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Literature review on primary CNS MALT lymphoma treatment and diagnosisA Rare Brain Tumor in the Ventricles Was Found and Treated

AI-generated summary of the cited source, checked by automated accuracy review. How we work

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.

A 62-year-old man went to the hospital with a problem that sounded scary. Doctors found a rare tumor growing inside a fluid-filled space deep in his brain. This space is called a ventricle. The tumor was not a common type. It was a slow-growing cancer called a MALT lymphoma. It is very rare in the brain and even rarer inside the ventricles.

This case matters because brain tumors inside the ventricles are often mistaken for other growths. They can look like meningiomas, metastases, or ependymomas on scans. That can delay the right treatment. MALT lymphoma is a type of B-cell lymphoma. It is usually low grade, which means it grows slowly. But location matters. A tumor in the ventricle can affect fluid flow and pressure in the brain.

In the past, doctors did not think of MALT lymphoma when they saw a ventricular lesion. The focus was on more common tumors. This case shows that MALT lymphoma should be on the list when a ventricular mass does not fit the usual pattern. It also suggests that the tumor may start from the pia mater folds of the choroid plexus. That is the thin tissue lining around the brain’s fluid-producing structures.

Think of the ventricles as a set of connected rooms filled with fluid. The fluid cushions the brain and carries nutrients. A tumor in one room can block the doors between rooms. That can cause pressure to build. In this case, the tumor sat in the right lateral ventricle. That is one of the main rooms on the right side of the brain.

The team removed the tumor completely through surgery. After that, the patient received a combination of medicines. One was ifosfamide, a chemotherapy drug. The other was a BTK inhibitor, a targeted medicine that blocks a signal cancer cells use to grow. He also had radiation after surgery. At the latest follow-up, there was no sign the tumor had come back.

This does not mean this treatment is available yet.

The study looked at one patient. That is called a case report. It also reviewed the medical literature. The authors found only eight other cases of ventricular MALT lymphoma. Many of those were first misdiagnosed. That shows how rare and tricky this tumor can be.

Why does this happen in the ventricles. Chronic inflammation is linked to MALT lymphomas in other parts of the body. But in the brain, the triggers are not clear. Some researchers think infections or long-term irritation could play a role. Others suspect the tumor may arise from immune cells that gather near the choroid plexus. The exact cause is still unknown.

The treatment plan here combined three tools. Surgery removed the mass. Chemotherapy with ifosfamide targeted any remaining cells. The BTK inhibitor blocked a key pathway that helps B cells survive. Radiation added extra protection. This layered approach is common for aggressive tumors, but it is notable for a slow-growing lymphoma in this location.

What does this mean for you or a loved one. If a brain lesion is found in a ventricle and does not look typical, ask about the full list of possibilities. MALT lymphoma is rare, but it should be considered. A team that includes neurosurgery, neurology, and oncology can help weigh options. Treatment decisions should be personalized.

There are limits to this report. It is one patient. The follow-up time may be short. The treatment used multiple therapies, so it is hard to know which part mattered most. Larger studies are needed to confirm the best approach.

Looking ahead, more cases and longer follow-up will help. Researchers will study whether surgery alone is enough for some patients. They will also explore whether targeted medicines can reduce the need for radiation. For now, this case adds to the map of what is possible and what to watch for.

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.
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