Locally Destructive Skull Base Lesion: IgG4-related Sclerosing Disease

Abstract

A unique case of IgG4+ sclerosing disease was diagnosed in the sphenoid sinus, a previously unreported location, and was treated in a novel manner. This study describes the clinical presentation and management of IgG4 sclerosing disease in the paranasal sinuses. A retrospective case review and review of the medical literature were performed. A 38-year-old woman with a 2-year history of constant frontal headaches presented to our clinic. Imaging showed bony destruction of the sphenoid sinus and sellar floor. The patient underwent a right-sided sphenoidotomy with debridement and biopsy. Pathological evaluation showed a dense plasmacytic infiltrate with >150 IgG4+ cells/high-power field. She was subsequently started on a nasal corticosteroid with improved patency of the sphenoid antrostomy. We report an unusual case of a middle-aged woman who presented with IgG4-sclerosing disease (IGSD) isolated to the sphenoid sinus. Although our knowledge concerning treatment in extrapancreatic organs is lacking, there is evidence that glucocorticoid treatment improves nasal sinus opacification on CT findings (Sato Y, Ohshima K, Ichimura K, et al., Ocular adnexal IgG4-related disease has uniform clinicopathology, Pathol Int 58:465–470, 2008). This case study and literature review adds to the growing literature describing IGSD in the head and neck and more specifically isolated to the sphenoid sinus with preliminary data concerning local control with topical steroids.

Comorbid diseases of IgG4‐related sialadenitis in the head and neck region

“…Our results indicate that immunomodulatory therapy is effective for IgG4‐RS and its comorbid diseases in the head and neck, which is consistent with previous reports . Recently, Alt et al reported a case of localized IgG4‐RD in the sphenoid sinus, with normal serum IgG4 level successfully treated by sphenoidotomy combined with nasal corticosteroid spray . For the patients with IgG4‐related disease isolated to the nasal cavity and/or paranasal sinus, topical application of steroids with or without limited surgery would be a good choice of treatment because it lessens the risks of steroid complications.…”

“…13,26,31,40 Recently, Alt et al reported a case of localized IgG4-RD in the sphenoid sinus, with normal serum IgG4 level successfully treated by sphenoidotomy combined with nasal corticosteroid spray. 41 For the patients with IgG4-related disease isolated to the nasal cavity and/or paranasal sinus, topical application of steroids with or without limited surgery would be a good choice of treatment because it lessens the risks of steroid complications. A mean period of 10.4 6 5.9 months for follow-up is relatively short for precise evaluation of our treatment protocol.…”

IgG4-Related Disease

“…Lesions usually are not destructive, and bone adjacent to the lesions demonstrates remodeling without destruction (26). However, there are case reports of destructive bone involvement in biopsy-proven IgG4-RD, especially in cases affecting the paranasal sinuses (7,(27)(28)(29). IgG4-RD involving the nasal cavity and paranasal sinuses also may show perineural and bone marrow infiltration mimicking malignant lymphoma (30).…”

Unusual cause of maxillary sinus mass with proptosis

“…However, involvement of the paranasal sinus is quite rare, with only six adult cases reported previously in the English literature. Of the paranasal sinuses, the order of involvement is maxillary sinus6–10 followed by sphenoid sinus 8 11. Nasal septal involvement7 has also been described.…”