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Published on: March 30, 2018
Conjunctival mass as an initial presentation of mantle cell lymphoma: a case report
Mahsa Khanlari1, Babak Bagheri, Reza Vojdani
1Institute of Hematopathology, Dr Daneshbod Pathology Laboratory, Shiraz, Iran.
Insights
This study details a rare case of mantle cell lymphoma (MCL) affecting the conjunctiva. Accurate diagnosis requires specific immunohistochemistry and genetic testing to distinguish it from other lymphomas.
Area of Science:
- Ophthalmology
- Hematology
- Oncology
Background:
- Mantle cell lymphoma (MCL) is a rare B-cell neoplasm.
- Ocular adnexal lymphomas are typically extranodal marginal zone B-cell lymphomas (MALT).
- Ocular MCL is an uncommon presentation requiring specific diagnostic considerations.
Observation:
- A 64-year-old male presented with a three-month history of a right conjunctival mass.
- Histopathology revealed a subconjunctival proliferation of small-to-medium-sized lymphoid cells with cleaved nuclei.
- Immunohistochemistry confirmed the lymphoid infiltrate as positive for CD20, CD5, BCL-2, cyclin D1, and SOX11.
Findings:
- Fluorescent in situ hybridization identified the IGH-CCND1 fusion, indicative of the t(11;14) translocation.
- The findings confirm a diagnosis of mantle cell lymphoma in the conjunctiva.
- This presentation highlights a rare ocular manifestation of MCL.
Implications:
- Accurate diagnosis of ocular adnexal lymphomas is crucial.
- Immunohistochemical and genetic analysis are essential for identifying ocular MCL.
- Distinguishing ocular MCL from more common lymphomas ensures appropriate patient management and staging.
Background:
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). 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).
Conclusion:
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.
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