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Outcome of patients with fibrolamellar hepatocellular carcinoma
Article first published online: 13 FEB 2006
Copyright © 2006 American Cancer Society
Volume 106, Issue 6, pages 1331–1338, 15 March 2006
How to Cite
Stipa, F., Yoon, S. S., Liau, K. H., Fong, Y., Jarnagin, W. R., D'Angelica, M., Abou-Alfa, G., Blumgart, L. H. and DeMatteo, R. P. (2006), Outcome of patients with fibrolamellar hepatocellular carcinoma. Cancer, 106: 1331–1338. doi: 10.1002/cncr.21703
- Issue published online: 2 MAR 2006
- Article first published online: 13 FEB 2006
- Manuscript Accepted: 29 SEP 2005
- Manuscript Revised: 1 SEP 2005
- Manuscript Received: 16 JUN 2005
- hepatocellular carcinoma;
- hepatic resection;
Fibrolamellar hepatocellular carcinoma (FL-HCC) is a rare variant of hepatocellular carcinoma, has distinct pathologic features, and typically occurs in young patients without underlying hepatitis or cirrhosis.
Forty-one patients with the pathologic diagnosis of FL-HCC evaluated at our institution between 1986 and 2003 were identified from a prospective database.
Median age of all patients was 27 years. None of these patients had underlying hepatitis or cirrhosis, and only 3 (7%) patients had an α-fetoprotein level > 200 ng/mL. Twenty-eight patients with primary disease underwent complete gross resection, and 13 patients were unresectable. In patients treated with resection, median tumor size was 9 cm (range, 3–17), 9 (36%) had vascular invasion, and 14 (50%) had lymph node metastases. There were no perioperative deaths. With a median follow-up of 34 months, 5-year overall survival for resected patients was 76%. However, 5-year recurrence-free survival was only 18%, and of the 9 resected patients with more than 5 years of follow-up, 7 had recurrences. Lymph node metastasis was the only significant negative prognostic factor. Seventeen (61%) patients underwent a second operation for recurrent disease. Median survival for unresected patients with FL-HCC was only 12 months, and no patient survived beyond 5 years.
FL-HCC occurs in a distinctly different population of patients than common HCC, and patients with FL-HCC generally fare better after complete resection. These tumors have a relatively indolent tumor biology, and late recurrences are common. Repeat resections for recurrence should be considered given the lack of other effective treatment options. Cancer 2006. © 2006 American Cancer Society.