334 CLINICAL CONSIDERATIONS 



The fact that the paraganglioma are derived from epineph- 

 rine-producing cells and themselves give all the reactions 

 of epinephrine has led to the belief that they may give rise to 

 intermittent and essential hypertension. Patients with vaso- 

 motor instability in whom sudden death follows minor injury 

 {e.g., the extraction of a tooth or the injection of a drug) have 

 been found in several instances to have had such a tumor. The 

 sudden liberation of epinephrine from the tumor has been 

 assumed to be the causative agent of death in these patients. 



Labbe, Tinel, and Doumel, 372 in 1922, first observed the 

 phenomenon of intermittent paroxysmal hypertension asso- 

 ciated with paraganglioma. Mayo, 446 Shipley, 566 Kalk, 347 and 

 others 38 • 512 have reported similar cases cured by the removal of 

 a palpable tumor. These tumors not only arise from the me- 

 dulla but more commonly from other chromaphil tissues, par- 

 ticularly from the organ of Zuckerkandl. 



The typical case of paroxysmal hypertension, associated with 

 a chromaphil cell tumor, is characterized by periodic attacks 

 which occur at intervals of some hours, or in response to some 

 stimulus, for example, vigorous massage of the palpable tumor. 

 The attacks last for some minutes during which the patient 

 becomes apprehensive and deathly pale. Sweating, mydriasis, 

 hypertension, and hyperglycemia are noted during the par- 

 oxysm. Operative removal of the tumor is curative of the 

 condition. In Kalk's 347 case, the ablated tumor was found to 

 contain about 460 milligrams of epinephrine. 



The association of attacks of paroxysmal hypertension with 

 tumors of the chromaphil tissue, as described above, may be 

 rationally explained by assuming that the tumor when stimu- 

 lated sets free into the circulation a large amount of epinephrine 

 which gives rise to the observed symptoms. Sudden death 

 in these patients may also be attributed to the toxic effects of 

 the epinephrine secreted by the tumor and released under the 

 stimulus of one of the numerous factors (Chapter VI) which 

 cause an increased secretion of epinephrine. 



