Revista espanola de enfermedades digestivas · 2013
Adult hepatoblastoma.
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Cienfuegos Javier A, Labiano Tania, Pedano Nicolás, Zozaya Gabriel N, Martí-Cruchaga Pablo, Panizo Ángel, Rotellar Fernando
Аннотация
Adult hepatoblastoma (AHB) is a very rare tumor, having been described 45 cases up to June 2012. In contrast to HB in infancy (IHB), it has poor prognosis. We present the case of a 37-year-old asymptomatic woman who consulted for a large -12 cm diameter- mass involving segments 5 and 6 of the liver, and alfa-fetoprotein of 1,556,30 UI/mL. A bisegmentectomy was carried out. The microscopic study confirmed the AHB diagnosis, revealing the presence of epithelial cells forming clusters, trabecular patterns and tubules. The patient died on the 10th postoperative month due to progression disease.The Wnt/Beta-Catenin signaling pathway mutation has been reported and associated with a poor prognosis in IHB. Due to the AHB poor prognosis, seems reasonable to introduce the therapeutic regimens described in children who have a better outcome.