz 2625. DOI: 10.1038/ajg.2009.440
Fibrolamellar hepatocellular carcinoma (FHLCC) generally occurs in young individuals lacking a background of chronic liver disease and other risk factors for hepatocellular carcinoma. The clinical presentations of FLHCC are generally nonspecific, and the alpha-fetoprotein level is typically within the normal range in most cases. Imaging studies have a major role in clinical diagnosis, but pathology is the gold standard in confirming diagnosis. Pathological characteristics of FLHCC include the presence of tumor cells with a deeply eosinophilic cytoplasm and macronucleoli surrounded by abundant fibrous bands. The most effective treatment for FLHCC is aggressive surgical resection. This comprehensive literature review gives a full account of the clinical, pathological, and molecular features of FLHCC.
Available from: S. Chaouir
- "was hypointense in T1-and T2-weighted images. However, delayed enhancement of the scar was unusual  . Capsular retraction adjacent to a hepatic tumour is very suggestive for malignancy, being associated with epithelioid hemangioendotheliomas, intrahepatic cholangiocarcinomas and breast cancer liver metastases  . "
Available from: Justin G Peacock
- "Fibrolamellar hepatocellular carcinoma (FLHCC), a rare variant of hepatocellular carcinoma (HCC) comprising 1-2% of the US HCC cases. FLHCC was first described by Edmondson in 1956;1,2 it histologically consists of polygonal, eosinophilic hepatocyte cords separated by bands of fibrous stroma.1,3 FLHCC tumors typically present with symptoms of abdominal pain, fullness, and nausea.1,3 "
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ABSTRACT: Fibrolamellar hepatocellular carcinoma (FLHCC) is a rare variant of hepatocellular carcinoma (HCC) that commonly affects young individuals without a prior history of liver disease. FLHCC commonly results in a better prognosis than HCC; however, the risk of recurrence and metastatic disease is high. FLHCC is typically treated by primary resection of the tumor with 50-75% cure rates. The use of radiation therapy in FLHCC has not been assessed on its own, and may show some success in a very few reported combination therapy cases. We report on the successful use of radiation therapy in a case of metastatic FLHCC to the lung following primary and secondary resections. Our treatment of the large, metastatic, pulmonary FLHCC tumor with 40 Gy in 10 fractions resulted in an 85.9% tumor volume decrease over six months. This suggests FLHCC may be a radiosensitive tumor and radiotherapy may be valuable in unresectable or metastatic tumors.
Available from: PubMed Central
- "Subsequent case reports and cases series have confirmed and expanded the unique clinical and pathologic features of FLC. Major review articles on the clinical, pathological, and biological findings have been published in 2007 , 2009 , and 2011 . "
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ABSTRACT: 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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