Dural MALT Lymphoma with Disseminated Disease

Abstract

Central nervous system (CNS) lymphoma involving the dura mater is very rare and histologically is usually a subtype of non-Hodgkin's lymphoma (NHL) termed mucosa-associated lymphoid tissue (MALT) lymphoma. We present a case of a 46-year old woman with dural MALT lymphoma that was found to also involve a lacrimal gland, inguinal lymph nodes, and bone marrow. Magnetic resonance imaging of the brain showed an extra-axial enhancing mass approximately 6 cm in maximum diameter along the right frontotemporal convexity. Histopathology of the resected dural mass showed MALT lymphoma expressing CD20, CD52, CD19, and CD38. Molecular studies of the B-cell receptor heavy chain demonstrated monoclonality at the involved sites. The patient was treated with four cycles of fludarabine, mitoxantrone, and rituximab with complete remission. She had recurrence in the subcutaneous tissue of the back at 12 months but has remained free of intracranial disease for 31 months. A review of the literature reveals 57 cases of dural MALT lymphoma. Only 4 had extra-CNS involvement at presentation, and only 3 had local recurrence of the dural tumor. Because of the indolent behavior of this tumor, the intracranial portion can be treated conservatively after resection with or without chemotherapy. Deferral of brain radiation can be considered with close clinical and neuroimaging follow up.

How we manage Bing–Neel syndrome

“…In a study of 21 patients with astrocytoma and solid cancers metastatic to the brain, CNS concentrations of cladribine appeared to be dose‐dependent (Saven et al , ). Clinically, fludarabine was shown to be effective in patients with CLL or MZL involving the CNS (Knop et al , ; Matmati et al , ). In patients with BNS, both cladribine and fludarabine have induced clinical benefit based on prior reports (Richards, ; Delgado et al , ; Simon et al , ; Castillo et al , ; Vos et al , ).…”

Primary Intracranial Mucosa-Associated Lymphoid Tissue Lymphoma

“…The hyperintense diffusion signal abnormality with diffusion restriction associated with lesion high cellular packing is very relevant to diagnose atypical lesions 23 since the most frequent benign meningiomas will not restrict diffusion, while lymphomas and other aggressive dural-based lesions will frequently do so. The transient "response" to corticosteroids in duralbased lesions may also favour the diagnosis of lymphoma over benign meningiomas 24 . The second most frequent site of MALT lymphoma involvement intracranially is the glomus of the choroid plexus in the lateral ventricles, an ectodermal origin structure devoid of a blood brain barrier.…”

Rare case of cerebral MALToma presenting with stroke-like symptoms and seizures

“…As there are so few cases there is no evidence-based management and it is unclear whether systemic chemotherapy is necessary. Owing to the nature of CNS MALToma, there is a good curative rate7 with a low recurrence rate reported after surgical resection and local radiotherapy 8Learning points

Central nervous system mucosa-associated lymphoid tissue lymphomas (CNS MALTomas) are a rare subtype of primary CNS tumour that can present with stroke-like features.

To acknowledge the limitations of radiological investigations as well as the value of tissue diagnosis.

Recognition and histological diagnosis of this subtype is important as they remain indolent and localised and can be cured with surgical resection and local radiotherapy.

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