Intra and Extra-Axial Cavernous Malformation with Venous Linkage: Immune Cellular Inflammation associated with Aggressiveness

Abstract Objectives Intra-orbital and intracerebral cavernous malformations (CM) lesions are considered independent entities. Purely cerebral cavernous malformations have variable biology with recent evidence depicting inflammation as an important player and a risk factor for aggressiveness. We describe a case of concomitant left intra and extra-axial cavernous malformations, linked by ipsilateral basal vein, where the extra-axial component have developed a very aggressive behavior. Methods A 35 years-old female patient presented with a rapid and progressive exophthalmos and loss of vision on the left eye. Cranial magnetic resonance and angiography examinations demonstrated a left cranio-facial cavernous malformation and a large intra-orbital component. The lesion was connected through a large basal vein to a cerebral intraventricular cavernous malformation. Transconjunctival resection showed typical findings of cavernous malformation. A complete histopathology and immunostainning analysis was performed and revealed a clear acute linfomononuclear reaction with a predominant immune cellular inflammation. Conclusion A case of intra-orbital and extra-cranial cavernomatous mass, connected to a cerebral intra-ventricular cavernous malformation through a large basal vein, has presented with a very aggressive course. A complete histopathological and immunohistochemical analysis of the orbital mass has pictured a clear immune-cellular inflammatory reaction adding to the amounting evidence of association between inflammation and site agressiveness in the setting of cavernous malformations.