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The Korean Journal of Medicine 2007;72(2): 235-240.
증례 : 갈색세포종을 표현한 제1형 신경섬유종 1예
박훈석
A case of neurofibromatosis type 1 presenting with pheochromocytoma
Hoon Suk Park
고려대학교 의과대학 내과학교실
ABSTRACT
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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