Chemotherapy is an efficient treatment in primary CNS MALT lymphoma

Guideline for the diagnosis and management of marginal zone lymphomas: A British Society of Haematology Guideline

“…3 MZLs rarely involve the central nervous system (CNS), either primarily or secondarily. Most CNS MZL is dural EMZL/MALT lymphoma, with lesions often radiologically indistinguishable meningioma [9][10][11][12][13] ; however, MRI may distinguish from meningioma by a more prominent dural tail. 14 Histological confirmation has important implications for prognosis and management, as dural EMZL/MALT lymphoma has a better prognosis than high-grade primary central nervous system lymphomas.…”

“…Due to its rarity, therapy for CNS MZL is not standardised. ISRT or ISRT plus whole brain radiotherapy (WBRT) to 24-30 Gy is most common, especially for dural tumours 15,134 and achieves excellent long-term disease control 10,11,14,15,[134][135][136] even in the presence of concomitant leptomeningeal disease. 135 Doses of 30-36 Gy produce high CR rates and low neurotoxicity, 14,135 with outcomes similar to chemotherapy alone and combined chemoradiotherapy.…”

Primary central nervous system marginal zone lymphoma

“…Given that most published cases are represented by single-patient reports, only few case series estimated an incidence for these tumours of 1.7%-6.3% among all NHL primarily arising in the CNS. [2][3][4] The growing number of case reports in the literature across recent years likely reflects an improvement in diagnosis rather than an actual increased incidence of PCNSMZL. At variance with primary diffuse large B-cell lymphoma (DLBCL) of the CNS (PCNSL), PCNSMZL exhibits a marked female predilection (69%); the reason(s) for this figure are not known.…”

“…To the best of our knowledge, only 12 cases of PCNSMZL involving the spine have been reported. 3,4,28,49,53,56,[63][64][65][66][67][68] The thoracic segment was the most common site of involvement, but cases arising in the lumbar, cervical and sacral regions have been described as well. In these patients, back and radicular pain, paraparesis, extremity weakness and urinary disturbance are the most common presenting symptoms, 28,63,64,67 followed by acute spinal cord compression.…”

“…53,56 PCNSMZL exhibits multiple sites within the CNS (brain or spinal cord) at diagnosis in 17% of reported cases. [3][4][5]19,21,24,45,49,53,54,56,62,[68][69][70][71][72]…”