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. 2015 Jan;50(1):153-6.
doi: 10.1016/j.jpedsurg.2014.10.039. Epub 2014 Dec 10.

Prognostic factors in fibrolamellar hepatocellular carcinoma in young people

David G Darcy  1 , Marcus M Malek  1 , Rachel Kobos  2 , David S Klimstra  3 , Ronald DeMatteo  4 , Michael P La Quaglia  5
Affiliations

Prognostic factors in fibrolamellar hepatocellular carcinoma in young people

David G Darcy et al. J Pediatr Surg. 2015 Jan.

Abstract

Background/purpose: Fibrolamellar hepatocellular carcinoma (FL-HCC) arises in pediatric/adolescent patients without cirrhosis. We retrospectively evaluated the impact of resection, nodal status, metastasis, and PRETEXT stage on overall survival (OS).

Methods: With IRB approval, we reviewed records of 25 consecutive pediatric patients with FL-HCC treated at our institution from 1981 to 2011. We evaluated associations between OS and PRETEXT stage, nodal involvement, metastasis, and complete resection.

Results: Median age at diagnosis was 17.1years (range, 11.6-20.5). Median follow-up was 2.74years (range, 5-9.5). Five (28%) patients had PRETEXT stage 1 disease, 10 (56%) had stage 2, 2 (11%) had stage 3, and 2 (11%) had stage 4 disease. On presentation, 17 (68%) patients had N1 disease, and 7 (28%) had parenchymal metastases. Complete resection was achieved in 17 (80.9%) of 21 patients who underwent resection. Five-year OS was 42.6%. Survival was positively associated with complete resection (P =0.003), negative regional lymph nodes (P =0.044), and lower PRETEXT stage (P <0.001), with a trend for metastatic disease (P =0.05).

Conclusions: In young patients with FL-HCC, lower PRETEXT stage and complete resection correlated with prolonged survival, while metastatic disease and positive lymph node status were associated with poor prognosis. Thus, we recommend complete resection and regional lymphadenectomy whenever possible.

Keywords: Adolescent; Fibrolamellar; Hepatocellular carcinoma; Pediatric; Prognostic.

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Figures

Figure 1
Figure 1
Kaplan-Meier curve showing the overall survival rate for the entire cohort. Censored data points reflect the time that a patient was last seen; there were no known deaths during the study period.
Figure 2
Figure 2
Kaplan-Meier curve showing the overall survival rate by resection margin status. R0 = complete resection, R1 = microscopically positive margins, R2 = gross residual disease; all patients were alive at last follow-up.
Figure 3
Figure 3
Kaplan-Meier curve showing the overall survival rate by PRETEXT stage.
Figure 4
Figure 4
Kaplan-Meier curve showing the overall survival rate by regional lymph node status. N0 = negative regional lymph node involvement, N1 = positive regional lymph node involvement.
See this image and copyright information in PMC

References

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Supplementary concepts