Intracranial intravascular papillary endothelial hyperplasia

Intracranial intravascular papillary endothelial hyperplasia (IPEH) is very rare, and long-term follow-up results have not been previously published.

Reports of central nervous system involvement include intraparenchymal, sellar, cerebellar, brainstem, meningeal, venous sinus, calvarial, skull base, superior orbital fissure, and spinal cord/meningeal lesions.

see Pineal region intravascular papillary endothelial hyperplasia.

An 11-year-old boy presented with a 6-month history of progressive visual impairment in the right eye. Magnetic resonance imaging revealed a well-enhanced, large parasellar mass involving the cavernous sinus, right frontal skull base, and ethmoid and sphenoid sinuses. Frontotemporal craniotomy and subtotal resection were performed, and the diagnosis of IPEH was confirmed. The mass increased in size during the following 3 months. A second operation was performed via frontotemporal craniotomy combined with a transsphenoidal approach, and gross-total resection of the tumor was achieved. Adjuvant radiotherapy (5040 cGy) and chemotherapy with interferon were administered. The patient's visual symptoms improved, and there was no recurrence during a 13-year follow-up period. The results of this case indicate that intracranial IPEH can recur with subtotal resection; however, optimal resection with multimodal adjuvant treatment can control the disease for many years, if not permanently ¹⁾.