Multiple intracerebral intravascular papillary endothelial hyperplasia

Abstract

Intravascular papillary endothelial hyperplasia (IPEH) is a rare benign reactive lesion usually found in thrombosed subcutaneous blood vessels. It uncommonly occurs in the central nervous system and may be mistaken for a more malignant type of tumour such as angiosarcoma. We present a first case of multiple IPEH occurring intracranially in a 51-year-old woman. She developed neurological compromises secondary to the mass affect of the haematoma arising from one of the lesions. Prompt surgical evacuation of the haematoma stabilized her condition. Surgical treatment, pathological findings, radiographic characteristics, and a review of the literature are presented.

Thirteen-year follow-up of parasellar intravascular papillary endothelial hyperplasia successfully treated by surgical excision: case report

“…Most cases were of the primary type, although in 5 cases (21.7%), the lesions arose from preexisting vascular malformations, including cavernous malformations (n = 3), 3,7,8 an arteriovenous malformation (n = 1), 17 and a venous angioma (n = 1). 13 Multiple intracranial IPEHs have been reported in 3 cases, 5,12,14 and all were deemed primary type IPEH.…”

“…4D-F [1][2][3][4]12,14,[16][17][18][19] Among these many names for this condition, IPEH is increasingly used, as it directly describes the disease pathology. 3,[5][6][7][8][9][10]13,15 IPEH was originally considered as a neoplasm, but it is currently regarded as an unusual nonneoplastic, reactive endothelial proliferation associated with organizing thrombi. These masses can occur either within the normal intravascular space (primary or pure type), within a preexisting vascular malformation (secondary or mixed type), or less commonly, in extravascular hematomas (undetermined or extravascular type).…”

“…[13][14][15] IPEH in the petrous bone include lesions in the internal auditory meatus, 2 jugular foramen, 19 and Meckel's cave. 16 IPEH in the brain parenchyma (n = 9 [39.1%]) has been reported with varying prevalence in the parietal (n = 4 [44.4%]), [3][4][5]12 frontal (n = 3 [33.3%]), 4,5,17 temporal (n = 2 [22.2%]), 12,15 and occipital (n = 2 [22.2%]) 5,7 lobes and in the posterior fossa (n = 2 [22.2%]), cerebellum, and brainstem, 6,14 including cases involving multiple lesions. Two cases (8.7%) were confined purely to the intravascular space (in an aneurysm of the posterior inferior cerebellar artery and in the torcular herophili), 9,18 without extending into the brain parenchyma and bony structures.…”

“…Given that the most common location is the base of the skull, from which cranial nerves exit through foramina, the most common symptoms of intracranial IPEH are single or multiple cranial nerve palsies (n = 12 [52.2%]). [1][2][3]5,7,8,13,19 Increased intracranial pressure is the second most common presenting manifestation (n = 8 [34.8%]); [3][4][5][6]13,15,16,18 followed by seizure (n = 5 [21.7%]); 3,4,7,12,17 local mass effect, exclusive of cranial nerve effects, including proptosis, ataxia, and hemiparesis (n = 5 [21.7%]); 6,7,14,15,17 and intracranial hemorrhage (n = 2 [8.7%]). 7,17 Interestingly, intracranial hypertension secondary to venous outflow obstruction because of IPEH in the venous sinus was reported in 1 case.…”

“…To date, only 22 cases of intracranial IPEH have been reported. [1][2][3][4][5][6][7][8][9][12][13][14][15][16][17][18][19] Because of its rarity, the natural history and treatment guidelines for intracranial IPEH have not been fully elucidated. Moreover, to our knowledge long-term follow-up data after surgical treatment of intracranial IPEH have not previously been published.…”