Open AccessAN AUTOPSY CASE OF CONGENITAL LIPOID HYPERPLASIA OF THE ADRENAL CORTEX
Author(s) -
TSUTSUI Yoshihiro,
HIRABAYASHI Norio,
ITO Gen
Publication year - 1970
Publication title -
acta patholigica japonica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.73
H-Index - 74
ISSN - 0001-6632
DOI - 10.1111/j.1440-1827.1970.tb02748.x
Subject(s) - adrenal cortex , hyperplasia , pathology , autopsy , cortex (anatomy) , myelin , medicine , endocrinology , biology , central nervous system , neuroscience
A case of congenital lipoid hyperplasia of the adrenal cortex in a male baby with male external pseudohermaphroditism who died 79 days after birth was presented. Prominent clinical signs were recurrent vomiting, brown pigmentation of the skin and retardation of the growth. Histologically, the adrenal cortex was characterized by nodular hyperplasia of so‐called spongiocytes loaded with cholesterol ester. Electron‐microscopic study showed that the spongiocytes were filled with numerous vacuoles of very low electron density. These cells also showed cytoplasmic degeneration as indicated by autophagosomes and myelin figures. Chemical analysis of the adrenal tissue revealed that the total cholesterol was four times higher than that in the control, while the ratio of total to esterized form was not significantly different. ACTA PATH. JAP. 20: 227 ‐ 237, 1970.