von Recklinghausen's disease and pheochromocytomas.

作者: McClellan M. Walther , Judi Herring , Erik Enquist , Harry R. Keiser , W. Marston Linehan

DOI: 10.1016/S0022-5347(05)68171-2

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摘要: Purpose:: We review the literature and characterize clinical findings of von Recklinghausen’sassociated pheochromocytoma.Materials Methods:: A Grateful Med search for years 1966 to 1999 was performed on subjects, “von Recklinghausen” “neurofibromatosis.” Articles from were then reviewed identify older publications. Of 325 articles 118 are included in this review.Results:: Pheochromocytomas have been clinically identified 0.1 5.7% patients with Recklinghausen’s disease. Mean patient age 42 (range 1.5 74) 87 women 61 men at presentation pheochromocytoma. 148 84% had solitary adrenal tumors, 9.6% bilateral disease 6.1% ectopic pheochromocytomas. Symptoms related pheochromocytoma or hypertension noted 78% patients. Tumors secreted epinephrine norepinephrine, 87% demonstrated metaiodobenzylguanidine uptake. 6% died during pregnancy a medical procedure...

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