Ocular adnexal lymphoproliferative disease: a series of 73 cases

McKelvie, Penelope; McNab, Alan A.; Francis, Ian C; Fox, Richard M.; O’Day, Justin

doi:10.1046/j.1442-9071.2001.00450.x

Cited by 40 publications

(85 citation statements)

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“…[3][4][5][6]22,23 A higher prevalence in males was only reported in studies originating from Asian countries like Japan 15,24,25 and Korea. 7,[26][27][28] The gender difference seen in our series was due to a higher prevalence of conjunctival disease in females, with no difference at other locations.…”

Section: Discussionmentioning

confidence: 99%

“…2,3 While different subtypes of NHL may present as POAL, extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALT) is the most common histological subtype, accounting for 38% to 64% of POAL cases. [4][5][6][7] Multiple retrospective studies have described clinical and epidemiological features of primary ocular adnexal MALT lymphoma (POAML), including female preponderance, frequent occurrence in the orbit, and increased incidence of bilateral involvement. 8 Association with Chlamydia psittaci infection in certain geographic areas has also been reported.…”

Section: Introductionmentioning

confidence: 99%

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Long-term course of patients with primary ocular adnexal MALT lymphoma: a large single-institution cohort study

Desai

Joag

Lekakis

et al. 2017

Blood

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Key Points• POAML (specifically Ann Arbor stage I disease) has an excellent clinical outcome, with only a few patients succumbing to lymphoma.• POAML patients face a continuous risk of distant relapse, including in the central nervous system, and transformation to aggressive lymphoma.While primary ocular adnexal mucosa-associated lymphoid tissue (MALT) lymphoma (POAML) is the most common orbital tumor, there are large gaps in knowledge of its natural history. We conducted a retrospective analysis of the largest reported cohort, consisting of 182 patients with POAML, diagnosed or treated at our institution to analyze long-term outcome, response to treatment, and incidence and localization of relapse and transformation. The majority of patients (80%) presented with stage I disease. Overall, 84% of treated patients achieved a complete response after first-line therapy. In patients with stage I disease treated with radiation therapy (RT), doses ‡30.6 Gy were associated with a significantly better complete response rate (P 5 .04) and progression-free survival (PFS) at 5 and 10 years (P < .0001). Median overall survival and PFS for all patients were 250 months (95% confidence interval [CI], 222 [upper limit not reached]) and 134 months (95% CI, 87-198), respectively. Kaplan-Meier estimates for the PFS at 1, 5, and 10 years were 91.5% (95% CI, 86.1% to 94.9%), 68.5% (95% CI, 60.4% to 75.6%), and 50.9% (95% CI, 40.5% to 61.6%), respectively. In univariate analysis, age >60 years, radiation dose, bilateral ocular involvement at presentation, and advanced stage were significantly correlated with shorter PFS (P 5 .006, P 5 .0001, P 5 .002, and P 5 .0001, respectively). Multivariate analysis showed that age >60 years (hazard ratio [HR] 2.44) and RT<30.6Gy (HR54.17) were the only factors correlated with shorter PFS (P 5 .01 and P 5 .0003, respectively). We demonstrate that POAMLs harbor a persistent and ongoing risk of relapse, including in the central nervous system, and transformation to aggressive lymphoma (4%), requiring long-term follow-up. (Blood. 2017;129(3):324-332)

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Long-term course of patients with primary ocular adnexal MALT lymphoma: a large single-institution cohort study

Desai

Joag

Lekakis

et al. 2017

Blood

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“…[1][2][3][4][5][6] Lymphomagenesis is driven by multiple accumulated genetic abnormalities that determine the survival of neoplastic cells and involve mainly cell-cycle and apoptotic pathways. 7 MALT lymphomas in particular are a paradigm of alternative genetic events, involving different chromosomal aberration and/or apoptosis control protein expression, leading to the deregulation of apoptotic pathways through the activation of the NF-kB pathway.…”

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confidence: 99%

Nuclear bcl10 expression characterizes a group of ocular adnexa MALT lymphomas with shorter failure-free survival

Franco

Camacho

Caleo³

et al. 2006

Modern Pathology

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Ocular adnexa B-cell lymphomas are a relatively rare group of extranodal lymphomas, marginal-zone B-cell lymphomas of mucosa-associated lymphoid tissue (MALT lymphomas) being the most frequent type at this location. As with other nongastrointestinal MALT lymphomas, ocular adnexa MALT lymphomas have distinct characteristics from those of the gastric MALT model, implying specific pathogenic events, which could be of interest in the prediction of clinical behavior and the choice between therapeutic options. In a series of 39 cases of ocular adnexa MALT lymphomas, studied using a tissue microarray, we observed that the most frequent alteration was related to apoptosis regulation. Thus, caspase 3 activity was completely abolished, and phosphorylated IjBa, a marker of NF-jB activation, showed increased expression, while cases with an increased number of large cells displayed increased expression of survivin and other cell-cycle-related proteins, such as cyclin A, cyclin E and Ki67, and p16 expression was reduced. There were no occurrences of t(11;18)(q21,q21), while 5/37 cases exhibited t(14;18)(q32;q21). Aberrant nuclear expression of bcl10 was observed in 11 cases, independently of the presence of translocations, and was significantly associated with phosphorylated IjBa expression and a reduced TdT-mediated biotin-dUTP nicked-end labeling apoptotic index. Moreover, patients with tumoral bcl10 nuclear expression showed shorter failure-free survival.

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“…The overall risk of lymphoma related death secondary to DLBCL has been reported as 38%, however a recent study by Madge et al 15 of ocular adnexal DLBCL found that in 11 patients with concurrent systemic disease at presentation, 5-year KaplanMeier survival estimates were 36%. 5 Our patient presented with simultaneous orbital and leptomeningeal involvement, perhaps a unique presentation. Having a high clinical suspicion and early diagnosis and intervention is important in orbital lymphoma cases since there have been reports stating prior or concurrent systemic disease as being the most significant predictive factor for lymphoma-related deaths.…”

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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Ocular adnexal lymphoproliferative disease: a series of 73 cases

Cited by 40 publications

References 0 publications

Long-term course of patients with primary ocular adnexal MALT lymphoma: a large single-institution cohort study

Long-term course of patients with primary ocular adnexal MALT lymphoma: a large single-institution cohort study

Nuclear bcl10 expression characterizes a group of ocular adnexa MALT lymphomas with shorter failure-free survival

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

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