Primary dural lymphoma: a rare subtype of primary central nervous system lymphoma (PCNSL)

Primary dural lymphoma: A novel concept of heterogeneous disease

“…In contrast to cerebral PDL, where the vast majority of the reported cases are MZL (98 of 126 cases, 77.7%), only two (5%) of the spinal PDL cases were diagnosed as such . This suggests a difference in predilection of lymphoma type for the anatomical site of dural involvement, which may be related to different pathogenesis.…”

“…Our literature review investigated ‘dural’, ‘epidural’ and ‘meningeal’ lymphomas, and identified many PDL cases, including 166 well‐documented cases . Particularly, PDLs diagnosed prior to the Revised European American Lymphoma (REAL) classification (1994), and where more recent reclassification was not possible, were not included.…”

“…Due to the paucity of cases in individual series, no definite treatment protocol for PDL has been fully‐established. Different treatment combinations associating local decompressive surgery, systemic chemotherapy (corticosteroids, CHOP and rituximab), intrathecal methotrexate, and adjuvant radiation therapy for local control of disease, have been tried in these cases with good outcomes over variable follow‐up periods …”

“…31 Despite the controversial existence of a Mucosa Associated Lymphoid Tissue (MALT) proper to dura mater, and the lack of definite association of any autoimmune or infectious etiologies with PDL 3 , cerebral PDL has been considered as part of the spectrum of extra-nodal MZL secondary to repetitive antigenic stimulation of a dural intrinsic lymphoid tissue of mucosa associated lymphoid tissue (MALT)-type. [3][4][5]12,28 Nonetheless, anticipating a pathophysiology specific to the spinal counterpart of PDL is not straightforward, since most of these cases are of the DLBCL-type, itself encompassing several subtypes with different pathogenesis, including the germinal center and activated non-germinal center subtypes. 32 Despite the absence of sufficient evidence, our findings suggest there might be a link between spinal PDL pathogenesis and follicular germinal center origin due to the following reasons: 2 In addition, DLBCL may arise either de novo or as transformation of underlying FBCL.…”

“…Most cases of dural lymphoma consist of secondary dural involvement in the context of systemic or other organs' primary lymphomas, and well‐documented cases of primary dural lymphoma (PDL) are less common. Primary dural lymphomas are distinct from primary lymphomas of the central nervous system (CNS) that encompasses brain, spinal cord and subarachnoid space .…”