Lymphoma of the Ocular Adnexa: A Study of 353 Cases

Ferry, Judith A.; Fung, Claire Y.; Zukerberg, Lawrence; Lucarelli, Mark J.; Hasserjian, Robert P.; Preffer, Frederic I.; Harris, Nancy L.

doi:10.1097/01.pas.0000213350.49767.46

Cited by 365 publications

(314 citation statements)

References 69 publications

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“…Most are primary tumours and are usually NHL of B-cell type: the most common primary lymphoma subtype occurring in the ocular adnexa is the low-grade malignant extranodal marginal zone B-cell lymphoma (EMZL), accounting for approximately two thirds of all ocular adnexal lymphomas. [5][6][7][8][9] Interestingly, the EMZL is also the most common primary lymphoma occurring in the choroid. 3 In the following review, the molecular pathologies of only DLBCL and EMZL will be discussed.…”

Section: Introductionmentioning

confidence: 99%

Molecular pathology of lymphoma

Coupland

2012

Eye

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Ocular lymphomas can be divided into intraocular lymphomas and ocular adnexal lymphomas. The vitreoretinal lymphomausually a diffuse large B-cell lymphoma (DLBCL) of high-grade malignancy-is the most common lymphoid malignancy arising in the eye, while the extranodal marginal zone B-cell lymphoma (EMZL), an indolent often recurrent tumour, occurs most frequently in the ocular adnexal tissue. The two lymphoma subtypes differ significantly in their clinical presentation, subsequent course and outcome as well as in their underlying morphological, immunophenotypical and genetic features. The molecular processes involved in DLBCL and EMZL development are complex, and include chromosomal translocations, mutations caused by aberrant somatic hypermutation, sporadic somatic mutations, and copy number alterations, characterized by deletions and amplifications. These lead to alterations in particular signalling pathways, which in turn activate transcription factors, such as nuclear factor NF-kB. This review provides an overview of the histological features of DLBCL and EMZL, and discusses the current insights into the molecular mechanisms underlying the development of these tumours, when they occur systemically and particularly when they arise in ocular tissues.

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Section: Introductionmentioning

confidence: 99%

Molecular pathology of lymphoma

Coupland

2012

Eye

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“…Lymphoma is the most common orbital malignancy, has a predilection for the elderly, and classically presents as painless proptosis or adenexal swelling over a duration of 5-7 months. 7,9 Non-Hodgkin's lymphoma is reported 11 to have an orbital presentation in 2.4% of all non-Hodgkin's lymphoma cases, while its prevalence is about 5% in systemic NHL. 9 According to Liang et al, 12 the incidence of secondary central nervous system lymphoma in a patient with orbital non-Hodgkin's lymphoma is 5.9%, and the most common form of central nervous system involvement in lymphoma is leptomeningeal metastasis.…”

Section: Discussionmentioning

confidence: 99%

“…The second most common type is either follicular, 12% to 23%, or DLBCL, 7% to 21%, depending on the study. [4][5][6][7][8][9] The frequency of ocular adnexal lymphoma with localization to the orbit has been cited as 54%. 5 It has been suggested that it may be more appropriate to analyze ocular 10 found that for secondary ocular adnexal lymphoma, follicular type is most common (33%-66%) followed by (in approximate decreasing frequency) multiple myeloma/plasmacytoma, lymphoplasmocytic lymphoma/immunocytoma, mantle cell lymphoma, DLBCL, Burkitt lymphoma, MALT lymphoma and chronic lymphocytic leukemia.…”

Section: Discussionmentioning

confidence: 99%

Systemic non-Hodgkin’s lymphoma involving the orbit and leptomeninges

Ts¹,

Anis²,

Doych³

et al. 2010

Digit J Ophthalmol

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SummaryWe report a case of diffuse large B-cell lymphoma in a 46-year-old female presenting in an unusual manner with stage IVB disease including concurrent orbital and leptomeningeal involvement. The cytologic features and cytogenetics of the malignancy are noted, and the management and progression of the disease, with attention to orbital involvement, is recorded for a period of over 2.5 years, until the patient's death. Case ReportA 46-year-old woman presented to our clinic with a complaint of intermittent proptosis, diplopia, and ptosis in her right eye for 4 months. During this period, she also noted a loss of balance, gait difficulties, and lower extremity weakness, numbness, and tingling. For the 6 months prior to presentation, she had also experienced weight loss and fatigue.Ophthalmologic examination revealed visual acuities of 20/20 in both eyes. External examination demonstrated proptosis, soft tissue swelling in the upper and lower lids, and scleral "show" with mild conjunctival injection and chemosis in the right eye (Figure 1). Enlarged, matted, and non-tender cervical lymphadenopathy was also found. There was marked restriction of the right eye on adduction. The rest of the anterior and posterior segment examinations were unremarkable in both eyes. Neurological examination was pertinent for bilateral distal lower extremity motor and sensory deficits, diminished reflexes in the knees, and absent reflexes in the ankles. A Romberg test was positive.Computed tomography of the head showed an enhancing soft tissue mass in the medial aspect of the right orbital fossa. MRI demonstrated an enhancing right orbital mass with intraconal and extraconal involvement, which communicated with the right ethmoid sinus. An MRI of the spine showed diffuse enlargement and enhancement of the lumbar and sacral nerve roots.Biopsy of the left cervical lymph node (Figure 2) demonstrated diffuse large B-cell lymphoma (DLBCL). Diagnosis was confirmed with immunohistochemical stains that showed positive staining for B-cell markers CD19, CD20 (Figure 3) and lambda light chain restriction. Staining for CD3 and CD5 was negative, ruling out a T-cell lymphoma, small lymphocytic lymphoma/ chronic lymphocytic leukemia, and mantle cell lymphoma; negative staining for CD10 ruled out a follicular lymphoma. 1 Furthermore, the BCL-1 stain showed heavy background staining, and definitive positivity was not ascertained, while fluorescence in situ hybridization of 100 cells did not show a [t(11;14)] rearrangement-a typical hallmark of mantle-cell lymphoma. 2 The Ki-67 proliferation index was 20% to 30%. Cytogenetic stud-

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“…Most patients present with disseminated disease, with lymphadenopathy, often with splenomegaly, and frequent involvement of the bone marrow. A minority of patients present with extranodal disease, most often involving the gastrointestinal tract 1 and occasionally Waldeyer ring 2 , but rarely involving other sites, such as the skin 3 or the ocular adnexa 4 . Nearly all patients with extranodal presentation of MCL will be found to have lymphadenopathy or more widespread disease on staging.…”

Section: Dear Editormentioning

confidence: 99%