Prognostic factors in fibrolamellar hepatocellular carcinoma in young people Journal Article


Authors: Darcy, D. G.; Malek, M.; Kobos, R.; Klimstra, D. S.; DeMatteo, R.; La Quaglia, M. P.
Article Title: Prognostic factors in fibrolamellar hepatocellular carcinoma in young people
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 patientswith 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 17.1 years (range, 11.6-20.5). Median follow-up was 2.74 years (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, whilemetastatic disease and positive lymph node status were associated with poor prognosis. Thus, we recommend complete resection and regional lymphadenectomy whenever possible.
Keywords: adolescent; adult; child; clinical article; school child; survival rate; young adult; overall survival; cancer recurrence; hepatocellular carcinoma; artificial embolism; liver cell carcinoma; cancer adjuvant therapy; outcome assessment; lymph node metastasis; lymph node dissection; anorexia; pain; nausea; retrospective study; childhood cancer; precocious puberty; pediatric; fever; lung embolism; minimal residual disease; cancer size; surgical infection; jaundice; postoperative infection; total parenteral nutrition; abdominal mass; liver biopsy; liver lobectomy; intraoperative radiotherapy; prognostic; xiao chai hu tang; cancer prognosis; stomach distension; fibrolamellar; adjuvant chemoradiotherapy; human; male; female; priority journal; article; fibrolamellar hepatocellular carcinoma
Journal Title: Journal of Pediatric Surgery
Volume: 50
Issue: 1
ISSN: 0022-3468
Publisher: W.B. Saunders Co-Elsevier Inc.  
Date Published: 2015-01-01
Start Page: 153
End Page: 156
Language: English
DOI: 10.1016/j.jpedsurg.2014.10.039
PROVIDER: scopus
PUBMED: 25598114
PMCID: PMC4558902
DOI/URL:
Notes: Export Date: 2 March 2015 -- Source: Scopus
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MSK Authors
  1. Ronald P DeMatteo
    627 DeMatteo
  2. David S Klimstra
    922 Klimstra
  3. Rachel Kobos
    68 Kobos
  4. David Gene Darcy
    6 Darcy
  5. Marcus Malek
    2 Malek