AIM OF THE STUDY: To investigate the characteristics of patients with hepatoblastoma and low serum alpha-fetoprotein (AFP) at diagnosis. PATIENTS AND METHODS: Inclusion of all 21 patients accrued onto SIOPEL trials, whose serum AFP was <100ng/ml at diagnosis. Slides of all 15 patients with available histological material were centrally reviewed. RESULTS: Median age: 10 months. Disease extension at diagnosis: PRETEXT group: II (3 patients), III (10 patients) and IV (8 patients). Extra-hepatic extension: 8 patients. Multifocal tumour: 8 patients. Histology at review: wholly epithelial subtype: 11/15 patients including nine with a small-cell undifferentiated histology. OUTCOME: only 9 patients achieved a partial response and 16 died. Median survival: 4.4 months. Two-year overall survival: 24% (confidence interval 10-45%). CONCLUSION: This study clearly identifies patients with hepatoblastoma and low serum AFP at diagnosis as a high-risk subgroup with extensive disease at diagnosis, poor response to chemotherapy and a poor outcome.
AIM OF THE STUDY: To investigate the characteristics of patients with hepatoblastoma and low serum alpha-fetoprotein (AFP) at diagnosis. PATIENTS AND METHODS: Inclusion of all 21 patients accrued onto SIOPEL trials, whose serum AFP was <100ng/ml at diagnosis. Slides of all 15 patients with available histological material were centrally reviewed. RESULTS: Median age: 10 months. Disease extension at diagnosis: PRETEXT group: II (3 patients), III (10 patients) and IV (8 patients). Extra-hepatic extension: 8 patients. Multifocal tumour: 8 patients. Histology at review: wholly epithelial subtype: 11/15 patients including nine with a small-cell undifferentiated histology. OUTCOME: only 9 patients achieved a partial response and 16 died. Median survival: 4.4 months. Two-year overall survival: 24% (confidence interval 10-45%). CONCLUSION: This study clearly identifies patients with hepatoblastoma and low serum AFP at diagnosis as a high-risk subgroup with extensive disease at diagnosis, poor response to chemotherapy and a poor outcome.
Authors: Huabo Wang; Jie Lu; Lia R Edmunds; Sucheta Kulkarni; James Dolezal; Junyan Tao; Sarangarajan Ranganathan; Laura Jackson; Marc Fromherz; Donna Beer-Stolz; Radha Uppala; Sivakama Bharathi; Satdarshan P Monga; Eric S Goetzman; Edward V Prochownik Journal: J Biol Chem Date: 2016-10-13 Impact factor: 5.157
Authors: Laura L Stafman; Mary G Waldrop; Adele P Williams; Jamie M Aye; Jerry E Stewart; Elizabeth Mroczek-Musulman; Karina J Yoon; Kimberly Whelan; Elizabeth A Beierle Journal: J Pediatr Surg Date: 2019-02-28 Impact factor: 2.545
Authors: Howard M Katzenstein; Wayne L Furman; Marcio H Malogolowkin; Mark D Krailo; M Beth McCarville; Alexander J Towbin; Greg M Tiao; Milton J Finegold; Sarangarajan Ranganathan; Stephen P Dunn; Max R Langham; Eugene D McGahren; Carlos Rodriguez-Galindo; Rebecka L Meyers Journal: Cancer Date: 2017-02-17 Impact factor: 6.860
Authors: Angela D Trobaugh-Lotrario; Gail E Tomlinson; Milton J Finegold; Lia Gore; James H Feusner Journal: Pediatr Blood Cancer Date: 2009-03 Impact factor: 3.167