344 CLINICAL CONSIDERATIONS 



does priapism, spermatogenesis, or other signs of true puberty 

 occur in the male. It is thus a misnomer to refer to the condi- 

 tion as one of precocious puberty as is usually done. True 

 precocious puberty occurs with tumors of the pineal gland 

 (chiefly in males, rarely in the female) and with tumors of the 

 testicles or ovaries. In these cases there is an actual functional 

 precocity of the reproductive organs. In the female, menstrua- 

 tion occurs as early as the first year, while in males, spermato- 

 genesis, priapism, and masturbation regularly occur. This 

 difference should serve as an important clinical sign to differen- 

 tiate the adreno-genital syndrome from cases of true preco- 

 cious puberty. 365 



As regards the treatment for this condition, surgical removal 

 of the affected gland before metastasis has occurred is obviously 

 necessary. Improvement following such removal with retro- 

 gression of the abnormal signs of masculinization have been 

 reported. 120228 



In many cases reported in the literature, as in the one cited 

 above, there has been an anomaly or complete absence of the 

 opposite adrenal. Hence the surgeon should ensure himself 

 of the state of the unaffected gland before undertaking the 

 removal of the affected adrenal. Should the adrenal on the 

 unaffected side be absent, removal of the diseased gland would 

 lead to death of the patient from adrenal cortical insufficiency. 

 In such cases it is, therefore, necessary to preserve some of the 

 normal cortical tissue either by allowing it to remain in situ 

 or transplanting it to an ovary or testicle. Treatment of the 

 patient postoperatively with the adrenal cortical hormone in 

 minimal doses until regeneration of new cortical tissue has 

 occurred is also necessary. 



The juvenile form of the adreno-genital syndrome is prob- 

 ably caused by hyperplasia of the androgenic zone. Instead 

 of disappearing as it does in normal children during the first 

 year of life, this tissue for some unknown reason proliferates 

 and ultimately assumes malignant characteristics. 



