PMC5391482_01
Endoscopic view of the fibrous sinonasal mass emerging from the left maxillary and ethmoid sinuses and filling a significant volume of the middle meatus and ethmoid cavity.
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F1
Nasal endoscopy revealed a fleshy mass within the left middle meatus and bowing of the left lateral nasal wall of the inferior meatus (Fig. 1).
Radiographic images of left sinonasal mass. (A and B) CT without contrast, coronal and axial views demonstrating a mass filling the left maxillary and ethmoid sinuses with erosion of the left medial orbital floor and lamina papyracea. (C and D) MRI T1 with contrast, coronal and axial views demonstrating same lesion extending into the orbit with infiltration of the inferior and medial rectus muscles, but sparing the globe. Complete opacification of the left maxillary sinus also shown. (E and F) MRI T2, coronal and axial views further illustrating the aforementioned findings in panels C and D.
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F2
CT highlighted erosion of the lamina papyracea and orbital floor, and MRI exhibited soft-tissue extension into the orbit without globe involvement (Fig. 2).
Histopathological specimens from surgical debridement of left nasal cavity pseudotumor. (A) IgG4 stain, x 400 high power view. Diffuse infiltration of IgG4 plasma cells, >50 cells/HPF. (B) H&E stain, x 200 medium power view. Respiratory mucosa demonstrating the characteristic irregular whorled pattern of fibrosis (storiform fibrosis) of IgG4-related disease.
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F3
IgG4 disease (Fig. 3). Immunologic testing revealed elevated IgG4 of 94.5 mg/dL (reference range, 4-86 mg/dL) in the setting of normal total IgG levels of 1088 mg/dL (reference range, 694-1618 mg/dL).
Post-surgical radiographic images of left sinonasal mass. (A) CT without contrast, 3 weeks after surgical debulking and initiation of high dose oral prednisone. (B) MRI T2, 6 weeks after surgical debulking.
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F4
There also was radiographic resolution of disease (Fig. 4).
Endoscopic view of the left maxillary sinus status 6 months after surgical debulking and initiation of steroid therapy. There is a patent antrostomy, and the site of previous tumor shows no residual or recurrent disease.
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F5
Subsequent clinical evaluation at 6 months showed continued symptom resolution, with no evidence of disease recurrence (Fig. 5).