Multiple Ruptured Cerebral Aneurysms as a Presenting Feature of Pheochromocytoma in a Patient with Neurofibromatosis 1.
10.3904/kjm.2015.89.3.327
- Author:
Min Kwan KWON
1
;
Da Hee KIM
;
Sung Jin CHOI
;
Hye Young LEE
Author Information
1. Division of Cardiology, Department of Internal Medicine, Sanggye Paik Hospital, Inje University College of Medicine, Seoul, Korea. hylee9663@paik.ac.kr
- Publication Type:Case Report
- Keywords:
Neurofibromatosis 1;
Pheochromocytoma;
Subarachnoid hemorrhage
- MeSH:
Adrenalectomy;
Aneurysm;
Blood Pressure;
Cerebral Hemorrhage;
Embolization, Therapeutic;
Humans;
Hypertension;
Intracranial Aneurysm*;
Neurofibromatoses*;
Neurofibromatosis 1*;
Penetrance;
Pheochromocytoma*;
Subarachnoid Hemorrhage;
Wills
- From:Korean Journal of Medicine
2015;89(3):327-330
- CountryRepublic of Korea
- Language:English
-
Abstract:
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.