Korean J Med.  2015 Sep;89(3):327-330. 10.3904/kjm.2015.89.3.327.

Multiple Ruptured Cerebral Aneurysms as a Presenting Feature of Pheochromocytoma in a Patient with Neurofibromatosis 1

• Kwon MK
• Kim DH
• Choi SJ
• Lee HY

• KMID: 2269006
• DOI: http://doi.org/10.3904/kjm.2015.89.3.327

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.

Keyword

Neurofibromatosis 1; Pheochromocytoma; Subarachnoid hemorrhage

MeSH Terms

Adrenalectomy
Aneurysm
Blood Pressure
Cerebral Hemorrhage
Embolization, Therapeutic
Humans
Hypertension
Intracranial Aneurysm*
Neurofibromatoses*
Neurofibromatosis 1*
Penetrance
Pheochromocytoma*
Subarachnoid Hemorrhage
Wills