title: IgG4-related disease initially presented as an orbital mass lesion mimicking optic nerve sheath meningioma.
abstract: We report a case of an optic nerve mass lesion associated with IgG4-related disease. A 39-year-old man presented with right blurred vision and proptosis 8 years before admission. Magnetic resonance imaging showed a mass lesion in the center of the right orbit, which was diagnosed as optic nerve sheath meningioma by neuroradiologists and neurosurgeons. Irradiation was selected for treatment of the lesion on the basis of the radiological diagnosis; subsequently, the lesion gradually reduced in size. However, regrowth of an optic nerve mass lesion observed during the previous 2 years caused remarkable exophthalmos, and removal of the orbital mass lesion was performed via a transcranial orbital approach. Pathological examinations resulted in a diagnosis of IgG4-related disease, and hematological tests revealed an elevated level of serum IgG4. Additional radiological examinations showed mass lesions in the left maxillary nerve, bilateral inferior alveolar nerves, paravertebral tissue, and left kidney. Treatment with oral steroids has produced a reduction in the size of these lesions.
authors.affiliation: Department of Neurosurgery, Sapporo Medical University, School of Medicine, South 1, West 16, Chuo-ku, Sapporo, Hokkaido, 060-8543, Japan.
authors.forename: Shouhei
authors.initials: S
authors.lastname: Noshiro
authors.affiliation: Department of Neurosurgery, Sapporo Medical University, School of Medicine, South 1, West 16, Chuo-ku, Sapporo, Hokkaido, 060-8543, Japan. wanibuti@sapmed.ac.jp.
authors.forename: Masahiko
authors.initials: M
authors.lastname: Wanibuchi
authors.affiliation: Department of Neurosurgery, Sapporo Medical University, School of Medicine, South 1, West 16, Chuo-ku, Sapporo, Hokkaido, 060-8543, Japan.
authors.forename: Yukinori
authors.initials: Y
authors.lastname: Akiyama
authors.affiliation: Department of Neurosurgery, Sapporo Medical University, School of Medicine, South 1, West 16, Chuo-ku, Sapporo, Hokkaido, 060-8543, Japan.
authors.forename: Satoshi
authors.initials: S
authors.lastname: Okawa
authors.affiliation: Department of Neurosurgery, Sapporo Medical University, School of Medicine, South 1, West 16, Chuo-ku, Sapporo, Hokkaido, 060-8543, Japan.
authors.forename: Shunya
authors.initials: S
authors.lastname: Ohtaki
authors.affiliation: Department of Neurosurgery, Sapporo Medical University, School of Medicine, South 1, West 16, Chuo-ku, Sapporo, Hokkaido, 060-8543, Japan.
authors.forename: Toshiya
authors.initials: T
authors.lastname: Sugino
authors.affiliation: Department of Neurosurgery, Sapporo Medical University, School of Medicine, South 1, West 16, Chuo-ku, Sapporo, Hokkaido, 060-8543, Japan.
authors.forename: Satoshi
authors.initials: S
authors.lastname: Iihoshi
authors.affiliation: Department of Neurosurgery, Sapporo Medical University, School of Medicine, South 1, West 16, Chuo-ku, Sapporo, Hokkaido, 060-8543, Japan.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Mikami
authors.affiliation: Department of Surgical Pathology, Sapporo Medical University, School of Medicine, Sapporo, Japan.
authors.forename: Shintaro
authors.initials: S
authors.lastname: Sugita
authors.affiliation: Department of Surgical Pathology, Sapporo Medical University, School of Medicine, Sapporo, Japan.
authors.forename: Tadashi
authors.initials: T
authors.lastname: Hasegawa
authors.affiliation: Department of Neurosurgery, Sapporo Medical University, School of Medicine, South 1, West 16, Chuo-ku, Sapporo, Hokkaido, 060-8543, Japan.
authors.forename: Nobuhiro
authors.initials: N
authors.lastname: Mikuni
mesh_terms: D000305:Adrenal Cortex Hormones / Q000008:administration & dosage; D000328:Adult; D015415:Biomarkers / Q000097:blood; D003937:Diagnosis, Differential; D038524:Diffusion Magnetic Resonance Imaging; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D008297:Male; D008577:Meningeal Neoplasms; D008579:Meningioma; D009901:Optic Nerve Diseases / Q000175:diagnosis* / Q000188:drug therapy / Q000209:etiology* / Q000473:pathology; D019574:Optic Nerve Neoplasms; D016896:Treatment Outcome; D014786:Vision Disorders / Q000209:etiology
chemical_list: D000305:Adrenal Cortex Hormones; D015415:Biomarkers; D007074:Immunoglobulin G
keywords: IgG4-related disease; Optic nerve sheath meningioma; Orbital tumor