Skip to main content

Advertisement

Log in

IgG4-related disease initially presented as an orbital mass lesion mimicking optic nerve sheath meningioma

  • Case Report
  • Published:
Brain Tumor Pathology Aims and scope Submit manuscript

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.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Fig. 1
Fig. 2
Fig. 3
Fig. 4

Similar content being viewed by others

References

  1. 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

    Article  CAS  PubMed  Google Scholar 

  2. 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

    Article  CAS  PubMed  Google Scholar 

  3. 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

    Article  CAS  PubMed  Google Scholar 

  4. 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

    Article  PubMed Central  PubMed  Google Scholar 

  5. 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

    Article  CAS  PubMed  Google Scholar 

  6. 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

    Article  CAS  PubMed  Google Scholar 

  7. 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

    Article  CAS  PubMed  Google Scholar 

  8. Turbin RE, Pokorny K (2004) Diagnosis and treatment of orbital optic nerve sheath meningioma. Cancer Control 11(5):334–341

    PubMed  Google Scholar 

  9. Espinoza GM (2010) Orbital inflammatory pseudotumors: etiology, differential diagnosis, and management. Curr Rheumatol Rep 12(6):443–447

    Article  PubMed  Google Scholar 

  10. Mendenhall WM, Lessner AM (2010) Orbital pseudotumor. Am J Clin Oncol 33(3):304–306

    PubMed  Google Scholar 

  11. 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

    Article  PubMed  Google Scholar 

  12. 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

    Article  PubMed  Google Scholar 

  13. 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

    Article  PubMed  Google Scholar 

  14. 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

    Article  CAS  PubMed  Google Scholar 

Download references

Conflict of interest

The authors have no conflicts of interest.

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Masahiko Wanibuchi.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

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

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10014-015-0223-7

Keywords

Navigation