Primary Ocular Adnexal MALT Lymphoma: Single institution experience in a large cohort of patients
Extranodal marginal zone B-cell lymphoma is the most common orbital tumour. We conducted a retrospective analysis to examine: a) the impact of initial presentation and staging on outcome; b) response to various treatment modalities and the effect of the latter on recurrence. 90 patients with primary...
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Published in | British journal of haematology Vol. 152; no. 1; pp. 72 - 80 |
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Main Authors | , , , |
Format | Journal Article |
Language | English |
Published |
18.11.2010
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Online Access | Get full text |
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Summary: | Extranodal marginal zone B-cell lymphoma is the most common orbital tumour. We conducted a retrospective analysis to examine: a) the impact of initial presentation and staging on outcome; b) response to various treatment modalities and the effect of the latter on recurrence.
90 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 < 30.6 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 30.6 Gy should be given in Ann-Arbor stage I disease, since lower doses may be more frequently associated with relapses, including CNS relapses. |
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Bibliography: | Contributed equally |
ISSN: | 0007-1048 1365-2141 |
DOI: | 10.1111/j.1365-2141.2010.08429.x |