Primary ocular adnexal mucosa‐associated lymphoid tissue lymphoma (MALT): single institution experience in a large cohort of patients

Abstract

SummaryExtranodal marginal zone B-cell lymphoma is the most common orbital tumour. We conducted a retrospective analysis to examine: (i) the impact of initial presentation and staging on outcome and (ii) response to various treatment modalities and the effect of the latter on recurrence. Ninety patients with primary ocular adnexal marginal zone lymphoma (POAML) diagnosed at our institution between 1984 and 2009 were studied. POAML was associated with monoclonal gammopathy (13%) at presentation. Most POAML patients (86%) presented with Ann-Arbor stage I disease. Radiotherapy led to excellent local control, but relapses occurred in 18% of Ann-Arbor stage I patients during a median follow-up of 5 years. Local relapses, including secondary central nervous system (CNS) involvement, were observed in patients receiving radiation doses <30AE6 Gy. No differences in relapse rate and survival were observed between patients who did or did not undergo staging bone marrow biopsy. Ann-Arbor stage II-IV disease and high lactate dehydrogenase levels were associated with shorter freedom from progression. In conclusion, POAML is an indolent lymphoma with continuous risk for relapse. Radiation doses of at least 30AE6 Gy should be given in Ann-Arbor stage I disease, since lower doses may be more frequently associated with relapses, including CNS relapses.

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

“…Twenty peer-reviewed articles were reviewed from 1997 to 2013 [4,8,12,14,15,16,22,23,24,25,26,27,28,29,30,31,32,33,34,35]. All patients treated with radiation therapy only were recorded.…”

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

“…However, an association between POAML and C psittaci was reported in other geographic regions. 8,9 Consequently, doxycycline treatment was studied as an initial therapeutic approach to POAML in several studies. [37][38][39] The overall response rate ranges from 45% to 65% in most studies 37,40 but was zero in a study reported by Grunberger et al 41 The 5-year For personal use only.…”

“…[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. 9 The pathogenesis of these tumors was elucidated by an investigation of the prevalence of chromosomal translocations and mutations, which showed alterations in genes that regulate cell survival and apoptosis pathways, as well as through interrogation of immunoglobulin gene usage and analysis of somatic mutations.…”

“…These patients were identified by review of the University of Miami tumor registry and pathology database. Ninety of these patients were reported previously, 8 but their clinical follow-up was updated for this study. The institutional review board of the University of Miami approved this retrospective study, which followed the tenets of the Declaration of Helsinki.…”

“…Radiation therapy (RT) is currently a well-accepted treatment option for limited stage POAML, resulting in up to 85% to 100% longterm complete remission (CR) rates. 8,16,17 However, cohort size and follow-up duration are limited in many of these studies. Furthermore, there is no consensus on the optimal radiation dose.…”