{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,21]],"date-time":"2026-08-21T12:38:04Z","timestamp":1787315884889,"version":"build-2736575974"},"reference-count":20,"publisher":"Springer Science and Business Media LLC","issue":"1","content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Diagn Pathol"],"published-print":{"date-parts":[[2012,12]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Endolymphatic sac tumor (ELST) is a rare neoplasm which can be encountered sporadically or in Von Hippel-Lindau (VHL) disease. Here we report a sporadic case of ELST in 31-year-old man. Neither the symptoms nor a family history of VHL disease were found in the patient. CT imaging demonstrated an expansile lytic lesion of the mastoid process of the left petrous bone. MR scanning revealed a 5.2 cm \u00d7 4.7 cm \u00d7 4.2 cm mass which showed hyperintensity on T1- and T2-weighted images. Histologic sections showed a papillary, cystic or glandular architecture. The papillary and glandular structures were lined by a single layer of flattened cuboidal-to-columnar cells. The stroma of the papillary fronds was richly vascularized and chronically inflamed. The tumor showed diffusely positive reactivity with cytokeratin (Pan), cytokeratin 19, cytokeratin 5\/6, cytokeratin 7, EMA, vimentin, CD56, and NSE and also showed variable reactivity with glial fibrillary acidic protein (GFAP) and VEGF. The Ki-67 immunostain showed a proliferation index of &lt; 1%. Because the mass was large, it was difficult to extirpate surgically. After surgery, the patient underwent gamma-knife radiosurgery for residual tumor. The findings indicate that ELST is a rare neoplasm with benign histopathological appearance and clinically destructive behavior. Because of the rarity of this tumor, it can easily be confused with other tumors such as paraganglioma, middle ear adenoma, adenocarcinoma, papillary carcinoma of thyroid or choroid plexus papilloma. Owing to its locally aggressive nature, it is difficult to extirpate surgically when it is large.<\/jats:p>\n                  <jats:p>\n                    <jats:bold>Virtual Slides<\/jats:bold>\n                  <\/jats:p>\n                  <jats:p>\n                    The virtual slide(s) for this article can be found here:\n                    <jats:ext-link xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" xlink:href=\"http:\/\/www.diagnosticpathology.diagnomx.eu\/vs\/7973320646763012\" ext-link-type=\"uri\">http:\/\/www.diagnosticpathology.diagnomx.eu\/vs\/7973320646763012<\/jats:ext-link>\n                  <\/jats:p>","DOI":"10.1186\/1746-1596-7-36","type":"journal-article","created":{"date-parts":[[2012,4,2]],"date-time":"2012-04-02T10:16:10Z","timestamp":1333361770000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":32,"title":["Endolymphatic sac tumor: case report and review of the literature"],"prefix":"10.1186","volume":"7","author":[{"given":"Yan-Hua","family":"Sun","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wen","family":"Wen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jun-Hui","family":"Wu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jian-Ming","family":"Song","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Hong","family":"Guan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kai-Xin","family":"Wang","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mei-Quan","family":"Xu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2012,4,2]]},"reference":[{"key":"543_CR1","doi-asserted-by":"publisher","first-page":"782","DOI":"10.1097\/00129492-200409000-00022","volume":"25","author":"ME Kupferman","year":"2004","unstructured":"Kupferman ME, Bigelow DC, Carpentieri DF, et al.: Endolymphatic sac tumor in a 4-year-old boy. Otol Neurotol. 2004, 25: 782-786. 10.1097\/00129492-200409000-00022.","journal-title":"Otol Neurotol"},{"key":"543_CR2","doi-asserted-by":"publisher","first-page":"773","DOI":"10.1097\/00129492-200409000-00021","volume":"25","author":"NC Bambakidis","year":"2004","unstructured":"Bambakidis NC, Megerian CA, Ratcheson RA: Differential grading of endolymphatie sac tumor extension by virtue of von Hippel-Lindau disease status. Otol Neurotol. 2004, 25: 773-781. 10.1097\/00129492-200409000-00021.","journal-title":"Otol Neurotol"},{"key":"543_CR3","doi-asserted-by":"publisher","first-page":"599","DOI":"10.1016\/j.prp.2008.01.016","volume":"204","author":"A Skalova","year":"2008","unstructured":"Skalova A, Sima R, Bohus P, et al.: Endolymphatic sac tumor (aggressive papillary tumor of middle ear and temporal bone) Report of two cases with analysis of the VHL gene. Pathol Res Pract. 2008, 204: 599-606. 10.1016\/j.prp.2008.01.016.","journal-title":"Pathol Res Pract"},{"key":"543_CR4","doi-asserted-by":"publisher","first-page":"790","DOI":"10.1097\/00000478-198810000-00009","volume":"12","author":"MJ Gaffey","year":"1988","unstructured":"Gaffey MJ, Mills SE, Fechner RE, et al.: Aggressive papillary middle-ear tumor, A clinicopathologic entity distinct from middle-ear adenoma. Am J Surg Pathol. 1988, 12: 790-797. 10.1097\/00000478-198810000-00009.","journal-title":"Am J Surg Pathol"},{"key":"543_CR5","first-page":"349","volume":"20","author":"D Cazals-Hatem","year":"2000","unstructured":"Cazals-Hatem D, Henin D, Bouccara D, et al.: Endolymphatic sac tumor: a rare tumor of internal ear. Report of two cases. Ann Pathol. 2000, 20: 349-352.","journal-title":"Ann Pathol"},{"key":"543_CR6","doi-asserted-by":"publisher","first-page":"421","DOI":"10.3171\/JNS-07\/08\/0421","volume":"107","author":"J Jagannathan","year":"2007","unstructured":"Jagannathan J, Butman JA, Lonser RR, et al.: Endolymphatic sac tumor demonstrated by intralabyrinthine hemorrhage. Case report. Neurosurgery. 2007, 107: 421-425. 10.3171\/JNS-07\/08\/0421.","journal-title":"Neurosurgery"},{"key":"543_CR7","doi-asserted-by":"publisher","first-page":"268","DOI":"10.3340\/jkns.2008.44.4.268","volume":"44","author":"CW Bae","year":"2008","unstructured":"Bae CW, Cho YH, Chung JW, et al.: Endolymphatic sac tumors: report of four cases. J Korean Neurosurg Soc. 2008, 44: 268-272. 10.3340\/jkns.2008.44.4.268.","journal-title":"J Korean Neurosurg Soc"},{"key":"543_CR8","first-page":"75","volume":"47","author":"K Krzysztolik","year":"2009","unstructured":"Krzysztolik K, Cybulski C, Sagan L, et al.: Endolymphatic sac tumours and von Hippel-Lindau disease - case report, molecular analysis and histopathological characterization. Folia Neuropathol. 2009, 47: 75-80.","journal-title":"Folia Neuropathol"},{"key":"543_CR9","first-page":"168","volume":"17","author":"VT Ho","year":"1996","unstructured":"Ho VT, Rao VM, Doan HT, et al.: Low-grade adenocarcinoma of probable endolymphatic sac origin: CT and MR appearance. AJNR Am J Neuroradiol. 1996, 17: 168-170.","journal-title":"AJNR Am J Neuroradiol"},{"key":"543_CR10","doi-asserted-by":"publisher","first-page":"480","DOI":"10.3171\/jns.2004.100.3.0480","volume":"100","author":"D Choo","year":"2004","unstructured":"Choo D, Shotland L, Mastroianni M, et al.: Endolymphatic sac tumors in von Hippel-Lindau disease. J Neurosurg. 2004, 100: 480-487. 10.3171\/jns.2004.100.3.0480.","journal-title":"J Neurosurg"},{"key":"543_CR11","doi-asserted-by":"publisher","first-page":"2478","DOI":"10.1002\/(SICI)1097-0142(19991201)86:11+<2478::AID-CNCR4>3.0.CO;2-5","volume":"86","author":"CA Friedrich","year":"1999","unstructured":"Friedrich CA: Von Hippel-Lindau syndrome. A pleomorphic condition. Cancer. 1999, 86: 2478-2482. 10.1002\/(SICI)1097-0142(19991201)86:11+<2478::AID-CNCR4>3.0.CO;2-5.","journal-title":"Cancer"},{"key":"543_CR12","doi-asserted-by":"publisher","first-page":"1272","DOI":"10.1053\/hupa.2001.28961","volume":"32","author":"S Hamazaki","year":"2001","unstructured":"Hamazaki S, Yoshida M, Yao M, et al.: Mutation of von Hippel-Lindau tumor suppressor gene in a sporadic endolymphatic sac tumor. Hum Pathol. 2001, 32: 1272-1276. 10.1053\/hupa.2001.28961.","journal-title":"Hum Pathol"},{"key":"543_CR13","doi-asserted-by":"crossref","first-page":"280","DOI":"10.1177\/019459988309100314","volume":"91","author":"DE Schuller","year":"1983","unstructured":"Schuller DE, Conley JJ, Goodman JH, et al.: Primary adenocarcinoma of the middle ear. Otolaryngol Head Neck Surg. 1983, 91: 280-283.","journal-title":"Otolaryngol Head Neck Surg"},{"key":"543_CR14","doi-asserted-by":"publisher","first-page":"47","DOI":"10.1288\/00005537-198201000-00010","volume":"92","author":"JF Pallanch","year":"1982","unstructured":"Pallanch JF, McDonald TJ, Weiland LH, et al.: Adenocarcinoma and adenoma of the middle ear. Laryngoscope. 1982, 92: 47-53.","journal-title":"Laryngoscope"},{"key":"543_CR15","first-page":"213","volume":"13","author":"AD Hassard","year":"1984","unstructured":"Hassard AD, Boudreau SF, Cron CC: Adenoma of the endolymphatic sac. Otolaryngol J. 1984, 13: 213-216.","journal-title":"Otolaryngol J"},{"key":"543_CR16","doi-asserted-by":"publisher","first-page":"2292","DOI":"10.1002\/1097-0142(19891201)64:11<2292::AID-CNCR2820641119>3.0.CO;2-#","volume":"64","author":"DK Heffner","year":"1989","unstructured":"Heffner DK: Low-grade adanoearcinoma of probable endolymphatic sac orisin a elinicopathologic study of 20 cases. Cancer. 1989, 64: 2292-2302. 10.1002\/1097-0142(19891201)64:11<2292::AID-CNCR2820641119>3.0.CO;2-#.","journal-title":"Cancer"},{"key":"543_CR17","doi-asserted-by":"publisher","first-page":"2481","DOI":"10.1056\/NEJMoa040666","volume":"350","author":"RR Lonser","year":"2004","unstructured":"Lonser RR, Kim HJ, Butman JA, et al.: Tumors of the endolymphatic sac in von Hippel-Lindau disease. N Engl J Med. 2004, 350: 2481-2486. 10.1056\/NEJMoa040666.","journal-title":"N Engl J Med"},{"key":"543_CR18","doi-asserted-by":"publisher","first-page":"104","DOI":"10.1007\/s12105-007-0016-3","volume":"1","author":"L Michaels","year":"2007","unstructured":"Michaels L: Origin of endolymphatic sac tumor. Head Neck Pathol. 2007, 1: 104-111. 10.1007\/s12105-007-0016-3.","journal-title":"Head Neck Pathol"},{"key":"543_CR19","doi-asserted-by":"publisher","first-page":"117","DOI":"10.1016\/j.anndiagpath.2010.08.009","volume":"15","author":"D Bell","year":"2011","unstructured":"Bell D, Gidley P, Levine N, et al.: Endolymphatic sac tumor (aggressive papillary tumor of middle ear and temporal bone): sine qua non radiology-pathology and the University of Texas MD Anderson Cancer Center experience. Ann Diagn Pathol. 2011, 15: 117-123. 10.1016\/j.anndiagpath.2010.08.009.","journal-title":"Ann Diagn Pathol"},{"key":"543_CR20","doi-asserted-by":"publisher","first-page":"216","DOI":"10.1097\/00005537-199702000-00014","volume":"107","author":"CA Megerian","year":"1997","unstructured":"Megerian CA, Pilch BZ, Bhan AK, et al.: Differential expression of transthyretin in papillary tumors of the endolymphatic sac and choroid plexus. Laryngoscope. 1997, 107: 216-221. 10.1097\/00005537-199702000-00014.","journal-title":"Laryngoscope"}],"container-title":["Diagnostic Pathology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1186\/1746-1596-7-36.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2021,9,1]],"date-time":"2021-09-01T15:05:23Z","timestamp":1630508723000},"score":1,"resource":{"primary":{"URL":"https:\/\/diagnosticpathology.biomedcentral.com\/articles\/10.1186\/1746-1596-7-36"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2012,4,2]]},"references-count":20,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2012,12]]}},"alternative-id":["543"],"URL":"https:\/\/doi.org\/10.1186\/1746-1596-7-36","relation":{},"ISSN":["1746-1596"],"issn-type":[{"value":"1746-1596","type":"electronic"}],"subject":[],"published":{"date-parts":[[2012,4,2]]},"assertion":[{"value":"16 February 2012","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"2 April 2012","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"2 April 2012","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}}],"article-number":"36"}}