Bilateral pheochromocytoma associated with neurofibromatosis

Present Status of Diagnosis and Treatment of Pheochromocytoma

“…The tumor usually develops in adult life, slightly more often in women than in men, but may occur in children;3 it is usually situated in or near the adrenal glands but may be found at other places along the sympathetic chain;4 5 it may be multiple and may also be malignant in a minority of cases;6' 7 it may show familial occurrence8' 9 and is sometimes associated with neurofibromatosis.10, 11 The untreated tumor usually leads to a fatal outcome within a relatively short number of years with a clinical course resembling that of malignant hypertension. An increasing number of cases is being reported where surgical complete removal of the tumor'2 has led to partial or complete reversal of the structural and functional changes in the circulatory system that had developed before operation.…”

The Incidence of Phaeochromocytoma in the Netherlands

“…The combination of phaeochromocytoma with neurofibromatosis has been noted by several authors (4, 13, 22,33,35). Glushien et al (13) extended this observation to the entire neurocutaneous syndrome (neurofibromatosis, von Hippel-Lindau disease, tuberous sclerosis and Sturge-Weber syndrome) and estimated that at least ten per cent of patients with phaeochromocytoma would be found to have some form of this complex of diseases.…”

Angiocardiography

“…Dangerous release of adrenal amines can occur in a patient with phiochromocytoma, causing a hypertensive crisis and even death from adrenaline shock. Two deaths have been reported in such patients after the use of contrast media for abdominal aortography [2,43,90].…”