The spectrum of pheochromocytoma in hypertensive patients with neurofibromatosis.
We have found an appreciable number of pheochromocytomas in patients with neurofibromatosis and concurrent hypertension (ten of 18 cases). At diagnosis, the patient age range was 15 to 62 years, the clinical appearance of the neurofibromatosis did not predict who would and who would not have pheochromocytomas, but the age at diagnosis was helpful in that our younger patients tended to have causes of hypertension other than pheochromocytoma. However, several causes of hypertension may coexist. The biochemical findings were highly diagnostic. The pheochromocytomas secreted epinephrine as well as norepinephrine and resided in or next to the adrenal gland. Where pheochromocytoma is the cause of hypertension, its resection generally results in a better control of hypertension than that obtained in patients whose BPs were elevated from other unknown causes.
D019797:3-Iodobenzylguanidine; D000293:Adolescent; D000310:Adrenal Gland Neoplasms / Q000150:complications* / Q000000981:diagnostic imaging / Q000378:metabolism; D000328:Adult; D004837:Epinephrine / Q000378:metabolism; D005260:Female; D006146:Guanidines; D006801:Humans; D006973:Hypertension / Q000150:complications*; D007457:Iodine Radioisotopes; D008297:Male; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary; D009456:Neurofibromatosis 1 / Q000150:complications*; D009638:Norepinephrine / Q000378:metabolism; D010673:Pheochromocytoma / Q000150:complications* / Q000000981:diagnostic imaging / Q000378:metabolism; D011877:Radionuclide Imaging; D014642:Vanilmandelic Acid / Q000652:urine
D006146:Guanidines; D007457:Iodine Radioisotopes; D019797:3-Iodobenzylguanidine; D014642:Vanilmandelic Acid; D009638:Norepinephrine; D004837:Epinephrine