Interesting case of base of skull mass infiltrating cavernous sinuses

A man aged 35 years presented with chronic headache and earache of 1-year duration. He had progressive vision loss and diplopia since last 9 months. He also had pain over the face and episodic profuse epistaxis. On examination, perception of light was absent in the right eye and hand movements were detected at 4 m distance in the left eye. Imaging revealed a lobulated mass in the nasopharynx extending into the bilateral cavernous sinuses and sphenoid sinus with bony erosions. Biopsy of the nasopharyngeal mass revealed pathological features which are characteristic of IgG4 disease. His serum IgG4 levels and acute inflammatory markers were also elevated. The patient was started on oral corticosteroid therapy. Fever, headache and earache resolved early and there was gradual improvement in the vision of the left eye. After 6 months, visual acuity in the left eye was 6/9, but right eye visual acuity had no change. Follow-up imaging revealed a significant reduction in the size of the mass.

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