Optic nerve lymphoma: report of two cases and review of the literature

Abstract

Lymphoma may involve the optic nerve as isolated optic nerve lymphoma or in association with central nervous system (CNS) or systemic lymphoma. We present two biopsy-proven non-Hodgkin lymphomas of the optic nerve and compare our findings with previously reported cases. We discuss the mechanism of metastasis, classification of optic nerve involvement, clinical features, radiologic findings, optic nerve biopsy indications and techniques, histologic features, and treatments. We propose a classification system of optic nerve lymphoma: isolated optic nerve involvement, optic nerve involvement with CNS disease, optic nerve involvement with systemic disease, and optic nerve involvement with primary intraocular lymphoma. Although it is an uncommon cause of infiltrative optic neuropathy, optic nerve metastasis should be considered in patients with a history of lymphoma. The recommended approach to a patient with presumed optic nerve lymphoma includes neuroimaging and cerebrospinal fluid evaluation as part of the initial workup, then judicious use of optic nerve biopsy, depending on the clinical situation.

Keywords: lymphoma; metastasis; optic nerve; optic nerve lymphoma.

Figure 1

Case 1. A. Fundus appearance showing optic nerve edema, vessel engorgement and intraretinal hemorrhages. B. MRI showing right optic nerve and optic nerve sheath swelling (arrow) compared with the left. C. Optic nerve biopsy showing distension of fibrovascular septa with lymphocytes and atrophy of nerve fiber bundles. D. Monotonous sheets of lymphocytes mainly in the fibrovascular pial septa with occasional cells present within the atrophic and necrotic nerve fibers. E. Higher magnification showing lymphocytes with round to oval, condensed nuclei and scant cytoplasm. F. Cells in the fibrovascular septa and nerve tissue stain for CD20. (C. hematoxylin and eosin 10×; D. hematoxylin and eosin 25×; E. hematoxylin and eosin 250×; F. peroxidase anti-peroxidase 100×)

Figure 2

Case 2. A. Fundus appearance of left eye showing disc edema and mild pigmentary changes of the retina. B. MRI with gadolinium showing enhancement of the left optic nerve and optic nerve sheath (arrow) compared with the right. C. Optic nerve biopsy showing infiltration of the fibrovascular pial septa with lymphocytes and sparing of the nerve fiber bundles. D. Higher magnification of lymphocytes within the pial septa. E. Lymphocytes with hyperchromatic nuclei, occasional histiocytes with larger nuclei, and associated crush artifact. F. Lymphocytes within the fibrovascular pial septa staining positive for CD20. (C. hematoxylin and eosin, 25×; D. hematoxylin and eosin 100×; E. hematoxylin and eosin 250×; F. peroxidase anti-peroxidase, 25×)