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Predisposing Conditions to Pediatric Hepatocellular Carcinoma and Association With Outcomes

Single-center Experience

Cowell, Elizabeth*; Patel, Kalyani; Heczey, Andras; Finegold, Milton; Venkatramani, Rajkumar; Wu, Hao; López-Terrada, Dolores§; Miloh, Tamir||

Journal of Pediatric Gastroenterology and Nutrition: May 2019 - Volume 68 - Issue 5 - p 695–699
doi: 10.1097/MPG.0000000000002285
Original Articles: Hepatology
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Objectives: Hepatocellular carcinoma (HCC) has been linked to chronic viral or metabolic liver disease and other conditions. The characteristics of children with HCC have not been fully elucidated and outcomes in children with predisposing liver disease are not well defined.

Methods: Patients ≤21 years old with HCC managed at our institution and through external consultation between 1996 and 2016 were included. Demographics, clinical history, and pathology were tabulated. Fisher exact test and Wilcoxon test were employed for subgroup comparison, and survival differences were evaluated by Kaplan-Meier method.

Results: Sixty-one cases of HCC were identified. Seven of 16 patients (44%) at our institution and 18 of 45 consult patients (40%) had a predisposing condition: cryptogenic cirrhosis/steatosis (9), genetic (7), biliary pathology (4), viral hepatitis (1), and other (4). Thirteen of 27 patients with de novo HCC had fibrolamellar HCC. Clinical characteristics were grouped by presence or absence of predisposing conditions: age at diagnosis (7.2 vs 10.2 years, P < 0.05), metastatic disease at presentation (15% vs 44%, P = n.s), and tumor size >4 cm (20% vs 100%, P < 0.05). In patients treated at our institution, 5 of 7 with predisposing conditions received liver transplant and achieved complete remission, whereas only 3 of 9 patients with de novo HCC received curative surgery and this group had decreased median overall survival (P < 0.05).

Conclusions: The majority of children with HCC did not have predisposing liver or associated disease. These patients were diagnosed later with more advanced stage disease and had significantly decreased overall survival.

*Department of Pediatrics

Department of Pathology

Department of Pediatrics-Oncology

§Department of Pathology, Pediatrics-Oncology

||Department of Pediatrics-Gastroenterology, Baylor College of Medicine, Houston, TX.

Address correspondence and reprint requests to Elizabeth Cowell, MD, Department of Pediatrics, Baylor College of Medicine, One Baylor Plaza, Houston, TX 77030 (e-mail: Elizabeth.Cowell@bcm.edu).

Received 22 June, 2018

Accepted 4 January, 2019

Supplemental digital content is available for this article. Direct URL citations appear in the printed text, and links to the digital files are provided in the HTML text of this article on the journal's Web site (www.jpgn.org).

The authors report no conflicts of interest.

© 2019 by European Society for Pediatric Gastroenterology, Hepatology, and Nutrition and North American Society for Pediatric Gastroenterology,