Extranodal Marginal Zone Lymphoma of Ocular Adnexa: Outcomes following Radiation Therapy

Platt, Sean; Zahrani, Yahya Al; Singh, Nakul; Hill, Brian T.; Cherian, Sheen; Singh, Arun D.

doi:10.1159/000453615

Cited by 23 publications

(31 citation statements)

References 39 publications

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“…In most cases, the disease was in a T2N0M0 stage and no relapse was noted. This finding is consistent with the current literature that supports the use of EBRT for most cases of primary OEL, especially for low-grade lymphomas such as EMZL, which represented the majority of cases in our series [38][39][40][41][42]. A newly published review by the American Academy of Ophthalmology on treatment of OEL has documented that EBRT has a very good effect on local control, disease-free survival and overall survival in patients with EMZL [43].…”

Section: Discussionsupporting

confidence: 92%

“…No association was detected in our study between the T category and recurrence. The AJCC proposed additional factors recommended just for clinical care: the International Prognostic Index (IPI), tumor cell growth fraction (Ki-67, MIB-1) and lactate dehydrogenase level [ 34 , 35 , 36 , 37 , 38 , 39 , 40 , 41 , 42 , 43 , 44 , 45 , 46 , 47 , 48 , 49 , 50 , 51 , 52 , 53 , 54 , 55 ]. Nonetheless, these factors were not considered in this series as complete data were available only for a small percentage of studied patients.…”

Section: Discussionmentioning

confidence: 99%

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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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Section: Discussionsupporting

confidence: 92%

Section: Discussionmentioning

confidence: 99%

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

Savino

Midena

Blasi

et al. 2020

Cancers

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“…Current guidelines recommend implementation of RT in patients with H. pylori-associated gastric EMZL not achieving remission after antibiotic therapy or stage I EMZL at other primary locations. RT was repeatedly shown to be a highly effective treatment [1,6,19,20]. However, some physicians are reluctant to use RT in view of potential side effects and despite improved safety of modern techniques [21].…”

Section: Introductionmentioning

confidence: 99%

Treatments and Outcomes in Stage I Extranodal Marginal Zone Lymphoma in the United States

Alderuccio

Florindez

Reis

et al. 2021

Cancers

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A considerable number of patients with extranodal marginal zone lymphoma (EMZL) are diagnosed with stage I disease. Information on treatments and survival by primary location remains limited. We extracted data from the Surveillance, Epidemiology, and End Results (SEER) database to assess treatment, primary location, and survival of patients with stage I EMZL. Results show that 7961 patients met inclusion criteria. Observation (no treatment) was the most common approach (31%) followed by radiation therapy (RT, 23%). The median overall survival (OS) was 17.3 years (95%CI 16.3 to 18.3). Shorter survival was observed in patients with stage I EMZL compared to expected survival in a cohort derived from the general U.S. population matched by sex, age, and calendar year at diagnosis. However, similar survival was observed in RT-treated patients. We identified age ≥ 60 years (SHR = 4.00, 95%CI 3.10–5.15; p < 0.001), higher grade transformation (SHR = 4.63, 95%CI 3.29–6.52; p < 0.001), and primary lung EMZL (SHR = 1.44, 95%CI 1.05–1.96; p = 0.022) as factors associated with shorter lymphoma-specific survival (LSS). Conversely, primary skin location (SHR = 0.50, 95%CI 0.33–0.77; p = 0.002) was associated with longer LSS. Our results support the use of RT as the preferred approach in localized EMZL.

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“…Moderate-dose EBRT (20–30 Gy delivered in 1.8–2 Gy daily fractions) has been the gold-standard treatment for the last number of decades. B cell lymphomas of the ocular system have traditionally been treated with similar doses with consistently high rates of local control for lesions of the adnexa [ 20 , 21 ]. Treatment of lesions of the choroid with similar EBRT doses (mean 30–36 Gy in 1.8–2 Gy daily fractions) has generated similar rates of tumour regression and local control, albeit with iatrogenic cataract, dry eye syndrome and decrease in BCVA being a common consequence of radiotherapy [ 1 , 3 ].…”

Section: Discussionmentioning

confidence: 99%

Ultra-low dose external beam radiotherapy for presumed choroidal lymphoma: a case report

Flanagan

Kibrom

et al. 2022

J Ophthal Inflamm Infect

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Primary choroidal lymphoma is a rare, slowly progressive intraocular malignancy. Most are low grade B cell lymphomas, often involving tissues adjacent to the choroid such as the subconjunctival space, lacrimal gland or orbit. Ideally, these lesions are biopsied to establish histopathological diagnosis. The most accessible ocular structure is biopsied. Obtaining tissue by transvitreal choroidal biopsy imparts a small but significant risk of ocular morbidity, including the need for multiple surgeries, retinal detachment and vision loss.External beam radiotherapy (EBRT) is a common and effective treatment of low-grade lymphomas. EBRT has been found to very successfully treat primary marginal zone lymphomas of the ocular adnexa, which are typically of the same cell type as most primary choroid lymphomas. Ultra-low dose EBRT, most commonly using a total dose of 4 Gy, has been shown to be as effective as higher doses of radiotherapy for follicular or marginal zone lymphomas. The use of this low dose regimen for conjunctival lymphomas has been recently explored. The role of EBRT, and especially ultra-low dose EBRT, for treatment of primary choroidal lymphoma has been confined to case reports.We describe a case of presumed primary choroidal lymphoma diagnosed on clinical findings alone as the risks of ocular biopsy were deemed too high, and report outcome following treatment with ultra-low dose EBRT.

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Extranodal Marginal Zone Lymphoma of Ocular Adnexa: Outcomes following Radiation Therapy

Cited by 23 publications

References 39 publications

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

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

Treatments and Outcomes in Stage I Extranodal Marginal Zone Lymphoma in the United States

Ultra-low dose external beam radiotherapy for presumed choroidal lymphoma: a case report

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