title: Fibrolamellar variant of hepatocellular carcinoma.
abstract: The fibrolamellar variant of hepatocellular carcinoma is a rare primary liver cancer occurring in adolescents and young adults without chronic liver disease or known risk factors. Histologically, it is defined by lamellar bands of fibrosis surrounding well-differentiated tumor cells. Radiologic imaging typically demonstrates a large, solitary mass with calcifications and a central scar. Lymph node metastases in the porta hepatis are frequently diagnosed upon presentation. More patients with fibrolamellar carcinoma are candidates for surgical resection than those with conventional hepatocellular carcinoma, owing to their young age and absence of cirrhosis. The most important prognostic factor is surgical resection, which results in 5-year overall survival rates ranging between 50 and 76 %. Despite complete surgical resection, relapse rates are high, and novel therapies are needed to prevent and treat recurrent disease.
authors.affiliation: Department of Surgical Oncology, Fox Chase Cancer Center, Philadelphia, PA 19111, USA. ys475@yahoo.com
authors.forename: Yun Shin
authors.initials: YS
authors.lastname: Chun
authors.forename: Giuseppe
authors.initials: G
authors.lastname: Zimmitti
mesh_terms: D000293:Adolescent; D006528:Carcinoma, Hepatocellular / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000401:mortality / Q000601:surgery; D006498:Hepatectomy; D006801:Humans; D008113:Liver Neoplasms / Q000175:diagnosis / Q000000981:diagnostic imaging / Q000401:mortality / Q000601:surgery; D008207:Lymphatic Metastasis; D009364:Neoplasm Recurrence, Local; D011379:Prognosis; D011859:Radiography; D015996:Survival Rate; D055815:Young Adult