Optic Nerve Infiltration in Primary Central Nervous System Lymphoma

Abstract

IMPORTANCEVisual impairment in primary central nervous system lymphoma (PCNSL) is caused mostly by intraocular lymphomatous involvement (vitritis and retinal infiltration), whereas optic nerve infiltration (ONI) is a rare condition.OBJECTIVE To describe the clinical presentation of ONI, its imaging characteristics, and outcome. DESIGN, SETTING AND PARTICIPANTSA total of 752 patients diagnosed with PCNSL were retrospectively identified from the databases of 3 French hospitals from January 1, 1998, through December 31, 2014. Of these, 7 patients had documented ONI. Exclusion criteria were intraocular involvement, orbital lymphoma, or other systemic lymphoma. Clinical presentation, neuroimaging, biological features, treatment, and outcomes were assessed. MAIN OUTCOMES AND MEASURESTreatment response was evaluated clinically and radiologically on follow-up magnetic resonance imaging (MRI) according to the International PCNSL Collaborative Group response criteria. RESULTSThe 7 patients included 5 women and 2 men. Median age at diagnosis was 65 years (range, 49-78 years). Two patients had initial ONI at diagnosis, and 5 had ONI at relapse. Clinical presentation was marked by rapidly progressive and severe visual impairment for all patients. The MRI findings showed optic nerve enlargement in 3 patients and contrast enhancement of the optic nerve in all patients. Additional CNS lesions were seen in 4 patients. Examination of cerebrospinal fluid samples detected lymphomatous meningitis in 2 patients. Clinical outcome was poor and marked by partial recovery for 2 patients and persistent severe low visual acuity or blindness for 5 patients. Median progression-free survival after optic nerve infiltration was 11 months (95% CI, 9-13 months), and median overall survival was 18 months (95% CI, 9-27 months).CONCLUSIONS AND RELEVANCE Optic nerve infiltration is an atypical and challenging presentation of PCNSL. Its visual and systemic prognosis is particularly poor compared with vitreoretinal lymphomas even in response to chemotherapy. Although intraocular involvement is frequent in PCNSL and clinically marked by slowly progressive visual deterioration, lymphomatous ONI is rare and characterized by rapidly progressive severe visual impairment.

Intraocular involvement is associated with a high risk of disease relapse in primary central nervous system lymphoma

“…Recent studies have concentrated on isolated PIOL (8,9), concluding that it is a less aggressive form of the disease (10,11). However, to the best of our knowledge, only a few studies to date have focused on IOL with CNS involvement or following intracerebral mass due to the difficulty of diagnosis and rarity of the disease (12,13). As a result, questions concerning the biological behavior of IOL remain unresolved.…”

Orbital Magnetic Resonance Imaging May Contribute to the Diagnosis of Optic Nerve Lymphoma

“…Contrast enhancement was seen in MRI in most optic nerve lymphoma patients. According to Ahle et al (24), MRI showed contrast enhancement of the optic nerve in all seven optic nerve infiltrated patients described. In our review, enhanced MRI was observed in 19 of the 22 cases.…”

“…Ahle et al described optic nerve infiltration in 7 patients from a 752-PCNSL cohort; MRI showed optic nerve thickening in only three of the seven cases. However, the authors considered that standard MRI findings without sequences focused on the optic nerves might be interpreted as usual, and that orbital MRI focusing on both optic nerves and including high-resolution fat-suppressed sequences might increase the chance of detecting optic nerve enlargement (24). Optic nerve lymphoma usually manifests as massive nerve swelling (2, 18) and could last for a long time without treatment.…”

“…Although there is no pathological examination of the optic nerve in these cases, it is reasonable to assume that they are single lymphomas involving optic nerves rather than multiple diseases affecting the optic nerve. Other than optic nerve biopsy, Ahle et al diagnosed optic nerve lymphoma in their patients by MRI and CSF analysis (24).…”

Stage IV Small Lymphocytic Lymphoma Presenting with Unilateral Vision Loss

“…The differential diagnosis for this patient, who presented with sudden onset right eye vision loss, is broad. Important diagnoses to consider include optic neuropathy, optic neuritis, autoimmune disorders, inflammatory pseudotumors, and other compressive neuropathies, including optic nerve glioma, optic nerve meningioma, optic nerve or orbital metastases, inherited disorder, and toxic, nutritional, or metabolic optic neuropathies [7]. Urgent ophthalmology consult may be necessary in order to influence the differential, ordering of subsequent testing, and prevention of further progression of the disease.…”