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.
Similar content being viewed by others
References
Hamano H, Kawa S, Horiuchi A et al (2001) High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med 344(10):732–738
Umehara H, Okazaki K, Masaki Y et al (2012) Comprehensive diagnostic criteria for IgG4-related disease (IgG4-RD), 2011. Mod Rheumatol 22(1):21–30
Masaki Y, Dong L, Kurose N et al (2009) Proposal for a new clinical entity, IgG4-positive multiorgan lymphoproliferative syndrome: analysis of 64 cases of IgG4-related disorders. Ann Rheum Dis 68(8):1310–1315
Nagai K, Andoh K, Nakamura N, Sakata K (2011) Suspected idiopathic sclerosing orbital inflammation presenting as immunoglobulin G4-related disease: a case report. J Med Case Rep 5:427
Plaza JA, Garrity JA, Dogan A, Ananthamurthy A, Witzig TE, Salomao DR (2011) Orbital inflammation with IgG4-positive plasma cells: manifestation of IgG4 systemic disease. Arch Ophthalmol 129(4):421–428
Yamamoto M, Tabeya T, Naishiro Y et al (2012) Value of serum IgG4 in the diagnosis of IgG4-related disease and in differentiation from rheumatic diseases and other diseases. Mod Rheumatol 22(3):419–425
Yamamoto M, Takahashi H, Naishiro Y et al (2008) Mikulicz’s disease and systemic IgG4-related plasmacytic syndrome (SIPS). Nihon Rinsho Men’eki Gakkai kaishi 31(1):1–8
Turbin RE, Pokorny K (2004) Diagnosis and treatment of orbital optic nerve sheath meningioma. Cancer Control 11(5):334–341
Espinoza GM (2010) Orbital inflammatory pseudotumors: etiology, differential diagnosis, and management. Curr Rheumatol Rep 12(6):443–447
Mendenhall WM, Lessner AM (2010) Orbital pseudotumor. Am J Clin Oncol 33(3):304–306
Ohtsuka K, Hashimoto M, Suzuki Y (2005) A review of 244 orbital tumors in Japanese patients during a 21-year period: origins and locations. Jpn J Ophthalmol 49(1):49–55
Shields JA, Shields CL, Scartozzi R (2004) Survey of 1264 patients with orbital tumors and simulating lesions: the 2002 Montgomery Lecture, part 1. Ophthalmology 111(5):997–1008
Oyama T, Takizawa J, Nakamura N, Aoki S, Aizawa Y, Abe H (2011) Multifocal mucosa-associated lymphoid tissue lymphoma associated with IgG4-related disease: a case report. Jpn J Ophthalmol 55(3):304–306
Ebbo M, Daniel L, Pavic M et al (2012) IgG4-related systemic disease: features and treatment response in a French cohort: results of a multicenter registry. Medicine 91(1):49–56
Conflict of interest
The authors have no conflicts of interest.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Noshiro, S., Wanibuchi, M., Akiyama, Y. et al. IgG4-related disease initially presented as an orbital mass lesion mimicking optic nerve sheath meningioma. Brain Tumor Pathol 32, 286–290 (2015). https://doi.org/10.1007/s10014-015-0223-7
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s10014-015-0223-7