Brain Tumor Res Treat.  2021 Oct;9(2):93-99. 10.14791/btrt.2021.9.e17.

Intracranial Tumors Associated With IgG4-Related Disease

• Kim D
• Kim SH
• Kim EH

• KMID: 2522226
• DOI: http://doi.org/10.14791/btrt.2021.9.e17

Abstract

IgG4-related disease (IgG4-RD) is an immune-mediated inflammatory condition which is characterized by dense lymphoplasmacytic infiltrations with a predominance of IgG4 plasma cells in the affected tissue. Although pachymeninx and pituitary gland are the most common sites where IgG4-RD infiltrates, the associations with IgG4-RD and a true intracranial tumor have not been yet reported in literature. Herein, we report two cases with intracranial tumors associated with IgG4-RD; a 36-year-old male patient with a huge meningioma and another 54-year old woman with a pituitary macroadenoma. Pathological examination revealed their tumors were substantially infiltrated by IgG4 plasma cells indicating its possible relation with IgG4-RD.

Keyword

IgG4-related disease; Meningioma; Pituitary adenoma

Figure

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  Fig. 1 Preoperative radiological findings of a 36-year-old male patient (Case 1). A: MRI shows a large extra-axial tumor on the right posterior fossa. B: CT reveals extensive destruction of the occipital bone. C and D: Cerebral angiography demonstrates a strong vascular supply from the right occipital artery, right posterior inferior cerebellar artery, and right posterior meningeal artery.

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  Fig. 2 Pathological findings of a 36-year-old male patient (Case 1). A: The first case shows a well demarcated extra-axial dura-based mass (H&E, ×12). B: High power view shows neoplastic meningothelial proliferation with many areas of plasmacytic infiltration (H&E, ×200). C: In addition, the meningothelial cells show EMA immunoreactivity (EMA, ×100). D and E: On immunohistochemistry, a high IgG4 (IgG4, ×100)/IgG (IgG, ×100) ratio is noted.

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  Fig. 3 Radiological findings of a 54-year-old female patient (Case 2). A: MRI shows invasive adenoma invading right cavernous sinus. B: Postoperative MRI shows subtotal removal status of pituitary adenoma. C: MRI shows newly developed bulging contour in the left cavernous sinus with adjacent dural thickening. D: After repetitive administration of steroid, follow up MRI shows decreased dural thickening lesion in the left cavernous sinus.

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  Fig. 4 Pathological findings of a 54-year-old female patient (Case 2). A: Low power (H&E, ×12) shows a portion of pituitary adenoma (left lower) and dura with inflammation (right upper). B: High power view (H&E, ×100) shows a classic histological findings of pituitary adenoma C: Dura with dense inflammation (H&E, ×40) is noted. D: High power view (H&E, ×200) shows dense inflammatory cell infiltrates with many plasma cells. E and F: The IgG4 positive plasma cells/IgG positive cells ratio was 24.8% based on IgG (E, ×200) and IgG4 (F, ×200) staining.

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