{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,7]],"date-time":"2026-08-07T17:50:46Z","timestamp":1786125046521,"version":"build-2736575974"},"reference-count":34,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"4","license":[{"start":{"date-parts":[[2019,3,4]],"date-time":"2019-03-04T00:00:00Z","timestamp":1551657600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/academic.oup.com\/journals\/pages\/open_access\/funder_policies\/chorus\/standard_publication_model"}],"funder":[{"name":"Mayo Clinic Institutional Review Board","award":["IRB 18\u2013003355"],"award-info":[{"award-number":["IRB 18\u2013003355"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Neurosurg.","NEUROS"],"published-print":{"date-parts":[[2019,10]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>BACKGROUND<\/jats:title>\n                    <jats:p>Erdheim-Chester disease (ECD) is a rare, non-Langerhans cell histiocytosis. Up to 50% of patients develop central nervous system involvement, and a subset of these patients can present with isolated tumor-like masses.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>OBJECTIVE<\/jats:title>\n                    <jats:p>To describe the skull base manifestations of ECD with an emphasis on aspects most pertinent to surgeons who may be referred such patients for primary evaluation.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>METHODS<\/jats:title>\n                    <jats:p>Scopus, Web of Science, and PubMed were searched from database inception to May 1, 2018 for articles reporting skull base ECD. An institutional retrospective analysis of all patients treated at the authors\u2019 institution since January 1, 1996 was also performed to supplement these data.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>RESULTS<\/jats:title>\n                    <jats:p>Of 465 retrieved articles, 18 studies totaling 20 patients met inclusion criteria. Institutional review identified an additional 7 patients. Collectively, the median age at diagnosis was 49 yr (interquartile range, 42-58) with a 4:1 male-to-female ratio. Patients frequently presented with diplopia (48%), headache (30%), dysarthria (22%), and vertigo or imbalance (22%), though trigeminal hypesthesia (11%), facial nerve paresis (7%), hearing loss (7%), and trigeminal neuralgia (7%) were also observed. ECD commonly mimicked meningioma (33%), trigeminal schwannoma (8%), neurosarcoidosis (8%), and skull base lymphoma (8%).<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>CONCLUSION<\/jats:title>\n                    <jats:p>Discrete skull base lesions frequently mimic more common pathology such as meningioma or cranial nerve schwannomas. Medical therapy comprises the initial treatment for symptomatic skull base disease. Surgical resection is not curative and the utility of surgical intervention is largely limited to biopsy to establish diagnosis and\/or surgical debulking to relieve mass effect.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1093\/neuros\/nyz027","type":"journal-article","created":{"date-parts":[[2019,1,28]],"date-time":"2019-01-28T23:08:39Z","timestamp":1548716919000},"page":"E693-E701","source":"Crossref","is-referenced-by-count":17,"title":["Skull Base Manifestations of Erdheim-Chester Disease: A Case Series and Systematic Review"],"prefix":"10.1227","volume":"85","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2350-8942","authenticated-orcid":false,"given":"John P","family":"Marinelli","sequence":"first","affiliation":[{"name":"Mayo Clinic School of Medicine, Mayo Clinic, Rochester, Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pierce A","family":"Peters","sequence":"additional","affiliation":[{"name":"Mayo Clinic School of Medicine, Mayo Clinic, Rochester, Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Augusto","family":"Vaglio","sequence":"additional","affiliation":[{"name":"Nephrology Unit, Parma University Hospital, Parma, Italy"},{"name":"Department of Biomedical Experimental and Clinical Sciences \u201cMario Serio\u201d, University of Firenze, Firenze, Italy"},{"name":"Nephrology and Dialysis Unit, Meyer Children's University Hospital, Firenze, Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jamie J","family":"Van Gompel","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, Minnesota"},{"name":"Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"John I","family":"Lane","sequence":"additional","affiliation":[{"name":"Department of Radiology, Mayo Clinic, Rochester, Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Matthew L","family":"Carlson","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology-Head and Neck Surgery, Mayo Clinic, Rochester, Minnesota"},{"name":"Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","published-online":{"date-parts":[[2019,10]]},"reference":[{"issue":"5","key":"bib1-20231011","doi-asserted-by":"crossref","first-page":"411","DOI":"10.1200\/JCO.2014.57.1950","article-title":"Reproducible and sustained efficacy of targeted therapy with vemurafenib in patients with BRAFV600E-mutated Erdheim-Chester disease","volume":"33","author":"Haroche","year":"2014","journal-title":"J Clin Oncol."},{"issue":"4","key":"bib2-20231011","doi-asserted-by":"crossref","first-page":"483","DOI":"10.1182\/blood-2014-03-561381","article-title":"Consensus guidelines for the diagnosis and clinical management of Erdheim-Chester disease","volume":"124","author":"Diamond","year":"2014","journal-title":"Blood"},{"issue":"10","key":"bib3-20231011","doi-asserted-by":"crossref","first-page":"1267","DOI":"10.1007\/s00415-006-0160-9","article-title":"Neurological manifestations and neuroradiological presentation of Erdheim-Chester disease: report of 6 cases and systematic review of the literature","volume":"253","author":"Lachenal","year":"2006","journal-title":"J Neurol."},{"issue":"5","key":"bib4-20231011","doi-asserted-by":"crossref","first-page":"639","DOI":"10.1016\/j.beem.2009.05.009","article-title":"Inflammatory and granulomatous expansive lesions of the pituitary","volume":"23","author":"Carpinteri","year":"2009","journal-title":"Best Pract Res Clin Endocrinol Metab."},{"issue":"2","key":"bib5-20231011","doi-asserted-by":"crossref","first-page":"e113","DOI":"10.1016\/S1470-2045(17)30031-1","article-title":"Histiocytoses: emerging neoplasia behind inflammation","volume":"18","author":"Haroche","year":"2017","journal-title":"Lancet Oncol."},{"issue":"3","key":"bib6-20231011","doi-asserted-by":"crossref","first-page":"179","DOI":"10.1159\/000360424","article-title":"Cerebral Erdheim-Chester disease mimicking high-grade glial tumor: a case report","volume":"49","author":"Eksi","year":"2013","journal-title":"Pediatr Neurosurg."},{"issue":"6","key":"bib7-20231011","doi-asserted-by":"crossref","first-page":"1115","DOI":"10.3171\/jns.2004.100.6.1115","article-title":"Erdheim-Chester disease mimicking a primary brain tumor. 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