Primary Diffuse Large B-cell Lymphoma of Dura and Cranial Vault

Abstract

Primary central nervous system lymphoma (PCNSL) is an extranodal non-Hodgkin lymphoma that is deep seated and despite effective treatment regimens the prognosis is poor with a 5-year survival of 10%. Primary dural lymphoma a rare form of PCNSL, is usually a low-grade B-cell lymphoma of the mucosa-associated lymphoid tissue type or marginal zone B-cell lymphomas. Primary dural diffuse large B-cell lymphoma, as an aggressive form, is extremely rare with only 13 cases reported in literature. We present a short series of 3 cases of this rare variety of primary dural lymphomas along with the review of literature. They mimic meningiomas and involve the cranial vault, with scalp swelling being the most common presentation. They are staged as Ann-Arbor stage IE primary high-grade large B-cell lymphoma of the dura and, at present, owing to rarity of the disease, are treated as PCNSLs. Whether the high-grade dural lymphomas behave like aggressive PCNSLs and exact management in view of the fact that they appear to be outside blood-brain barrier will require the review of larger series and case reports.