TY - JOUR
T1 - Current Treatment of Endolymphatic Sac Tumor of the Temporal Bone
AU - Mendenhall, William M.
AU - Suárez, Carlos
AU - Skálová, Alena
AU - Strojan, Primož
AU - Triantafyllou, Asterios
AU - Devaney, Kenneth O.
AU - Williams, Michelle D.
AU - Rinaldo, Alessandra
AU - Ferlito, Alfio
N1 - Publisher Copyright:
© 2018, The Author(s).
PY - 2018/7/1
Y1 - 2018/7/1
N2 - 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%.
AB - 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%.
KW - Endolymphatic sac tumor
KW - Heffner tumor
KW - Radiation therapy
KW - Surgery
KW - Temporal bone
KW - von Hippel–Lindau disease
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U2 - 10.1007/s12325-018-0730-0
DO - 10.1007/s12325-018-0730-0
M3 - Review article
C2 - 29923043
AN - SCOPUS:85048787322
SN - 0741-238X
VL - 35
SP - 887
EP - 898
JO - Advances in Therapy
JF - Advances in Therapy
IS - 7
ER -