Endolymphatic sac tumor: a rare cerebellopontine angle tumor

Abstract

Endolymphatic sac tumors (ELST) are rare papillary tumors of the temporal bone. Previously named as aggressive papillary middle ear tumors, they have recently been shown to arise from the endolymphatic sac. They are a rare in cerebello-pontine angle (CPA). We present a case of an ELST who presented as a CPA tumor with hydrocephalus. He underwent a ventriculo-peritoneal shunt initially. On exploration of the CP angle, the tumor was found to be extremely vascular. He was re-explored following embolization, and a subtotal excision of the tumor was done. Extensive petrous bone infiltration and vascularity of the tumor makes total excision almost impossible with high risk of cranial nerve deficits, excessive blood loss and CSF leak. This tumor should be considered in the differential diagnosis of vascular CPA tumors which erode the petrous temporal bone. The relevant literature is reviewed.