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KMID : 0882420150890030327
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Korean Journal of Medicine
2015 Volume.89 No. 3 p.327 ~ p.330
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Multiple Ruptured Cerebral Aneurysms as a Presenting Feature of Pheochromocytoma in a Patient with Neurofibromatosis 1
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Kwon Min-Kwan
Kim Da-Hee
Choi Sung-Jin
Lee Hye-Young
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Abstract
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Neurofibromatosis 1 (NF-1) shows an autosomal dominant pattern of inheritance with complete penetrance and variable expression. Vascular abnormalities are frequently associated with NF-1. Pheochromocytoma occurs in 0.1-5.7% of patients with NF-1. Spontaneous intracerebral hemorrhage in patients with NF-1-related pheochromocytoma is very rare. Herein, we report a case of subarachnoid hemorrhage caused by multiple ruptured cerebral aneurysms in a patient with concurrent NF-1 and pheochromocytoma. Coil embolization of the aneurysms and subsequent adrenalectomy for pheochromocytoma were successfully performed, and the patient remained normotensive thereafter. This case demonstrates the importance of a careful search for a remediable cause of episodic hypertension in patients with NF-1, who are predisposed to cerebral hemorrhage from fluctuating blood pressure.
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KEYWORD
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Neurofibromatosis 1, Pheochromocytoma, Subarachnoid hemorrhage
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