UNLABELLED: Hepatoblastoma is a highly malignant embryonal liver tumor that occurs almost exclusively in infants and toddlers. Trisomy 18 is the second most common autosomal trisomy after trisomy 21 and is generally considered a lethal disorder. Ten cases of hepatoblastoma in children with trisomy 18 have been published to date. Here, we report on two female patients with trisomy 18 and pretreatment extent of disease (PRETEXT) stage 1 hepatoblastoma, which support the presence of a nonrandom association between hepatoblastoma and trisomy 18. Both patients underwent primary surgical resection without any neoadjuvant or adjuvant chemotherapy. The histologies returned as pure fetal epithelial type, and combined fetal and embryonal epithelial type. There was no evidence of recurrence on serial abdominal ultrasound and serum alpha-fetoprotein levels on follow-up. CONCLUSION: Primary surgical resection is a treatment approach that can be considered in children with trisomy 18 and PRETEXT stage 1 tumor. However, in view of the overall prognosis for trisomy 18, the decision on the optimal treatment is a delicate one and has to be individualized in the context of the best interests of the child.
UNLABELLED: Hepatoblastoma is a highly malignant embryonal liver tumor that occurs almost exclusively in infants and toddlers. Trisomy 18 is the second most common autosomal trisomy after trisomy 21 and is generally considered a lethal disorder. Ten cases of hepatoblastoma in children with trisomy 18 have been published to date. Here, we report on two female patients with trisomy 18 and pretreatment extent of disease (PRETEXT) stage 1 hepatoblastoma, which support the presence of a nonrandom association between hepatoblastoma and trisomy 18. Both patients underwent primary surgical resection without any neoadjuvant or adjuvant chemotherapy. The histologies returned as pure fetal epithelial type, and combined fetal and embryonal epithelial type. There was no evidence of recurrence on serial abdominal ultrasound and serum alpha-fetoprotein levels on follow-up. CONCLUSION: Primary surgical resection is a treatment approach that can be considered in children with trisomy 18 and PRETEXT stage 1 tumor. However, in view of the overall prognosis for trisomy 18, the decision on the optimal treatment is a delicate one and has to be individualized in the context of the best interests of the child.
Authors: Elaine Maria Pereira; Robert Marion; K H Ramesh; Jane S Kim; Michelle Ewart; Rosanna Ricafort Journal: J Pediatr Hematol Oncol Date: 2012-05 Impact factor: 1.289
Authors: Rajkumar Venkatramani; Logan G Spector; Michael Georgieff; Gail Tomlinson; Mark Krailo; Marcio Malogolowkin; Wendy Kohlmann; Karen Curtin; Rachel K Fonstad; Joshua D Schiffman Journal: Am J Med Genet A Date: 2014-06-16 Impact factor: 2.802
Authors: Bartosz Czuba; Piotr Tousty; Wojciech Cnota; Dariusz Borowski; Agnieszka Jagielska; Mariusz Dubiel; Anna Fuchs; Magda Fraszczyk-Tousty; Sylwia Dzidek; Anna Kajdy; Grzegorz Świercz; Sebastian Kwiatkowski Journal: J Clin Med Date: 2022-04-08 Impact factor: 4.964