Literature review on primary CNS MALT lymphoma treatment and diagnosis

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Frontiers in Medicine Published May 11, 2026 Medically reviewed May 11, 2026 DOI ↗ By Dr. Julia Lee, PhD · Oncology, Genomics & Drug Development

Key Takeaway

Consider MALT lymphoma in the differential diagnosis of intraventricular lesions, based on limited case evidence.

This source is a literature review and case report describing a 62-year-old man with primary central nervous system mucosa-associated lymphoid tissue lymphoma. The review synthesizes 8 previously reported cases in addition to the new case. The intervention was gross total resection followed by postoperative treatment with ifosfamide combined with a Bruton's tyrosine kinase (BTK) inhibitor, and adjuvant radiotherapy. The main synthesized finding is no evidence of recurrence at the latest follow-up for the reported case. The authors note that many previously reported cases were initially misdiagnosed on imaging and that potential infectious or inflammatory triggers in ventricular MALT lymphoma remain unclear. A hypothesis is proposed that lateral ventricular tumors may originate from the pia mater folds of the choroid plexus. Limitations include the small number of cases and the preliminary nature of the findings. The authors suggest considering MALT lymphoma in the differential diagnosis of intraventricular lesions, but the practice relevance is constrained by the lack of controlled data.

Study Details

Study typeSystematic review

EvidenceLevel 1

PublishedMay 2026

View Original Abstract ↓

Primary central nervous system (CNS) mucosa-associated lymphoid tissue (MALT) lymphoma is a rare, low-grade B-cell lymphoma that typically arises in the dura, and its occurrence within the ventricular system is exceedingly rare. We report a case of primary CNS MALT lymphoma located in the right lateral ventricle of a 62-year-old man. The patient underwent gross total resection, followed by postoperative treatment with ifosfamide combined with a Bruton’s tyrosine kinase (BTK) inhibitor, and adjuvant radiotherapy. At the latest follow-up, no evidence of recurrence was observed. A review of the literature revealed only eight previously reported cases of ventricular MALT lymphoma, many of which were initially misdiagnosed on imaging as meningioma, metastasis, or ependymoma. Although chronic inflammation has been implicated in MALT lymphomas outside the CNS, potential infectious or inflammatory triggers in ventricular MALT lymphoma remain unclear. This case expands the recognized anatomical spectrum of CNS MALT lymphoma and proposes a hypothesis that lateral ventricular tumors may originate from the pia mater folds of the choroid plexus, highlighting the need to consider MALT lymphoma in the differential diagnosis of intraventricular lesions.