{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,27]],"date-time":"2026-01-27T16:53:37Z","timestamp":1769532817275,"version":"3.49.0"},"reference-count":18,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2019,12,16]],"date-time":"2019-12-16T00:00:00Z","timestamp":1576454400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["The Laryngoscope"],"published-print":{"date-parts":[[2020,11]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>IgG4\u2010related disease (IgG4\u2010RD) is a recently recognized disease characterized by fibroinflammatory infiltrates rich in IgG4+ plasma cells that can present as isolated tumor\u2010like lesions of the head and neck. The objective of the current study was to describe the cranial base manifestations of IgG4\u2010RD.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Review of all cases at three tertiary\u2010referral centers since disease description in 2003.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Eleven patients were identified at a median age at presentation of 58\u2009years (IQR, 38\u201365; 55% male). Ten (91%) patients had isolated skull base masses without systemic disease. Cranial neuropathies were commonly observed in the abducens (45%), trigeminal (18%), and facial nerves (18%). Lesions frequently involved the cavernous sinus (55%; 6\/11) with extension to the petroclival junction in 50% (3\/6). Infiltration of the internal auditory canal was present in 27% (3\/11) with one case demonstrating erosion of the bony labyrinth. Preliminary clinical diagnoses commonly included nasopharyngeal cancer, pituitary macroadenoma, cholesteatoma, and meningioma \/ multiple meningioma syndrome. Local biopsy demonstrated &gt;30 IgG4\u2010positive plasma cells per high\u2010powered field or an IgG4:IgG ratio greater than 40% in all cases. Rapid and durable clinical improvement was seen in 91% following corticosteroid and rituximab therapy.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>IgG4\u2010RD nonspecifically presents as a rare cause of the skull base mass. Often presenting without concomitant systemic disease, local diagnostic biopsies are required. Obtaining adequate tissue specimen is complicated by densely fibrotic cranial base lesions that are frequently in close proximity to critical neurovascular structures. Primary medical therapy with corticosteroids and rituximab is effective in most patients.<\/jats:p><\/jats:sec><jats:sec><jats:title>Level of Evidence<\/jats:title><jats:p>4 <jats:italic>Laryngoscope<\/jats:italic>, 130:2574\u20132580, 2020<\/jats:p><\/jats:sec>","DOI":"10.1002\/lary.28478","type":"journal-article","created":{"date-parts":[[2019,12,16]],"date-time":"2019-12-16T13:35:37Z","timestamp":1576503337000},"page":"2574-2580","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":19,"title":["Manifestations of Skull Base IgG4\u2010Related Disease: A Multi\u2010Institutional Study"],"prefix":"10.1002","volume":"130","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-2350-8942","authenticated-orcid":false,"given":"John P.","family":"Marinelli","sequence":"first","affiliation":[{"name":"Department of Otolaryngology\u2013Head and Neck Surgery Mayo Clinic  Rochester Minnesota U.S.A."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Chiara","family":"Marvisi","sequence":"additional","affiliation":[{"name":"Department of Rheumatology University of Modena and Reggio Emilia  Modena Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Augusto","family":"Vaglio","sequence":"additional","affiliation":[{"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":[{"role":"author","vocabulary":"crossref"}]},{"given":"Pierce A.","family":"Peters","sequence":"additional","affiliation":[{"name":"Department of Neurologic Surgery Mayo Clinic  Rochester Minnesota U.S.A."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eric M.","family":"Dowling","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology\u2013Head and Neck Surgery Mayo Clinic  Rochester Minnesota U.S.A."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alessandro A.","family":"Palumbo","sequence":"additional","affiliation":[{"name":"Department of Rheumatology University of Modena and Reggio Emilia  Modena Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"John I.","family":"Lane","sequence":"additional","affiliation":[{"name":"Department of Radiology Mayo Clinic  Rochester Minnesota U.S.A."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Eric N.","family":"Appelbaum","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology\u2013Head and Neck Surgery Baylor College of Medicine  Houston Texas U.S.A."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1551-1893","authenticated-orcid":false,"given":"Alex D.","family":"Sweeney","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology\u2013Head and Neck Surgery Baylor College of Medicine  Houston Texas U.S.A."}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4925-8593","authenticated-orcid":false,"given":"Matthew L.","family":"Carlson","sequence":"additional","affiliation":[{"name":"Department of Otolaryngology\u2013Head and Neck Surgery Mayo Clinic  Rochester Minnesota U.S.A."},{"name":"Department of Neurologic Surgery Mayo Clinic  Rochester Minnesota 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