Abstract
BackgroundTo describe a rare manifestation of mantle cell lymphoma (MCL) in conjunctiva, with clinical, hisologic, immunohistologic and genetic findings together with review of the Literature.Case presentationMost ocular adnexal lymphomas are extranodal marginal zone B-cell lymphomas of mucosa-associated lymphoid tissue (MALT). A few cases of ocular adnexal mantle cell lymphomas have been reported in the literature. We present a case of mantle cell lymphoma presenting as right conjunctival mass of at least three months duration in a 64-year-old man. Histopathologic examination showed a proliferation of monomorphous small-to-medium-sized lymphoid cells with cleaved nuclei in the subconjunctiva. By immunohistochemistry, the infiltrate was positive for CD20, CD5, BCL-2, cyclin D1, and the transcription factor SOX11. Fluorescent in situ hybridization demonstrated the presence of IGH-CCND1 fusion indicating t(11;14).ConclusionA rigorous approach to initial diagnosis and staging of small cell lymphomas of the ocular adnexa is needed. The recognition of ocular MCL requires appropriate immunohistochemical staining and/or genetic confirmation to differentiate this rare form of presentation of MCL from other more frequent small cell lymphomas.
Highlights
To describe a rare manifestation of mantle cell lymphoma (MCL) in conjunctiva, with clinical, hisologic, immunohistologic and genetic findings together with review of the Literature.Case presentation: Most ocular adnexal lymphomas are extranodal marginal zone B-cell lymphomas of mucosa-associated lymphoid tissue (MALT)
The recognition of ocular MCL requires appropriate immunohistochemical staining and/or genetic confirmation to differentiate this rare form of presentation of MCL from other more frequent small cell lymphomas
By fluorescence in situ hybridization (FISH) analysis on formalin-fixed, paraffin-embedded tissue sections using commercially available LSI IGH/CCND1 XT dual color, dual fusion and LSI CCND1 BAP break apart probes (AbbottVysis), juxtaposition of IGH and CCND1 and a breakpoint in the CCND1 locus were detected in 85% and 97% of nuclei, respectively, indicating presence of the MCL hallmark translocation t(11;14)(q13;q32) (Figure 5)
Summary
A rigorous approach to initial diagnosis and staging of small cell lymphomas of the ocular adnexa is needed. The recognition of ocular MCL requires appropriate immunohistochemical staining and/or genetic confirmation to differentiate this rare form of presentation of MCL from other more frequent small cell lymphomas. According to TNM staging of ocular lymphomas, orbital imaging as well as systemic workup is suggested for these rare ocular presentations. Competing interest No conflict of interest is declared, and source of funding is Dr Daneshbod Laboratory. Authors’ contributions "BB" made clinical diagnosis, "MK"and "YD" carried out the diagnostic histology and immunohistologic studies and drafted the article, "RV", "MM" and "SP" participated in the treatment and revising the manuscript. All authors read and approved the final manuscript
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