Undifferentiated small cell hepatoblastoma with rhabdoid morphology diagnosed prenatally: case report

Abstract

Liver tumors are rare in children Hepatoblastoma HB is usually diagnosed during the first three years of life and represents approximately of malignant tumors Occasionally it nbsp can nbsp be nbsp diagnosed nbsp in nbsp neonates nbsp or nbsp during nbsp the nbsp prenatal nbsp period nbsp It nbsp is nbsp an nbsp embryonic nbsp tumor nbsp that nbsp originates nbsp from nbsp hepatocyte nbsp precursors nbsp that nbsp recapitulate nbsp stages nbsp of nbsp organ nbsp development nbsp The nbsp variant nbsp of nbsp undifferentiated nbsp small nbsp cells nbsp or nbsp Small nbsp Cell nbsp Undifferentiated nbsp SCUD nbsp is nbsp an nbsp epithelial variant of HB that represents between to of cases occurring in a younger age nbsp group nbsp of nbsp nbsp years nbsp old nbsp are nbsp low nbsp or nbsp normal nbsp serum nbsp alpha fetoprotein nbsp unlike nbsp the nbsp classic nbsp variant and exhibits a very aggressive clinical behavior It is a population of small round and nbsp blue nbsp cells nbsp that nbsp co express nbsp stromal nbsp and nbsp epithelial nbsp markers nbsp and nbsp can nbsp grow nbsp in nbsp a nbsp diffuse nbsp pattern in groups nests or organoid pattern Histology is a prognostic factor of death risk factor There is a subgroup of SCUD that exhibits morphological and immunophenotypic characteristic of hepatic malignant rhabdoid tumors such as loss of nuclear expression of INI by immunohistochemistry with or without deletion of the INI gene It is an extremely aggressive neoplasm with a diffuse growth pattern and should be distinguished from other small and round small blue cell tumors The objective is to present an infrequent case of embryonic hepatocyte tumor with prenatal presentation