Stereotactic radiosurgery for the treatment of recurrent endolymphatic sac tumor: A case report and review of the literature
Nisha Dabhi, Stylianos Pikis, Georgios Mantziaris and Jason Sheehan
Endolymphatic sac tumors (ELST) are benign, slowly progressive, highly vascular papillary adenomatous neoplasms that originate from the epithelium of the endolymphatic sac [1-2]. ELSTs may occur sporadically or as a manifestation of von Hippel-Lindau (VHL) disease. While sporadic cases of ELSTs occur in 1:30,000 adults, there is a 3.6%-16% prevalence of ELSTs in patients with VHL [2].
ELSTs are invasive and destructive to local structures such as the temporal bone and labyrinth. Depending on their location and size, ELST may present as ipsilateral sensorineural hearing loss (SNHL), vertigo, tinnitus, or facial nerve palsy [1-2]. Invasion of the middle and posterior cranial fossa, and clivus, may cause cerebellar signs and symptoms and lower cranial nerve palsies [1-2].
Gross total resection (GTR) is the preferred management of ELST, as it often leads to long disease-free intervals and is associated with a 90% long-term cure rate [2-3]. However, GTR is often not possible in large, locally invasive tumors adjacent to or involving critical neurovascular structures, in which case subtotal resection (STR) is employed [2-4]. STR is associated with an increased risk of tumor recurrence and spread [2-4]. Non-invasive treatment options for patients with ELST are limited. Specifically, the exact role of stereotactic radiosurgery (SRS) in the management of ELST is not well defined.
Keywords: Endolymphatic sac tumor, stereotactic radiosurgery, Gamma Knife
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