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1 Departments of *Pediatrics,
University of Southern
California School of Medicine, Los
Angeles County-University of Southern
California Medical Center,
Los Angeles, California
2 Obstetrics and Gynecology, University of Southern
California School of Medicine, Los
Angeles County-University of Southern
California Medical Center,
Los Angeles, California
3 Pathology, University of Southern
California School of Medicine, Los
Angeles County-University of Southern
California Medical Center,
Los Angeles, California
Findings in a six-year-old boy with a virilizing
tumor of the left testis are presented. His bone
age was 14 years. Serum testosterone (5.9
ng/ml), urinary 17-ketosteroid (29 mg/24 hr), and
pregnanetriol (14 mg/24 hr) excretion was elevated. These values were not suppressed after
the administration of dexamethasone. Spermatic-peripheral vein concentration differences
showed that the tumor secreted 17-hydroxypregnenolone, dehydroepiandrosterone (DHEA),
dehydroepiandrosterone sulfate (DHEA-S), progesterone, 17-hydroxyprogesterone, androstenedione, testosterone, estrone, estradiol, 11-desoxycortisol, and corticosterone. The tumor
did not secrete cortisol. Electron microscopy
study of the tumor revealed a dimorphic population of light and dark cells with many features
seen in Leydig cell tumors. However, these
were not distinct from those of adrenal rest
tumors. Reinke crystalloids were not found.
True precocious puberty developed five
months after tumor removal, ie, the right testis
increased in size, and serum gonadotropins increased. Seven months after tumor removal,
the patient was demonstrated not to have impaired cortisol synthesis, ie, urinary 17-hydroxysteroids increased from 1.8 to 38.0 mg/24 hr
in response to adrenocorticotropic hormone
(ACTH) administration. The differential diagnosis of virilizing testicular tumors lies between
Leydig cell adenoma and adrenal rest tumor in
congenital adrenal hyperplasia. The demonstration that the patient did not have impaired
cortisol synthesis is consistent with his tumor
being a Leydig cell adenoma.
Key words: in vivo studies of steroid secretion, virilizing testicular tumor, testicular tumor, steroid secretion
Submitted on August 11, 1980
Revised on November 10, 1980
Accepted on November 10, 1980
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