CONGENITAL ADRENOCORTICAL HYPERPLASIA WITH GIANT CELLS IN A TWELVE-YEAR-OLD CHILD

Abstract

SUMMARY: The case of a 12-year-old female pseudohermaphrodite is reported in whom masculinization had reached such a degree as to suggest, on clinical grounds, homologous precocious puberty in a boy. Among the male accessories a well-developed prostate was found. Other outstanding features in the clinical picture were: marked hypertension with associated morphological changes in the cardiovascular system, skeletal and other somatic abnormalities suggestive of atavistic rudiments, excessive mental retardation and a diffuse brownish pigmentation of the skin. At autopsy bilateral adrenocortical hyperplasia involving all three zones was found. Microscopically, a conspicuous feature was the presence of numerous 'giant-like' and some genuine giant cells, mainly in the zona fasciculata. Such cells have so far only been observed in adrenocortical malignancy, during embryonic life and in very young infants. The significance and nature of the histological findings are discussed.