An unusual pheochromocytoma associated with an androgen secreting adrenocortical adenoma. Evaluation of its polypeptide hormone, catecholamine, and enzyme characteristics.
Our patient had a left suprarenal mass. His blood pressure was normal, but his urinary catecholamines (CA), vanillylmandelic acid (VMA), total metanephrines (TMn) and 5-hydroxyindolacetic acid (5HIAA) were elevated. In addition, he had elevated, nonsuppressible urinary 17-ketosteroids (17KS) and androsterone, but his urinary 17-hydroxycorticoids (17OHCS) and free cortisol were normal, as were his plasma cortisol and ACTH. After resection of the suprarenal mass, the patient's urinary hormone values reverted to normal. The mass contained a pheochromocytoma and an adrenocortical adenoma. The pheochromocytoma was unusual in that it contained very little norepinephrine (NE) and dopamine (DA) and an abundance of epinephrine (E) despite normal enzyme concentrations. Electron micrographs showed primarily E granules with few of the NE-type. The immunoperoxidase histochemical stains for vasoactive intestinal peptide (VIP) and serotonin (S) were strongly positive. The patient's blood pressure may have been normal because his pheochromocytoma secreted E, VIP, or S. The associated adrenocortical adenoma produced no symptoms and was probably coincidental.
D000236:Adenoma / Q000378:metabolism*; D000306:Adrenal Cortex Neoplasms / Q000378:metabolism*; D000728:Androgens / Q000378:metabolism*; D002395:Catecholamines / Q000378:metabolism* / Q000652:urine; D008401:Gas Chromatography-Mass Spectrometry; D006651:Histocytochemistry; D006801:Humans; D007124:Immunoenzyme Techniques; D008297:Male; D008854:Microscopy, Electron; D008875:Middle Aged; D009377:Multiple Endocrine Neoplasia / Q000378:metabolism*; D010673:Pheochromocytoma / Q000378:metabolism*; D012701:Serotonin / Q000378:metabolism; D014660:Vasoactive Intestinal Peptide / Q000378:metabolism
D000728:Androgens; D002395:Catecholamines; D012701:Serotonin; D014660:Vasoactive Intestinal Peptide