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A VIRILIZING ADRENAL TUMOR WITH BORDERLINE ELEVATION OF URINARY 17‐KETOSTEROIDS AND HISTOCHEMICAL DEMONSTRATION OF A DEFICIENCY IN THE δ5/δ4‐ISOMERASE, 3β‐HYDROXYSTEROID DEHYDROGENASE ENZYMATIC SYSTEM
Author(s) -
NETO R. MONTELEONE,
OLIVEIRA J. A. MELLO DE,
SA M. F. S.,
CORNICELLI J.
Publication year - 1979
Publication title -
acta pædiatrica
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.772
H-Index - 115
eISSN - 1651-2227
pISSN - 0803-5253
DOI - 10.1111/j.1651-2227.1979.tb05039.x
Subject(s) - medicine , urinary system , enzyme , dehydrogenase , endocrinology , biochemistry , biology
. A 3‐year‐old girl affected by a virilizing tumor of the adrenal gland, without significant elevation in the levels of 17 ketosteroids (17‐KS) urinary excretion, was studied clinically. Her symptoms started abruptly at the age of 2, with progressive enlargement of the clitoris and the appearance of pubic hair. In various tests, the 17‐KS levels barely exceeded the upper normal limits and at times remained within normal limits. The retropneumoperitoneum X‐ray suggested an enlargement of the right adrenal gland and the presence of a neoplasm, which was actually discovered during surgery. Histopathological examination revealed a well‐defined neoplasm, without capsule invasion and with accentuated cell polymorphism. Histoenzymology showed that the tissue lacked the enzymatic system involving 3β‐hydroxysteroid dehydrogenase (3β‐HSD). Indoxylesterase (I.EST‐A) activity identified the tumor as originating from the internal layers of the adrenal cortex. The histochemical findings were correlated to the clinical picture and the levels of urinary 17‐KS.