Current Treatment of Endolymphatic Sac Tumor of the Temporal Bone

Abstract

An endolymphatic sac tumor (ELST) is a rare, indolent but locally aggressive tumor arising in the posterior petrous ridge. Patients present with sensorineural hearing loss and tinnitus. As the tumor progresses, patients may experience vertigo, ataxia, facial nerve paresis, pain and otorrhea. Most patients present in their 4th or 5th decade with a wide age range. Patients with von Hippel-Lindau disease have an increased likelihood of developing ELST. Histologically, ELST is a low-grade adenocarcinoma. As it progresses, it destroys bone and extends into adjacent tissues. The likelihood of regional or distant metastases is remote. The optimal treatment is resection with negative margins. Patients with positive margins, gross residual disease, or unresectable tumor are treated with radiotherapy or radiosurgery. Late recurrences are common, so long follow-up is necessary to assess efficacy. The likelihood of cure depends on tumor extent and is probably in the range of 50-75%.

Papillary Mucinous Adenocarcinoma of the Endolymphatic Sac: A Rare Middle Ear Neoplasm

“…The different parts of our body are derived from three different germ layers. Similarly, the membranous labyrinth and endolymphatic sac (ELS) are derived from the ectoderm and are found postero-medial to the temporal bone [ 1 , 2 ]. The endolymphatic duct connects with the proximal part of the ELS and the posterior segment of the ELS contiguous with the posterior cranial fossa [ 3 ].…”

“…ELS tumors (ELSTs) are extremely uncommon, with only around 300 cases published in the literature [ 1 ]. The purpose of this case report is to discuss the histopathological presentation, diagnosis, and treatment of this rare entity.…”

“…Because of the sensitive location of this tumor, the adjuvant radiotherapy options are still questionable. In this case report, the author presents a 74-year-old woman with a past medical history of Schneiderian papilloma and was diagnosed with papillary mucinous adenocarcinoma of the ELS not associated with VHL disease.Categories: Pathology, Oncology Keywords: endolymphatic sac tumor, papillary mucinous adenocarcinoma, glandular neoplasm, middle ear tumor, neoplasm of temporal region and mastoids air cells ELS tumors (ELSTs) are extremely uncommon, with only around 300 cases published in the literature [1]. The purpose of this case report is to discuss the histopathological presentation, diagnosis, and treatment of this rare entity.…”

“…Categories: Pathology, Oncology Keywords: endolymphatic sac tumor, papillary mucinous adenocarcinoma, glandular neoplasm, middle ear tumor, neoplasm of temporal region and mastoids air cells ELS tumors (ELSTs) are extremely uncommon, with only around 300 cases published in the literature [1]. The purpose of this case report is to discuss the histopathological presentation, diagnosis, and treatment of this rare entity.…”

The biomechanical characteristics of human vestibular aqueduct: a numerical-based model construction and simulation

“…As the bony tunnel inside the petrous pyramid of temporal bone, vestibular aqueduct (VA) starts from the medial wall of inner ear vestibule, extends parallel to the common crus, passes the anterior opening of sigmoid sinus and terminates at the posterior cranial fossa (Alemi and Chan 2015;Clar os et al 2017). VA is filled with endolymphatic ducts connecting endolymphatic sac, inner ear vestibule and cochlea together, which plays a critical role in maintaining the metabolism and environment of membranous labyrinth and inner ear by draining the metabolic products in semicircular canals and utricle into sigmoid sinus (Ferster et al 2017;Mendenhall et al 2018). Therefore, VA is also putatively regarded as a crucial structure to ensure the circulation between endolymphma and perilympha, which deficiency can impair the nutrition of inner ear cells, the sonic transduction to hairy cells and balance sensation of sensory cells.…”