Dural MALT lymphoma with disseminated disease

Area of Science:

• Neurology
• Oncology
• Pathology

Background:

• Central nervous system (CNS) lymphoma involving the dura mater is exceptionally rare.
• Histologically, it is typically a subtype of non-Hodgkin's lymphoma (NHL) known as mucosa-associated lymphoid tissue (MALT) lymphoma.

Observation:

• A 46-year-old woman presented with dural MALT lymphoma extending to the lacrimal gland, lymph nodes, and bone marrow.
• MRI revealed a 6 cm extra-axial enhancing mass on the right frontotemporal convexity.
• Histopathology confirmed MALT lymphoma with specific immunophenotypic markers (CD20, CD52, CD19, CD38) and monoclonal B-cell receptor gene rearrangement.

Findings:

• The patient achieved complete remission after four cycles of fludarabine, mitoxantrone, and rituximab.
• A recurrence in subcutaneous tissue occurred at 12 months, but intracranial disease remained absent for 31 months.
• Literature review identified 57 dural MALT lymphoma cases, with only 4 showing extra-CNS involvement and 3 experiencing local recurrence.

Implications:

• The indolent nature of dural MALT lymphoma supports conservative management of intracranial disease post-resection, potentially including chemotherapy.
• Deferring whole-brain radiation therapy and opting for close clinical and neuroimaging follow-up is a viable strategy.
• This case contributes to understanding the behavior and management of rare dural MALT lymphomas.