Ocular/adnexal lymphoma: dissimilar to systemic lymphoma

Rubinstein, Tal J.; Aziz, Hassan A.; Bellerive, Claudine; Sires, Bryan S.; Hing, Andrew W.; Habermehl, Gabriel; Hsi, Eric D.; Singh, Arun D.

doi:10.1016/j.survophthal.2017.08.005

Cited by 15 publications

(12 citation statements)

References 38 publications

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“…Non-B-cell conjunctival lymphomas, derived from T lymphocyte or natural killer cells, are uncommon and carry a particularly poor prognosis, with a high predilection for systemic dissemination. Data on these lymphomas is scarce [3, 7–12].…”

Section: Reviewmentioning

confidence: 99%

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Classification, diagnosis, and management of conjunctival lymphoma

2019

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Lymphoma is a malignant lymphoproliferative tumor that can involve the conjunctiva. Approximately 5–15% of all extranodal lymphomas are found in the ocular adnexal region, with approximately 25% of those involving the conjunctiva. Ninety-eight percent of conjunctival lymphomas arise from B-lymphocytes. The most common subtype of conjunctival lymphoma is extranodal marginal zone lymphoma (80%), followed by follicular lymphoma (8%), diffuse large B-cell lymphoma (3%) and mantle cell lymphoma (3%). Natural killer and T cells (NK/T) are rare causes of lymphoma. While most conjunctival lymphomas are localized to the ocular adnexa at the time of presentation, systemic examination and management are of key importance in the long-term care of the patient. This review outlines the classification, etiology, presentation, diagnosis, and management of conjunctival lymphoma. The novel use of high resolution optical coherence tomography, both as a diagnostic tool and as a means for ongoing evaluation during treatment, is illustrated. Treatment options discussed include external beam radiation, chemotherapy, immunotherapy, antibiotic therapy, and combination regimens. Future investigation of the etiology and pathogenesis of conjunctival lymphoma is expected to reveal opportunities for innovative and individualized therapeutic agents. Collaboration between multiple disciplines is key in the advancement of the field.

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Section: Reviewmentioning

confidence: 99%

“…Up to 80% of T-cell NHL in the conjunctiva arise as secondary lymphomas from a distant source [3]. With any subtype of conjunctival lymphoma, long-term follow-up is key as systemic disease may develop months or years after the initial diagnosis [7, 15, 24].…”

Section: Reviewmentioning

confidence: 99%

Classification, diagnosis, and management of conjunctival lymphoma

2019

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“…The majority (73%) of OELs arise as a primary orbital or eyelid disease, whereas 27% occur secondarily as a metastatic spread. Secondary OELs are generally assumed to be of similar histologic subtype as the systemic lymphoma; however, in a large retrospective study, patients initially diagnosed with low-grade orbital lymphoma were subsequently diagnosed with a dissimilar systemic high-grade lymphoma, due to a Richter transformation [ 9 ]. Few researchers have focused exclusively on the biology of OEL [ 10 , 11 , 12 , 13 , 14 ].…”

Section: Introductionmentioning

confidence: 99%

Orbital and Eyelid B-Cell Lymphoma: A Multicenter Retrospective Study

Savino

Midena

Blasi

et al. 2020

Cancers

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Background: The aim of this study was to analyze patients diagnosed, staged and treated for orbital and eyelid B-cell lymphoma (OEL). Methods: One hundred and forty-one cases of OEL were included in this study. Primary endpoints were to analyze the histopathologic findings, the main risk factors and the type of treatment and to correlate them with recurrence of OEL. The secondary endpoint was to determine the progression-free survival (PFS) time. Results: Extranodal marginal zone B-cell lymphoma was the most frequent subtype (66%), followed by small lymphocytic lymphoma (12.7%), diffuse large B-cell lymphoma (DLBCL) (9.2%), follicular lymphoma (6.6%), mantle cell lymphoma (4.3%) and Burkitt lymphoma (1.2%). The probability of relapse was influenced by the histopathologic subtype DLBCL (OR = 7.7, 95% CI 1.8–32.3) and treatment with chemotherapy (OR = 14.9, 95% CI 2.6–83.7). Multivariate analysis showed that the histopathologic subtype DLBCL and chemotherapy treatment retained statistical significance for a poorer PFS, with hazard ratios of 8.581 (p = 0.0112) and 9.239 (p = 0.0094), respectively. Conclusions: Five lymphoma subtypes were found in patients with OEL. The histopathologic subtype and the type of treatment were found to be the main factors influencing treatment outcome.

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“…It was postulated that standard treatments, such as combination chemotherapy, autologous hematopoietic stem cell transplantation, and irradiation, might be driving factors for these phenotypic changes in neoplastic cells. The correlation between MALT and DLBCL may represent a Richter transformation, [15] wherein a low-grade lymphoma (such as MALT) transforms into an aggressive type (such as DLBCL).…”

Section: Discussionmentioning

confidence: 99%