Presumptive primary intraocular lymphoma presented as an intraocular mass involving the optic nerve head
© The Author(s) 2011
Received: 30 August 2011
Accepted: 18 October 2011
Published: 10 November 2011
Primary intraocular lymphoma (PIOL) is a subset of primary central nervous system (CNS) lymphoma which initially presents in the eye with or without simultaneous CNS involvement (1). It is considered a variant of extra-nodal, non-Hodgkin lymphoma that is usually of an aggressive diffuse large B cell type (1–3). Diagnosis of PIOL requires histopathologic evidence of malignant lymphoma cells in brain biopsy, vitreous or CSF specimen (1, 2). Cytological examination is the mainstay method to detect lymphomatous cells (3, 4). Other ancillary tests like flow cytometry, immunohistochemistry staining, cytokine analysis, and gene rearrangement studies also aid in the diagnosis and further classification of PIOL (1, 4).
Optic nerve and optic disc involvement may occur in the PIOL (4, 5). Here, we report a case with an unusual presentation of this malignancy presented as an intraocular mass with involvement of the optic nerve head.
Primary intraocular lymphoma is considered one of the most elusive ocular masquerade syndromes. The diagnosis is frequently delayed due to misdiagnosis and false-negative biopsy reports which may be a result of small sample size (inadequate sample, too few vitreous cells, and sampling from out of the lesion), improper handling of specimen (unsuitable transferring media, delay in transferring or processing the specimen, and high temperature), or prior steroid therapy (1, 3). To lessen the false-negative results, our patient was instructed to stop oral steroids for 2 weeks before vitreous tap. Pathologist was first alerted, and then specimen was obtained fresh and transported immediately to the cytologist for prompt processing. In spite all measures, the initial vitreous specimen was negative for malignant cells. In our case, the vitritis was limited to the posterior vitreous (just around the mass) which may result in low diagnostic yield by vitreous tap. There would probably have been a higher chance to obtain the malignant cells had a diagnostic vitrectomy been performed. The dramatic clinical response to systemic chemotherapy which was manifested by the reduction of the vitreous cells and the size of intraocular mass convinced us that nature of both intraocular and intracranial lesions must be the same.
In our patient, the optic disc was infiltrated by malignant cells. This was revealed by close vicinity between the intraocular mass and optic disc in B-scan and magnetic resonance images. The profound loss of vision and significant relative afferent pupillary defect early in the course of the disease further support this assumption. However, due to lack of confirmatory vitreous biopsy, we considered the nature of the mass presumptive.
Although optic nerve and optic disc infiltration have been reported in primary intraocular lymphoma (4, 5), this case represents an unusual presentation of presumptive intraocular lymphoma featuring as an intraocular mass with involvement of optic nerve head that to our knowledge has not been reported yet.
We are grateful for the assistance of Dr. Norman Chan for supporting us with histopathologic photo and description.
Declaration of interest
The authors report no conflicts of interest. The authors alone are responsible for the content and writing of the paper.
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