{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,15]],"date-time":"2026-05-15T01:13:50Z","timestamp":1778807630673,"version":"3.51.4"},"reference-count":29,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2025,1,13]],"date-time":"2025-01-13T00:00:00Z","timestamp":1736726400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Oncol."],"abstract":"<jats:sec><jats:title>Introduction<\/jats:title><jats:p>Endolymphatic sac tumor (ELST) is a rare neoplasm that exhibits aggressive growth primarily in the endolymphatic capsule and can potentially affect nearby neurovascular structures. The diagnosis of ELST poses challenges due to its low prevalence, gradual progression, and nonspecific symptomatology. It is currently believed that prompt surgical intervention is recommended for endolymphatic sac tumors upon diagnosis. In cases where the lesion is complex or in close proximity to vital blood vessels and nerves, making complete resection challenging, adjuvant therapy may be employed postoperatively. This approach aims to enhance treatment outcomes.<\/jats:p><\/jats:sec><jats:sec><jats:title>Case presentation<\/jats:title><jats:p>A case of a 53-year-old male was admitted to the Department of Neurosurgery of Shenzhen People\u2019s Hospital with the main cause of dizziness and blurred vision, and was found to have an occupation in the pontocerebellar angle for about 20 days. Preoperative cranial CT suggested that the temporal bone mastoid was widely damaged with worm-like bone, and some of them showed honeycomb changes. MRI suggested that shadow was seen in the posterior part of the left internal auditory canal, temporal bone mastoid and jugular foramen, with a size of about 28x21x27mm.The T1-weighted image showed low and equal signals, and the T2-weighted image showed equal and slightly low signals in the center with multicompartmental cystic high signals in the margin. The center of the mass strengthened significantly after enhancement. The patient had no other clinical manifestations and no family history. The clinical diagnosis was left-sided pontocerebellar angle occupation - nature to be determined. The occupation was resected microscopically using the distal lateral combined anterior approach to the sigmoid sinus. Due to the extremely rich blood supply of the tumor, the tumor was embedded in the petrous humerus, which was soft and tough, and the surrounding structures were not clearly displayed, making surgical resection extremely difficult. postoperative pathology and immunohistochemistry confirmed that this lesion was an endolymphatic cystic tumor. After surgery, most of the tumor was successfully resected while preserving neurological function well, and the residual tumor was treated with adjuvant gamma knife with good results.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>ELST is a rare, low-grade, locally aggressive tumor that is difficult to diagnose early. During the surgery, it was observed that the tumor had a significant blood supply, which made its removal challenging. Preoperative embolization of the tumor\u2019s blood supply artery would facilitate surgical resection and help avoid massive intraoperative bleeding. Complete surgical resection is the treatment of choice, and any remaining tumor remnants can be managed with adjuvant radiotherapy post-surgery, necessitating long-term follow-up to monitor any developments.<\/jats:p><\/jats:sec>","DOI":"10.3389\/fonc.2024.1463526","type":"journal-article","created":{"date-parts":[[2025,1,13]],"date-time":"2025-01-13T06:11:20Z","timestamp":1736748680000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":3,"title":["Case report: Endolymphatic sac tumor with blurred vision"],"prefix":"10.3389","volume":"14","author":[{"given":"Ke","family":"Yuan","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Weijian","family":"Luo","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jia","family":"Chen","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Quanzhou","family":"Peng","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Xiaoguang","family":"Tong","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jiliang","family":"Hu","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2025,1,13]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.14639\/0392-100x-1402","article-title":"Endolymphatic sac tumour in von Hippel-Lindau disease: management strategies","volume":"37","author":"Zanoletti","year":"2017","journal-title":"Acta otorhinolaryngologica Italica"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.1051\/medsci\/2004203304","article-title":"The endolymphatic sac: its roles in the inner ear","volume":"20","author":"Couloigner","year":"2004","journal-title":"Medecine sciences: M\/S"},{"key":"B3","doi-asserted-by":"publisher","first-page":"42","DOI":"10.25259\/sni_1168_2022","article-title":"Endolymphatic sac: A surreptitious anatomy for neurosurgeons","volume":"14","author":"Mohammad","year":"2023","journal-title":"Surg Neurol Int"},{"key":"B4","article-title":"Adenoma of the endolymphatic sac","volume":"13","author":"Hassard","year":"1984","journal-title":"J\u00a0Otolaryngol"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1097\/00000478-198810000-00009","article-title":"Aggressive papillary middle-ear tumor. A clinicopathologic entity distinct from middle-ear adenoma","volume":"12","author":"Gaffey","year":"1988","journal-title":"Am J Surg Pathol"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1186\/s41016-020-00191-4","article-title":"Endolymphatic sac tumor: case report and literature review","volume":"6","author":"Ge","year":"2020","journal-title":"Chin neurosurgical J"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1016\/j.rpor.2015.06.003","article-title":"Treatment of endolymphatic sac tumour (Papillary adenocarcinoma) of the temporal bone","volume":"21","author":"Poletti","year":"2016","journal-title":"Rep Pract Oncol Radiother"},{"key":"B8","doi-asserted-by":"publisher","first-page":"2","DOI":"10.1046\/j.1365-2559.1998.00460.x","article-title":"Endolymphatic sac tumours","volume":"33","author":"Kempermann","year":"1998","journal-title":"Histopathology"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.1007\/s00405-019-05511-8","article-title":"Clinicoradiologic characteristics of endolymphatic sac tumors","volume":"276","author":"Le","year":"2019","journal-title":"Eur Arch Oto-rhino-laryngol"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.5152\/iao.2017.2237","article-title":"Sporadic endolymphatic sac tumor-A very rare cause of hearing loss, tinnitus, and dizziness","volume":"13","author":"Schnack","year":"2017","journal-title":"J Int Advanced Otology"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.1038\/modpathol.3880380","article-title":"Endolymphatic sac tumor associated with a von Hippel-Lindau disease patient: an immunohistochemical study","volume":"14","author":"Horiguchi","year":"2001","journal-title":"Modern Pathol"},{"key":"B12","article-title":"Von Hippel-Lindau Syndrome","volume-title":"GeneReviews(\u00ae)","author":"van Leeuwaarde","year":"1993"},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.1177\/01945998221108313","article-title":"Systematic review of endolymphatic sac tumor treatment and outcomes","volume":"168","author":"Tang","year":"2023","journal-title":"Otolaryngology\u2013head Neck Surg"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.1177\/01455613221139408","article-title":"Endolymphatic sac tumor","volume":"14","author":"Lyons","year":"2022","journal-title":"Ear Nose Throat J"},{"key":"B15","doi-asserted-by":"publisher","first-page":"25","DOI":"10.1016\/j.anl.2012.01.009","article-title":"The Gruppo Otologico experience of endolymphatic sac tumor","volume":"40","author":"Husseini","year":"2013","journal-title":"Auris Nasus Larynx"},{"key":"B16","article-title":"Current opinions on diagnosis and management of endolymphatic sac tumors","volume-title":"Chin J Ophthalmol Otorhinolaryngol","author":"Chunfu","year":"2022"},{"key":"B17","doi-asserted-by":"publisher","DOI":"10.1053\/crad.2001.0898","article-title":"Radiological considerations in the diagnosis of an endolymphatic sac tumour","volume":"57","author":"Joy","year":"2002","journal-title":"Clin Radiol"},{"key":"B18","doi-asserted-by":"publisher","DOI":"10.1055\/s-2007-991116","article-title":"Tumors and pseudotumors of the endolymphatic sac","volume":"17","author":"Diaz","year":"2007","journal-title":"Skull Base"},{"key":"B19","doi-asserted-by":"publisher","DOI":"10.1002\/1097-0142(19891201)64:11<2292::aid-cncr2820641119>3.0.co;2-","article-title":"Low-grade adenocarcinoma of probable endolymphatic sac origin A clinicopathologic study of 20 cases [J","volume":"64","author":"Heffner","year":"1989","journal-title":"Cancer"},{"key":"B20","doi-asserted-by":"publisher","DOI":"10.1097\/00129492-200409000-00021","article-title":"Differential grading of endolymphatic sac tumor extension by virtue of von Hippel-Lindau disease status","volume":"25","author":"Bambakidis","year":"2004","journal-title":"Otology Neurotol"},{"key":"B21","doi-asserted-by":"publisher","DOI":"10.2310\/7070.2006.0082","article-title":"Endolymphatic sac tumours: surgical management","volume":"35","author":"Schipper","year":"2006","journal-title":"J Otolaryngol"},{"key":"B22","doi-asserted-by":"publisher","DOI":"10.1007\/s00405-020-06185-3","article-title":"Grading system and surgical approaches for endolymphatic sac tumors","volume":"278","author":"Li","year":"2021","journal-title":"Eur Arch Oto-rhino-laryngol"},{"key":"B23","doi-asserted-by":"publisher","DOI":"10.1002\/hed.22962","article-title":"Sporadic endolymphatic sac tumor: its clinical, radiological, and histological features, management, and follow-up","volume":"35","author":"Poletti","year":"2013","journal-title":"Head Neck"},{"key":"B24","doi-asserted-by":"publisher","first-page":"899","DOI":"10.1097\/mao.0000000000000299","article-title":"Management of endolymphatic sac tumors: sporadic cases and von Hippel-Lindau disease","volume":"35","author":"Nevoux","year":"2014","journal-title":"Otology Neurotol"},{"key":"B25","doi-asserted-by":"publisher","DOI":"10.1016\/B978-0-444-62702-5.00010-X","article-title":"Von Hippel-Lindau disease","volume":"132","author":"Chittiboina","year":"2015","journal-title":"Handb Clin Neurol"},{"key":"B26","doi-asserted-by":"publisher","DOI":"10.5152\/iao.2023.22957","article-title":"Surgical Management of Endolymphatic Sac Tumor: A Systematic Review and Meta-Analysis","volume":"19","author":"Gioacchini","year":"2023","journal-title":"J Int Adv Otol"},{"key":"B27","doi-asserted-by":"publisher","DOI":"10.1080\/00016489.2020.1722855","article-title":"Clinical features and treatment of endolymphatic sac tumor","volume":"140","author":"Bae","year":"2020","journal-title":"Acta Otolaryngol"},{"key":"B28","doi-asserted-by":"publisher","DOI":"10.1007\/s00701-002-0969-7","article-title":"Endolymphatic sac tumor: unique features of two cases and review of the literature","volume":"144","author":"Ferreira","year":"2002","journal-title":"Acta Neurochir (Wien)"},{"key":"B29","doi-asserted-by":"publisher","DOI":"10.1007\/s00405-012-2298-7","article-title":"Endolymphatic sac tumors: experience of three cases","volume":"270","author":"Bastier","year":"2013","journal-title":"Eur Arch Otorhinolaryngol"}],"container-title":["Frontiers in Oncology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fonc.2024.1463526\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,5,1]],"date-time":"2025-05-01T08:08:57Z","timestamp":1746086937000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fonc.2024.1463526\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,1,13]]},"references-count":29,"alternative-id":["10.3389\/fonc.2024.1463526"],"URL":"https:\/\/doi.org\/10.3389\/fonc.2024.1463526","relation":{},"ISSN":["2234-943X"],"issn-type":[{"value":"2234-943X","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,1,13]]},"article-number":"1463526"}}