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Abstract Details
Histologic type predicts disparate outcomes in pediatric hepatocellular neoplasms: A Pediatric Surgical Oncology Research Collaborative study
Cancer. 2022 Jul 15;128(14):2786-2795. doi: 10.1002/cncr.34256. Epub 2022 May 13.
1Division of Pediatric Surgery, Primary Children's Hospital, University of Utah, Salt Lake City, Utah.
2Division of Pediatric Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio.
3Department of Surgery, Memorial Sloan Kettering Cancer Center, New York, New York.
4Department of Surgery, St Jude Children's Research Hospital, Memphis, Tennessee.
5Department of Surgery, Ann and Robert H. Lurie Children's Hospital of Chicago, Northwestern University, Chicago, Illinois.
6Division of Pediatric Surgery, Children's Hospital of Pittsburgh, Pittsburgh, Pennsylvania.
7Division of Pediatric Surgery, Children's Hospital Los Angeles, Keck School of Medicine, University of Southern California, Los Angeles, California.
8Department of Pediatric Surgery, Children's Hospital of Colorado, Denver, Colorado.
9Division of Pediatric Surgery, Children's Medical Center, University of Texas Southwestern, Dallas, Texas.
10Division of Pediatric General and Thoracic Surgery, Department of Surgery, Seattle Children's Hospital, University of Washington, Seattle, Washington.
11Division of Pediatric Surgery, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas.
12Division of Pediatric Surgery, Medical College of Wisconsin, Milwaukee, Wisconsin.
13Division of Pediatric Surgery, Children's Hospital, London Health Sciences Center, London, Ontario, Canada.
14Department of General and Pediatric Surgery, Hospital for Sick Children, Toronto, Ontario, Canada.
15Division of Pediatric Pathology, Hospital for Sick Children, Toronto, Ontario, Canada.
16Division of Pediatric Surgery, Duke University Medical Center, Durham, North Carolina.
17Division of Pediatric Surgery, Department of Surgery, Nationwide Children's Hospital, The Ohio State University College of Medicine, Columbus, Ohio.
18Division of Pediatric Surgery, American Family Children's Hospital, University of Wisconsin School of Medicine and Public Health, Madison, Wisconsin.
19Division of Pediatric Surgery, Cohen Children's Medical Center, Zucker School of Medicine at Northwell/Hofstra, New Hyde Park, New York.
20Division of Pediatric Surgery, Oregon Health and Science University Doernbecher Children's Hospital, Portland, Oregon.
Abstract
Background: Hepatocellular carcinoma (HCC) is a rare cancer in children, with various histologic subtypes and a paucity of data to guide clinical management and predict prognosis.
Methods: A multi-institutional review of children with hepatocellular neoplasms was performed, including demographic, staging, treatment, and outcomes data. Patients were categorized as having conventional HCC (cHCC) with or without underlying liver disease, fibrolamellar carcinoma (FLC), and hepatoblastoma with HCC features (HB-HCC). Univariate and multivariate analyses identified predictors of mortality and relapse.
Results: In total, 262 children were identified; and an institutional histologic review revealed 110 cHCCs (42%; 69 normal background liver, 34 inflammatory/cirrhotic, 7 unknown), 119 FLCs (45%), and 33 HB-HCCs (12%). The authors observed notable differences in presentation and behavior among tumor subtypes, including increased lymph node involvement in FLC and higher stage in cHCC. Factors associated with mortality included cHCC (hazard ratio [HR], 1.63; P = .038), elevated α-fetoprotein (HR, 3.1; P = .014), multifocality (HR, 2.4; P < .001), and PRETEXT (pretreatment extent of disease) stage IV (HR, 5.76; P < .001). Multivariate analysis identified increased mortality in cHCC versus FLC (HR, 2.2; P = .004) and in unresectable tumors (HR, 3.4; P < .001). Disease-free status at any point predicted survival.
Conclusions: This multi-institutional, detailed data set allowed a comprehensive analysis of outcomes for children with these rare hepatocellular neoplasms. The current data demonstrated that pediatric HCC subtypes are not equivalent entities because FLC and cHCC have distinct anatomic patterns and outcomes in concert with their known molecular differences. This data set will be further used to elucidate the impact of histology on specific treatment responses, with the goal of designing risk-stratified algorithms for children with HCC.
Lay summary: This is the largest reported granular data set on children with hepatocellular carcinoma. The study evaluates different subtypes of hepatocellular carcinoma and identifies key differences between subtypes. This information is pivotal in improving understanding of these rare cancers and may be used to improve clinical management and subsequent outcome in children with these rare malignancies.