Outcome of patients with fibrolamellar hepatocellular carcinoma Journal Article
| Authors: | Stipa, F.; Yoon, S. S.; Liau, K. H.; Fong, Y.; Jarnagin, W. R.; D'Angelica, M.; Abou-Alfa, G.; Blumgart, L. H.; DeMatteo, R. P. |
| Article Title: | Outcome of patients with fibrolamellar hepatocellular carcinoma |
| Abstract: | BACKGROUND. 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. METHODS. Forty-one patients with the pathologic diagnosis of FL-HCC evaluated at our institution between 1986 and 2003 were identified from a prospective database. RESULTS. 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. CONCLUSIONS. 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. © 2006 American Cancer Society. |
| Keywords: | adolescent; adult; cancer survival; clinical article; treatment outcome; aged; middle aged; survival rate; clinical feature; liver cell carcinoma; carcinoma, hepatocellular; liver neoplasms; recurrence risk; follow-up studies; lymph node metastasis; lymphatic metastasis; neoplasm staging; prospective studies; neoplasm recurrence, local; tumor volume; tomography, x-ray computed; cancer invasion; outcome; hepatectomy; alpha fetoprotein; rare disease; hepatic resection; fibrolamellar; hepatocellolar carcinoma; fibrolamellar liver cell carcinoma |
| Journal Title: | Cancer |
| Volume: | 106 |
| Issue: | 6 |
| ISSN: | 0008-543X |
| Publisher: | Wiley Blackwell |
| Date Published: | 2006-03-15 |
| Start Page: | 1331 |
| End Page: | 1338 |
| Language: | English |
| DOI: | 10.1002/cncr.21703 |
| PUBMED: | 16475212 |
| PROVIDER: | scopus |
| DOI/URL: | |
| Notes: | --- - "Cited By (since 1996): 37" - "Export Date: 4 June 2012" - "CODEN: CANCA" - "Source: Scopus" |
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