Primary Low-Grade B-Cell Lymphoma of the Dura

Abstract

The clinicopathologic findings in five patients with primary low-grade B-cell lymphoma of the intracranial dura are described. All patients were women, 40-62 years of age, who presented with focal neurologic symptoms. Radiologic studies showed a well-localized dural mass in each case, raising a preoperative diagnosis of meningioma. Cytologically these were composed of a diffuse infiltrate of small lymphocytes with plasmacytoid differentiation, with a variable admixture of centrocytelike cells. Lambda light chain restriction was found in three cases, and kappa light chain in one. VJ polymerase chain reaction for immunoglobulin heavy-chain rearrangement was positive in three of four cases, including one case in which immunostaining results were equivocal. Staging procedures did not show involvement at any other site. Therapy consisted of radiation (n = 3), chemotherapy (n = 1), or both (n = 1), with excellent response. There was no evidence of recurrence or subsequent dissemination at follow-up of up to 63 months. Low-grade B-cell lymphomas arising in the intracranial dura are rare but appear to be similar to other low-grade B-cell lymphomas arising in extranodal sites in terms of clinical presentation as stage 1E disease, indolent behavior, and favorable response to treatment, suggesting that they may be part of the mucosa associated lymphoid tissue (MALT) lymphoma spectrum. They appear to arise at dural sites where meningothelial cells are concentrated.

Follicular lymphoma of the dura associated with meningioma: A case report and review of the literature

“…4,17 This is also a possible presentation for cutaneous lymphomas of the cranial vault, among which only aggressive large B cell lymphoma had been reported to infiltrate the meninx, 18,19 unlike the primary cutaneous FL that has been reported in only one instance to invade the galea. 20 Additionally, the tumor reported here caused a large, plaque-like fibrous thickening of the dura, as previously described in other Malt-typeindolent meningeal reactions, 11 as well as in inflammatory reactions, 21 indicating a possible longstanding lymphoproliferative disease, forerunning high-grade lymphoma development. …”

“…Isla et al, 4 in their review of primary cranial vault lymphomas, pointed out that all 7 cases published up to 1996, including their low-grade FL case, showed no evidence of disease (NED) at 5-month to 3-year follow up. Similarly, Kumar et al, 11 in their series of primary Malttype dural lymphoma cases, obtained NED at 9-to 63-month follow up in all patients.…”

The genetic landscape of dural marginal zone lymphomas

“…The dura is a recognized but uncommon, primary site of occurrence of marginal zone lymphoma (MZL) and rare cases of intra-parenchymal MZLs, occurring in a perivascular location, have also been described [3, 4]. A female predilection has been documented for dural MZLs, and since they often present as solitary masses, they can mimic meningioma's clinically and on imaging studies [5–7]. Most patients are cured following excision and local adjuvant therapy but systemic recurrences have occasionally been reported [8].…”

“…Most patients are cured following excision and local adjuvant therapy but systemic recurrences have occasionally been reported [8]. Based on their morphologic and phenotypic features, primary dural MZLs are considered similar to mucosa-associated lymphoid tissue (MALT) lymphomas [5, 9]. Intriguingly, a high proportion of dural MZLs (up to 47%) show a prominent, light chain-restricted plasmacytic component, with a subset displaying clonal IgG4+ plasma cells in the absence of an IgG4-related disorder [6, 7].…”

Linfoma de células B tipo MALT, de baixo-grau, primitivo da dura-máter: relato de caso

“…Por outro lado, o linfoma primário de células B das meninges é excepcional [7][8][9] . Kumar et al 10 descrevem cinco casos do ponto de vista clínico e histológico, sugerindo que esses tumores fazem parte do linfoma tipo MALT, representando muitos, senão a maioria dos casos de localização extra-nodal do linfoma de baixo grau de células B [11][12][13] . Do ponto de vista histológico, é idêntico ao linfoma tipo MALT do tubo digestivo e de outras sedes.…”