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Type 1a glycogen storage disease with hepatoblastoma in siblings
Author(s) -
Ito Etsuro,
Sato Yuichi,
Kawauchi Kyoichi,
Munakata Hirohumi,
Kamata Yosimasa,
Yodono Hirahu,
Yokoyama Masaru
Publication year - 1987
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(19870515)59:10<1776::aid-cncr2820591016>3.0.co;2-t
Subject(s) - hepatoblastoma , medicine , glycogen storage disease type i , glycogen storage disease , glycogen , hepatocellular carcinoma , endoplasmic reticulum , pathology , disease , neoplasm , biology , genetics
Clinical and histologic details of the two siblings with type 1a glycogen storage disease (GSD‐1a) who developed hepatoblastoma are presented. The light microscopic studies on hepatic tumor in both siblings revealed fetal type of hepatoblastoma. Ultrastructural findings in Patient 2 showed markedly altered mitochondria, which were frequently surrounded by the rough endoplasmic reticulum. This is the first known occurrence with this association, and the third report on the familial occurrence of this neoplasm. Glycogen storage disease may increase the risk of hepatocellular carcinoma and hepatoblastoma.