J Cerebrovasc Endovasc Neurosurg.  2015 Sep;17(3):257-262. 10.7461/jcen.2015.17.3.257.

Unusual Presentation of Cerebral Cavernous Malformation

• Kim WH
• Lim DJ
• Choi JI
• Ha SK
• Kim SD
• Kim SH

• KMID: 2069253
• DOI: http://doi.org/10.7461/jcen.2015.17.3.257

Abstract

Cerebral cavernous malformations (CMs) are vascular malformations of the central nervous system, which can be detected in the absence of any clinical symptoms. Nodules and cysts with mixed signal intensity and a peripheral hemosiderin rim are considered brain magnetic resonance imaging (MRI) findings typical of CMs. A 48-year-old man was admitted to our hospital because of abnormal MRI findings without significant neurological symptoms. A cyst with an internal fluid-fluid level was found in the left basal ganglia on the initial brain MRI. We decided to observe the natural course of the asymptomatic lesion with serial MRI follow-up. On MRI at the 5-month follow-up, the cystic mass was enlarged and showed findings consistent with those of cystic CM. Surgical resection was performed and the pathological diagnosis was CM. Our experience suggests that the initial presentation of a CM can be a pure cyst and neurosurgeons should consider the likelihood of CMs in cases of cystic cerebral lesions with intracystic hemorrhage.

Keyword

Cavernous malformation; Cyst; Magnetic resonance imaging

MeSH Terms

Basal Ganglia
Brain
Central Nervous System
Diagnosis
Follow-Up Studies
Hemangioma, Cavernous, Central Nervous System*
Hemorrhage
Hemosiderin
Humans
Magnetic Resonance Imaging
Middle Aged
Vascular Malformations
Hemosiderin

Figure

• 

  Fig. 1 Initial brain imaging findings for this patient. Axial T1-weighted (A), T2-weighted (B) magnetic resonance images, and computed tomography (C) showed a single cystic mass measuring 16 mm in diameter with a fluid-fluid level in the left basal ganglia. Note that there was no evidence of solid components or a hemosiderin rim. An angiogram (D) showed neither abnormal tumor staining nor vascular malformation.

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  Fig. 2 First repeated brain imaging performed 5 months after the initial studies (A and B) and preoperative imaging performed 7 months after the initial studies (C and D). Axial T1-weighted (A), T2-weighted (B) magnetic resonance images showed a reticulated "salt and pepper" pattern in the core and a peripheral halo of low signal intensity. Axial T1-weighted (C), and T2-weighted (D) magnetic resonance images showed an increase in the size of the lesion with aggravated peri-lesional edema. Mixed signal intensity in the core with peripheral low signal intensity gradually becomes more distinct.

• 

  Fig. 3 In the histopathological examination, the lesion showed irregularly dilated vascular channels lacking a smooth muscle layer. A rim of fibrosis and hemosiderin-laden macrophages on a hemorrhagic background surrounded the lesion. In the lumen of the vascular channels, there were some organizing thrombi. However, there was no evidence of microcalcification. These histopathological findings were consistent with CM. Hematoxylin-eosin staining; magnification ×100 (A and B). A post-operative axial T2-weighted magnetic resonance image (C) shows no residual lesion.

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