Which histologic findings are characteristic of ocular lymphoma?

Updated: Mar 15, 2019
  • Author: Manolette R Roque, MD, MBA, FPAO; Chief Editor: Hampton Roy, Sr, MD  more...
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Answer

A key factor in obtaining an accurate diagnosis of intraocular lymphoma is a cytopathologist with experience in intraocular specimens.

Mucosa-associated lymphoid tissue (MALT) lymphoma, diffuse large cell lymphoma, and small lymphocytic lymphoma are some common types of intraocular lymphoma. Because of the fragility of neoplastic lymphocytes, a specimen may contain numerous abnormal-appearing but uninterruptable cells.

Tumor cells can involve the vitreous, retina, optic nerve, or choroid. They are found less often in the anterior segment. Choroidal involvement by primary central nervous system lymphoma with ocular involvement (PCNSLO) is typically diffuse, whereas retinal involvement may be more perivascular. When present, retinal necrosis can be extensive.

Molecular analysis detecting immunoglobulin gene rearrangements and ocular cytokine levels showing elevated interleukin (IL)–10 (IL-10), with an IL-10–to–IL-6 ratio greater than 1.0, are helpful adjuncts to cytology for establishing the diagnosis of PCNSLO. [40] CDR3 polymorphism analysis is recommended to confirm clonality.

Extranodal marginal zone B-cell lymphoma represents the most common orbital lymphoma subtype. [41, 42] Most patients with ocular adnexal lymphoma have stage IE disease. Immunohistochemical staining with CD markers helps classify lymphomas.

Gross specimens appear salmon-colored with a fish-flesh consistency. Hypercellular proliferations are seen with sparse stroma. Immunologic identification of cell surface markers on lymphocytes can be used to classify tumors as containing T or B cells and as being monoclonal or polyclonal in origin.

The vast majority of orbital lymphomas are of B-cell origin with monoclonal proliferation from a single neoplastic cell. Well-differentiated monoclonal lesions have associated systemic disease in 20% of cases, while the association increases to 60% with less well-differentiated lesions.

The MIB-1 proliferation rate and p53 positivity may aid in the prediction of disease stage and disease progression, whereas polymerase chain reaction (PCR) testing can support the diagnosis and reduce the number of histologically indeterminate lesions. [43]


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