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

Abstract

Aim: The aim of this study was to report outcomes following radiation therapy in patients with biopsy-proven extranodal marginal zone lymphoma of the ocular adnexa and uvea. Methods: Records from a single institution were retrospectively reviewed from January 1997 to December 2015. The mean follow-up duration was 38 months (range 0-194). Radiation therapy was administered to 77 eyes (60 patients); 57 of the 77 eyes (74%) were treated with radiation only (range 20-36 Gy, median 15 fractions). Radiation cataract, radiation retinopathy, and optic neuropathy assessments were performed on all eyes treated with radiation. Results: 100% of the 47 patients treated with radiation therapy only had local control with an average dose of 26.5 Gy (median 25.2 [range 20-36] Gy; 150-200 cGy per fraction). Four patients lost 2 lines or more of vision after radiation. The most common complication of radiation therapy was cataract formation/progression in 19 eyes (25%). Radiation retinopathy was observed only in 1 patient (1%). Conclusion: Our results confirm that radiation therapy (median 25 Gy) for extranodal marginal zone lymphoma of the ocular adnexa is associated with high local control and low risk of visually significant complications.

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

“…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].…”

“…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.…”

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

“…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].…”

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

“…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 ].…”