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Case Reports
. 2012 Jul 15;318(1-2):31-5.
doi: 10.1016/j.jns.2012.04.010. Epub 2012 Apr 28.

IgG4-related inflammatory pseudotumor of the central nervous system responsive to mycophenolate mofetil

Heather E Moss  1 , Luis J Mejico, Gustavo de la Roza, Thomas M Coyne, Steven L Galetta, Grant T Liu
Affiliations
Case Reports

IgG4-related inflammatory pseudotumor of the central nervous system responsive to mycophenolate mofetil

Heather E Moss et al. J Neurol Sci. .

Abstract

Orbital apex and skull base masses often present with neuro-ophthalmic signs and symptoms. Though the localization of these syndromes and visualization of the responsible lesion on imaging is typically straightforward, definitive diagnosis usually relies on biopsy. Immunohistochemistry is important for categorization and treatment planning. IgG4-related disease is emerging as a pathologically defined inflammatory process that can occur in multiple organ systems. We present two patients with extensive inflammatory mass lesions of the central nervous system with immunohistochemistry positive for IgG4 and negative for ALK-1 as examples of meningeal based IgG4-related inflammatory pseudotumors. In both patients, there was treatment response to mycophenolate mofetil.

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Conflict of interest statement

Conflicts of Interest

The authors disclose no financial or personal relationships with other people or organizations that could inappropriately influence this work

Figures

Figure 1
Figure 1
Post contrast T1 MRI of brain axial (left column) and coronal (center and right column) images from patient 1. These demonstrate multifocal extra-axial lesions including middle cranial fossa (closed arrow), foramen magnum (open arrow) and superior frontal (circle). Top row shows initial imaging, middle row shows imaging obtained at representation 2 years later, and bottom row shows imaging 6 months after repeat resection of cavernous sinus lesion and medical therapy.
Figure 2
Figure 2
Histologic features of middle cranial fossa biopsy from patient one. Hematoxylin and eosin stain at low (10X) (left) and high (middle) (40X) power depict abundant macrophages, lymphocytes and plasma cells. Immunohistochemical stain for IgG4 (right) demonstrates multiple immunoreactive plasma cells. (scale bars: left 200 µm, middle, right 50 µm).
Figure 3
Figure 3
Post contrast T1 MRI of brain axial (left column), coronal (center column) and saggital (right column) images from patient 2. These demonstrate an extra-axial lesion involving left middle and anterior cranial fossa, petrous apex, cavernous sinus, cerebellopontine angle and tentorium. Top row shows initial imaging and bottom row shows 18 month follow up.
Figure 4
Figure 4
Histologic features of middle cranial fossa biopsy from patient two. Hematoxylin and eosin stain at low (10× objective lens) (left) and high (40× objective lens) (middle) power. These demonstrate a mixed inflammatory infiltrate and fibrosis. IgG4 immunohistochemical stain (right) demonstrates multiple immunoreactive plasma cells.
See this image and copyright information in PMC

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