Marginal zone lymphoma of the thoracic dura causing spinal cord compression

Abstract

Primary extra-nodal marginal zone B cell lymphoma (Ex-MZBCL) or mucosa-associated lymphoid tissue (MALT) lymphoma of the cranial dura is a rare but well-known entity. We describe a 58-year-old woman with primary MALT lymphoma of the spinal dura causing extreme thickening of the dura and spinal cord compression who initially presented with acute spinal cord compression from a chronic epidural lesion. She was treated with surgery and radiotherapy and diagnosed with a mature B-cell lymphoma based on gene rearrangement studies. Two years following the completion of radiotherapy, she presented with an increase in the size of the residual mass that was suggestive of an epidural lesion. On re-exploration, no epidural lesion was found; however, the dura was extremely thickened causing spinal cord compression. Clinical course, histological evaluation, immunostaining and gene rearrangement studies resulted in a final diagnosis of primary Ex-MZBCL of the spinal dura. To our knowledge, this is the first report of Ex-MZBCL in the spinal dura. This diagnosis should be considered when evaluating spinal cord lesions in patients with primary central nervous system (CNS) lymphoma, especially recurrent lesions, since this group of tumors carries a favorable outcome compared to other primary CNS lymphomas.