The Korean Journal of Internal Medicine

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Original Article
Korean J Med. 2008;75(1):104-107.
증례 : 갈색세포종이 동반된 신경섬유종증 1형에서 발생한 자발성 뇌출혈1예
김무곤.이진배.홍승표.김소연.이영수.류재근.최지용, , , , , ,
A case of spontaneous intracerebral hemorrhage in neurofibromatosis type 1 associated with pheochromocytoma
Moo Gon Kim, Jin Bae Lee, Seung Pyo Hong, So Yern Kim, Young Soo Lee, Jae Kean Ryu, Ji Yong Choi
경희대학교 의과대학 순환기내과학교실


Abstract
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. (Korean J Med 75:103-107, 2008) Key Words : Neurofibromatosis; Pheochromocytoma; Intracerebral hemorrhage

Keywords :Neurofibromatosis, Pheochromocytoma, Intracerebral hemorrhage
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