Primary dural lymphoma: A novel concept of heterogeneous disease

Abstract

Spinal primary dural lymphoma (PDL) is uncommon with a total of 37 previous well-documented cases reported, including one diagnosed in the authors' institution. More recently we encountered an additional case of spinal PDL that, similarly to our previous case, was grade 1-2 follicular B-cell PDL. Our two cases were diagnosed over a 3-year interval in a 72-year-old female and a 74-year-old male, respectively. An exhaustive literature review on PDL was performed consequently to reveal that: (i) spinal and cerebral sites of involvement by PDL are constantly mutually exclusive; and (ii) unlike cerebral PDL, which is usually of marginal zone B-cell type, only two of the 38 cases of spinal PDL were diagnosed as such, diffuse large B-cell lymphoma being the most commonly encountered type in the spine. This divergence infers that, in contrast to the prevailing concept that PDL is a unique disease group, PDL appears to be rather heterogeneous with a difference in predilection of lymphoma type for the anatomical site of dural involvement. Such a site-specific lymphoma-type predilection phenomenon, well-recognized in other organ systems, has not been acknowledged in PDL. This report brings new insights into PDL, and may contribute to a better understanding of nervous system pathophysiology and lymphoma classification.

Primary Dural Spinal Lymphoma Presentation of a Rare Spinal Tumor Case

“…Spinal primary dural lymphoma (PDL) is uncommon, with a total of 38 well-documented case reports [ 4 ]. The most common masses seen at the upper thoracic region include primary bone tumors, metastasis, multiple myeloma, and nonspecific pyogenic infections ( Staphylococcus aureus infections (60%), Enterobacter , Salmonella , Klebsiella , Pseudomonas , and Serratia ).…”

“…In 119 patients with primary bone lymphoma, surgical intervention was needed for pathologic fractures, avascular necrosis, spinal cord compression (2 cases), or lesions on the weight-bearing bones, which compromise stability and/or joint motion [ 6 ]. Mneimneh et al [ 4 ] reviewed 38 cases involving the dural spinal cord in which the thoracic region was prominent and described primary dural lymphoma in 2 cases.…”

“…The entity of primary spinal dural lymphoma is totally different from spinal cord lymphoma both in its characteristic and prognostic features which are more favorable and also different from cranial dural lymphoma by its histopathologic features which show more insidious course [ 4 , 8 , 10 – 12 ].…”

“…Spinal dural involvement in lymphoma is an entity diagnosed with secondary progression of systemic or other organs' disease which is metastatic primary lymphoma dissemination [ 1 , 2 ]. Solely spinal dural lymphoma invading epidural/extradural region (which is also hardly distinguishable from dura) implying diffuse large B-cell lymphoma DLBCL (non-Hodgkin B-cell lineage) is less common [ 1 – 4 ] than cerebral dural lymphoma where marginal zone lymphoma is predominant [ 4 ]. Lymphoid tissue is absent in the dura, the pathogenesis of PDL is still unclear, and many hypotheses have been formulated including the role of chronic inflammatory process, chronic infection, autoimmune disease, and the meningoepithelial component [ 5 – 8 ].…”

Primary Spinal Marginal Zone Lymphoma Relapse at a Different Spinal Level after Remission of the Primary Lesion

“…Extranodal MZL (MALT lymphoma) rarely occurs in the CNS. Thirty-seven patients with MALT lymphomas in the CNS have been reported 7. In most reported patients, the lesions were localized at the cranial dura mater in the head and MALT lymphomas originating from the spine are extremely rare.…”