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31 July 2013 - Case of the Week #281

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Case of the Week #281

Clinical History:

A 48 year old man presented with a left eyelid mass. PET-CT scan showed a markedly increased uptake in the ocular region. An excisional biopsy was performed:

PET scan

Micro images:


What is your diagnosis?


Ocular adnexal mantle cell lymphoma


Left to right: CD20+, CD3- (T cells are CD3+), CD5+

Left to right: CD23-, cyclinD1+, Ki-67+

Left to right: bcl2+, CD43+

The H&E stained sections show sheets of an atypical lymphoid infiltrate with occasional hyalinized small vessels in the background. The tumor cells are monomorphic, small mature lymphocytes with scant cytoplasm, round to oval, cleaved nuclei, dense chromatin and inconspicuous nucleoli. No mitotic figures were seen. Flow cytometry (not shown) revealed monoclonal B cells with lambda light chain restriction, which coexpress CD19 and CD5. These cells were also positive for CD20 (bright) and FMC7, and are negative for CD23, CD10 and CD11C. Immunostains demonstrated that the neoplastic cells were B cells (CD20+, PAX5+) with coexpression of bcl2, CD5, CD43 and cyclin D1, and they were negative for AE1/AE3, CD3, CD10, CD23 and CD30. The cell proliferation index marker Ki-67 was 20-30%, confirming a relatively low grade process. Conventional cytogenetics analysis showed 46, XY, t(11;14)(q13;q32).

Ocular lymphomas can be primary or secondary, and occur in the orbit, conjunctiva and eyelids. Most lymphomas in the ocular adnexa are B cell non-Hodgkin lymphomas, most commonly extranodal marginal zone B cell lymphoma of mucosa associated lymphoid tissue (MALToma). Less common B cell subtypes include lymphoplasmacytic lymphoma, mantle cell lymphoma, plasmacytoma and follicular lymphoma. At all sites, mantle cell lymphoma (MCL) represents 3-5% of non-Hodgkin lymphomas. It predominantly occurs in older ages with a higher frequency in men, usually presenting with widespread disease and an aggressive course. Mantle cell lymphoma most commonly occurs in lymph nodes and in the GI tract, but it presents as secondary involvement of ocular adnexal structures in 6% of the ocular lymphomas. There are limited studies of primary ocular mantle cell lymphoma.

1. Oh DE, Kim YD. Lymphoproliferative lesions of the ocular adnexa, Arch Ophthalmol 2007;125:1668

2. Rasmussen P, Sjo LD, Prause JU, Ralfkiaer E, Heegaard S. Mantle cell lymphoma in the orbital and adnexal region, Br J Ophthalmol 2009;93:1047

3. Sander B. Mantle cell lymphoma: recent insights into pathogenesis, clinical variability, and new diagnostic markers, Semin Diagn Pathol 2011;28:245

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