Papillary Endothelial Hyperplasia of the Sella, Cavernous Sinus, Ethmoid and Sphenoid Sinus

ABSTRACT

Papillary endothelial hyperplasia(PEH), usually originates in extracranial soft tissue, is considered a reactive proliferation of endothlium and an unusual form of thrombus organization. However the occurance of PEH in the cranial cavity is very rare. The authors report a case of a PEH involving intracranial sella, parasella, cavernous sius, ethmoid and sphenoid sinus in an 11-year-old boy. The patient was presented with sudden right visual disturbance. Magnetic resonance image showed a well-enhanced mass in the right cavernous sinus extending into the ethmoid and sphenoid sinus. The cerebral angiography demonstrated a vigorous tumor staining supplied by both internal and external carotid arteries. The right fronto-temporal craniotomy and subtotal resection of the tumor were performed. The mass was comprised in a highly vascular red-black nodular tissue associated with a mixture of fresh and organizing thrombi. The histopathological examination revealed papillary structures lined by endothelium within vascular space with organized thrombus. While the patient was waiting for second operation in order to remove remaining small tumor in the ethmoid and sphenoid sinues, the lesion exhibited rapid regrowth and the size became to the preoperative size at three months after operation. At reoperation, gross total removal was accomplished by a combination of trans-cranial and trans-sphenoidal approaches. Postoperative radiotherapy was given.