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KMID : 0882420080750010104
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Korean Journal of Medicine
2008 Volume.75 No. 1 p.104 ~ p.107
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A case of spontaneous intracerebral hemorrhage in neurofibromatosis type 1 associated with pheochromocytoma
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Kim Moo-Gon
Lee Jin-Bae
Hong Sung-Pyo
Kim So-Yeon
Lee Young-Soo
Ryu Jae-Kean
Choi Ji-Yong
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Abstract
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Spontaneous intracerebral hemorrhage is very rare in patients with neurofibromatosis type 1. A 30-year-old woman was referred to our hospital for evaluation of headaches, nausea, and vomiting. She was diagnosed with neurofibromatosis type 1 and a spontaneous intracerebral hemorrhage. She was treated with conservative management, including antihypertensive medications for 32 months. However, because of sustained hypertension, we performed screening tests for a pheochromocytoma. The 24 hr urine VMA and urine metanephrines were elevated. Abdominal CT showed a right adrenal mass, 7.5×5.8 cm in size.. After a successful resection of the tumor, the hypertension resolved without medication for > 1 year. This case illustrates that delayed diagnosis and treatment of pheochromocytoma can cause serious complications from hypertension, such as cerebrovascular hemorrhage. Thus, in patients with neurofibromatosis and hypertension, screening for pheochromocytoma is important for the early detection of an adrenal tumor.
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KEYWORD
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Neurofibromatosis, Pheochromocytoma, Intracerebral hemorrhage
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