TY - JOUR
T1 - IgG4-Related Disease of the Skull and Skull Base–A Systematic Review and Report of Two Cases
AU - Cler, Samuel J.
AU - Sharifai, Nima
AU - Baker, Brandi
AU - Dowling, Joshua L.
AU - Pipkorn, Patrik
AU - Yaeger, Lauren
AU - Clifford, David B.
AU - Dahiya, Sonika
AU - Chicoine, Michael R.
N1 - Funding Information:
Conflict of interest statement: M.R.C. received funding from 1) IMRIS Inc . for an unrestricted educational grant to support an iMRI database and outcomes analysis project, the IMRIS Muliticenter intraoperative MRI Neurosurgery Database (I-MiND), 2) The Head for the Cure Foundation , and 3) Mrs. Carol Rossfeld and The Alex & Alice Aboussie Family Charitable Foundation . I-MiND is maintained in The REDCap server at Washington University in St. Louis and is supported by Clinical and Translational Science Award (CTSA) Grant ( UL1 TR000448 ) and The Siteman Comprehensive Cancer Center and NCI Cancer Center Support Grant P30 CA091842 . D.B.C. serves as a consultant for Biogen, Takeda, Millennium, Genzyme, Amgen, Roche/Genentech, Glaxo Smith Kline, Serono, Inhibikase, Dr Reddy's Lab, Bristol Myers Squibb, Atara, Mitsubishi Tanabe, Excision BioTherapeutics, Up to Date, Wolters Kluwer; Data Safety Monitoring Boards: Genentech/Roche, Wave, EMD Serono, Shire, Pfizer, Sanofi, offers legal consulting for Cook County, State Farm, Wilke & Wilke PC, Shevlin Smith, Sal Indomenico PC, and receives grant support from NIH : NIMH , NINDS , NIAID , NIA , NCATS . S.C. received funding from NIH T35 NHLBI Training Grant Fellowship. The authors have no personal financial or institutional interest in any of the drugs, materials, or devices described in this article.
Funding Information:
Conflict of interest statement: M.R.C. received funding from 1) IMRIS Inc. for an unrestricted educational grant to support an iMRI database and outcomes analysis project, the IMRIS Muliticenter intraoperative MRI Neurosurgery Database (I-MiND), 2) The Head for the Cure Foundation, and 3) Mrs. Carol Rossfeld and The Alex & Alice Aboussie Family Charitable Foundation. I-MiND is maintained in The REDCap server at Washington University in St. Louis and is supported by Clinical and Translational Science Award (CTSA) Grant (UL1 TR000448) and The Siteman Comprehensive Cancer Center and NCI Cancer Center Support Grant P30 CA091842. D.B.C. serves as a consultant for Biogen, Takeda, Millennium, Genzyme, Amgen, Roche/Genentech, Glaxo Smith Kline, Serono, Inhibikase, Dr Reddy's Lab, Bristol Myers Squibb, Atara, Mitsubishi Tanabe, Excision BioTherapeutics, Up to Date, Wolters Kluwer; Data Safety Monitoring Boards: Genentech/Roche, Wave, EMD Serono, Shire, Pfizer, Sanofi, offers legal consulting for Cook County, State Farm, Wilke & Wilke PC, Shevlin Smith, Sal Indomenico PC, and receives grant support from NIH: NIMH, NINDS, NIAID, NIA, NCATS. S.C. received funding from NIH T35 NHLBI Training Grant Fellowship. The authors have no personal financial or institutional interest in any of the drugs, materials, or devices described in this article.
Publisher Copyright:
© 2021
PY - 2021/6
Y1 - 2021/6
N2 - Objective: IgG4-related disease (IgG4-RD) is an inflammatory process that uncommonly can present in the skull base and calvarium and mimic a tumor but the nature of this condition is not well summarized in the neurosurgical literature. Methods: A review was performed of 2 cases of IgG4-RD in the skull base highlighting the diagnostic challenges with assessment of these skull base lesions, and a systematic review of relevant literature was carried out. Results: A systematic review of the literature conducted in accordance with PRISMA guidelines identified 113 articles, with 184 cases of IgG4-RD in the skull base or calvarium. The most commonly affected locations include the meninges, cavernous sinus, base of the posterior fossa, clivus, and mastoid bone. Headache, visual and auditory disturbances, cranial nerve dysfunction, and seizures were the most common presenting symptoms. Medical treatment was highly successful and most commonly consisted of corticosteroids coadministered with immunosuppressive agents such as rituximab. Prevalence seemed to be equal between sexes, and serum IgG4 levels were increased in 61% of patients. Delayed diagnosis and a need for multiple biopsies were reported in numerous cases. Two cases of skull base IgG4-RD from the authors' institution show the variable presentations of this disease. More invasive surgical biopsies were required in both cases, and corticosteroid treatment led to significant clinical improvement. Conclusions: IgG4-RD is an uncommon condition with an increasing body of reported cases that can affect the skull base and calvarium and should be in the differential diagnosis, because delay in diagnosis and treatment may be common.
AB - Objective: IgG4-related disease (IgG4-RD) is an inflammatory process that uncommonly can present in the skull base and calvarium and mimic a tumor but the nature of this condition is not well summarized in the neurosurgical literature. Methods: A review was performed of 2 cases of IgG4-RD in the skull base highlighting the diagnostic challenges with assessment of these skull base lesions, and a systematic review of relevant literature was carried out. Results: A systematic review of the literature conducted in accordance with PRISMA guidelines identified 113 articles, with 184 cases of IgG4-RD in the skull base or calvarium. The most commonly affected locations include the meninges, cavernous sinus, base of the posterior fossa, clivus, and mastoid bone. Headache, visual and auditory disturbances, cranial nerve dysfunction, and seizures were the most common presenting symptoms. Medical treatment was highly successful and most commonly consisted of corticosteroids coadministered with immunosuppressive agents such as rituximab. Prevalence seemed to be equal between sexes, and serum IgG4 levels were increased in 61% of patients. Delayed diagnosis and a need for multiple biopsies were reported in numerous cases. Two cases of skull base IgG4-RD from the authors' institution show the variable presentations of this disease. More invasive surgical biopsies were required in both cases, and corticosteroid treatment led to significant clinical improvement. Conclusions: IgG4-RD is an uncommon condition with an increasing body of reported cases that can affect the skull base and calvarium and should be in the differential diagnosis, because delay in diagnosis and treatment may be common.
KW - Calvarium
KW - IgG4-related disease
KW - Skull base
UR - http://www.scopus.com/inward/record.url?scp=85103988425&partnerID=8YFLogxK
U2 - 10.1016/j.wneu.2021.03.054
DO - 10.1016/j.wneu.2021.03.054
M3 - Review article
C2 - 33746107
AN - SCOPUS:85103988425
SN - 1878-8750
VL - 150
SP - 179-196.e1
JO - World Neurosurgery
JF - World Neurosurgery
ER -