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The Korean Journal of Medicine 2007;72(2): 235-240.
利앸 : 媛덉깋꽭룷醫낆쓣 몴쁽븳 젣1삎 떊寃쎌꽟쑀醫 1삁
A case of neurofibromatosis type 1 presenting with pheochromocytoma
Hoon Suk Park
怨좊젮븰援 쓽怨쇰븰 궡怨쇳븰援먯떎
Pheochromocytoma is usually derived from the adrenal medulla or the chromaffin cells in the sympathetic nerve system. The majority of pheochromocytomas are sporadic, but approximately 10% or more occur in association with familial disorders such as MEN (multiple endocrine neoplasia) type 2A or 2B, VHL (von Hippel-Lindau) disease and neurofibromatosis (von Recklinghausen`s disease). Patients with hereditary pheochromocytoma and its related familial disorders can present with hypertension, tachycardia, anxiety and symptoms that are produced by disorders of the other endocrine organs. Yet there are few reports about hereditary pheochromocytoma and associated endocrine disorders. In this report, we describe a patient with pheochromocytoma that was associated with neurofibromatosis type 1, and this was successfully treated after adrenalectomy.(Korean J Med 72:S235-S240, 2007) Key Words : Hereditary pheochromocytoma, Neurofibromatosis type 1, von Recklinghausen`s disease
Keywords: Hereditary pheochromocytoma, Neurofibromatosis type 1, von Recklinghausen`s disease
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