title: Hepatocellular Carcinoma: Referral to a Transplantation Unit.
abstract: AimHepatocellular carcinoma (HCC), although being infrequent, is the second-most common primary hepatic malignancy in children, after hepatoblastoma (HB). The prognosis is very poor. We present our series of children with HCC referred to our transplant unit to be assessed as candidates for liver transplantation (LT).MethodsA retrospective review of HCCs referred to our transplant unit in the past 20 years (1994-2015) was performed. Age at diagnosis, disease-free survival, location of recurrence, initial treatment, secondary treatment, and mortality were noted.Main ResultsTen patients (8 boys, 2 girls) met the inclusion criteria. Median age at diagnosis was 11.5 years (0.5-14). HCC was associated with tyrosinemia in two patients, while the tumor developed in absence of previous liver disease in eight. Seven children attempted tumor resection earlier elsewhere. LT was not considered suitable in six patients due to extrahepatic tumor extension and finally it was performed in four (two with tyrosinemia and two with "de novo" HCC). Only one of the transplants was primary, and the other three were performed as rescue therapy. After 78 (66-90) months of follow-up, the two patients with tyrosinemia remain alive and disease free, while the other two had distant relapses, 35 and 37 months after LT, respectively, and finally died due to tumor progression.ConclusionsHCC is a rare, very aggressive tumor in children who has a very poor prognosis. Our results suggest the need for new strategies. Early referral of all cases to highly specialized centers with a liver transplant unit and perhaps a more liberal use of LT, even for selected, apparently resectable cases, are possible options.
authors.affiliation: Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: Paloma
authors.initials: P
authors.lastname: Triana
authors.affiliation: Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: Mariela
authors.initials: M
authors.lastname: Dore
authors.affiliation: Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: Martha Muñoz
authors.initials: MM
authors.lastname: Romo
authors.affiliation: Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: Javier Jimenez
authors.initials: JJ
authors.lastname: Gomez
authors.affiliation: Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: Alba Sánchez
authors.initials: AS
authors.lastname: Galán
authors.affiliation: Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: Francisco
authors.initials: F
authors.lastname: Hernandez
authors.affiliation: Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: Ane M Andres
authors.initials: AM
authors.lastname: Moreno
authors.affiliation: Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: Jose Luis
authors.initials: JL
authors.lastname: Encinas
authors.affiliation: Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: Leopoldo
authors.initials: L
authors.lastname: Martinez
authors.affiliation: Department of Pediatric Surgery, Hospital Universitario La Paz, Madrid, Spain.
authors.forename: Manuel Lopez
authors.initials: ML
authors.lastname: Santamaria
mesh_terms: D000293:Adolescent; D006528:Carcinoma, Hepatocellular / Q000401:mortality / Q000601:surgery*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007223:Infant; D008113:Liver Neoplasms / Q000401:mortality / Q000601:surgery*; D016031:Liver Transplantation; D008297:Male; D012017:Referral and Consultation; D012189:Retrospective Studies; D016019:Survival Analysis; D016896:Treatment Outcome