Intravascular Papillary Endothelial Hyperplasia of the Central Nervous System-Four Case Reports-

Abstract

Four rare cases of intracranial intravascular papillary endothelial hyperplasia (IPEH) manifesting as cranial nerve disturbances occurred in 16-, 18-, 24-, and 28-year-old females. Magnetic resonance imaging showed all lesions as isointense with strong enhancement on T 1 -weighted images, and as hyperintense on T 2 -weighted images. All lesions were removed via craniotomies. Histological examination found vascular structures and papillary spaces lined with endothelial cells showing immunoreactivity for CD31. Complete removal was curative in two cases, whereas incomplete removal resulted in cure in one case and residual deficits in one case. Iatrogenic deficits should be avoided in IPEH treatment by surgery. Differentiation from neoplasm such as angiosarcoma depends on histological characteristics.

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

“…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). 3,6,9,14,19 Reported cases, including the present one, indicate that the age at presentation of symptoms of intracranial IPEH can range from 2 days to 75 years of age, with biphasic peaks in the 2nd to 3rd decade and in the 6th decade of life (Table 1). There is a strong female predominance (19 [82.6%] of 23 reported cases) in the incidence of intracranial IPEH, and a less obvious female predominance in IPEH at other locations.…”

“…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).…”

“…3,6,11,14,19 IPEH can occur in various parts of the body, including a number of internal organs (gastrointestinal tract, kidney, liver, lung, uterus, etc. ), head and neck structures (nasal cavity, pharynx, and larynx), and most commonly, in skin and subcutaneous tissue of the upper extremities.…”

“…), head and neck structures (nasal cavity, pharynx, and larynx), and most commonly, in skin and subcutaneous tissue of the upper extremities. 3,6,14,19 While extracranial IPEH usually presents as a slow-growing painful benign nodule, 6,14 IPEH involving intracranial structures sometimes leads to serious local compressive symptoms, such as cranial nerve palsy, or a potentially fatal increase in intracranial pressure. To date, only 22 cases of intracranial IPEH have been reported.…”

“…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.…”