Sight-threatening optic neuropathy is associated with paranasal lymphoma

Abstract

Malignant lymphoma around the orbit is very rare. We present a rare case of optic neuropathy caused by lymphoma. A 61-year-old Japanese woman was referred to our hospital for evaluation of idiopathic optic neuropathy affecting her right eye. The patient was treated with steroid pulse therapy (methyl-predonisolone 1 g daily for 3 days) with a presumed diagnosis of idiopathic optic neuritis. After she had been switched to oral steroid therapy, endoscopic sinus surgery had been performed, which revealed diffuse large B cell lymphoma of the ethmoidal sinus. Although R-CHOP therapy was immediately started, prolonged optic nerve compression resulted in irreversible blindness. Accordingly, patients with suspected idiopathic optic neuritis should be carefully assessed when they show a poor response, and imaging of the orbits and brain should always be done for initial diagnosis because they may have compression by a tumor.

Keywords: malignant lymphoma; optic neuropathy; paranasal lymphoma; rhinogenic optic neuropathy.

Figure 1

Tumor mass occupying the frontal sinuses and part of the right anterior ethmoidal sinus. Before steroid pulse therapy, the mass occupies much of the ethmoidal sinus on computed tomography (CT) (A) and magnetic resonance imaging (MRI) (B) T2-weighed MRI showed isointense mass. (C) Despite steroid pulse therapy and endoscopic sinus surgery, the tumor expanded to compress the optic nerve (yellow arrow) and oculomotor nerve. Contrast-enhanced MRI showed low intensity mass in T2 image. (Broken circle indicates the tumor, broken white line indicates the oculomotor nerve and broken yellow arrow indicates the compression point).

Figure 2

Histological features of the resected specimen. (A) Resected specimen revealed diffuse proliferation of atypical lymphoid cells in the ethmoidal sinus. Insert: There are large-sized cells with prominent nucleoli (arrowheads). These cells express CD20 (B) and CD79a (C), but do not express CD3 (D). These immunohistochemical reactions are consistent with that of diffuse large B cell lymphoma.