Fibrolamellar Carcinoma: A Concise Review

Archives of Pathology & Laboratory Medicine
Chun-Chieh Lin, Hui-Min Yang

Abstract

Fibrolamellar carcinoma is a rare primary hepatocellular malignancy arising in noncirrhotic livers of young individuals. Patients commonly present with a large solitary liver mass and nonspecific symptoms. Characteristic histologic features include large polygonal cells with oncocytic cytoplasm and prominent nucleoli separated into trabeculae and cords by dense parallel bands of collagen. Important differential diagnoses include classical hepatocellular carcinoma and intrahepatic cholangiocarcinoma, which may be distinguished by a judicious panel of immunohistochemical studies, including cytokeratin 7, CD68, and hepatocyte paraffin 1 (HepPar-1). In addition, fibrolamellar carcinomas are characterized by activation of protein kinase A. Prognosis of fibrolamellar carcinoma is similar to classical hepatocellular carcinoma occurring in the absence of liver cirrhosis and is strongly correlated with tumor resectability. Other treatment options include liver transplant, chemotherapy, and hepatic artery embolization. In this article, we review the clinical features, gross and microscopic pathology, molecular genetics, differential diagnosis, treatment, and prognosis of this rare and interesting tumor.

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Citations

Nov 5, 2019·Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc·Monika VyasOlca Basturk
Jun 28, 2019·Journal of Gastrointestinal Surgery : Official Journal of the Society for Surgery of the Alimentary Tract·Ayesha FarooqTimothy M Pawlik
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Sep 21, 2021·American Journal of Clinical Pathology·Anisha JainXuchen Zhang

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