# Primary central nervous system mucosa-associated lymphoid tissue lymphoma: a diagnostic challenge

**Authors:** Danial Nasiri, Theoni Maragkou, Andreas Raabe, Anna Katharina Krähenbühl, Franca Wagner

PMC · DOI: 10.1093/bjrcr/uaaf037 · BJR | Case Reports · 2025-07-17

## TL;DR

This paper discusses a rare case where a brain tumor was initially mistaken for meningioma but was later diagnosed as a primary CNS MALT lymphoma, highlighting the importance of accurate diagnosis for proper treatment.

## Contribution

The paper presents a case study emphasizing the diagnostic challenges of distinguishing primary CNS MALT lymphoma from meningioma.

## Key findings

- A 69-year-old woman's tumor was initially suspected to be meningioma but was diagnosed as primary CNS MALT lymphoma.
- The case highlights the importance of recognizing imaging characteristics to avoid misdiagnosis.
- Secondary cutaneous tumor infiltration was observed, delaying adjuvant radiotherapy.

## Abstract

Primary central nervous system (CNS) mucosa-associated lymphoid tissue (MALT) lymphoma is a rare condition frequently mistaken for meningioma. Since these conditions require distinct treatment approaches, recognizing their imaging characteristics is essential for accurate clinical decision-making. A 69-year-old woman presented with headaches and forehead swelling, prompting MRI of the CNS. Suspecting an intracranial meningioma, the tumour board recommended surgical resection. However, histopathological analysis identified the lesion as a primary CNS MALT lymphoma. Follow-up revealed secondary cutaneous tumour infiltration, leading to a delay in adjuvant radiotherapy. Understanding the differential diagnoses of meningioma is critical for neuroradiologists and neurosurgeons to ensure appropriate treatment planning. This case highlights a misdiagnosis of meningioma that was ultimately identified as a primary CNS MALT lymphoma, emphasizing key imaging and clinical characteristics essential for distinguishing between the most important differential diagnoses of primary CNS MALT lymphoma.

## Linked entities

- **Diseases:** MALT lymphoma (MONDO:0007650), meningioma (MONDO:0003057)

## Full-text entities

- **Genes:** CD79A (CD79a molecule) [NCBI Gene 973] {aka IGA, IGAlpha, MB-1, MB1}, FCER2 (Fc epsilon receptor II) [NCBI Gene 2208] {aka BLAST-2, CD23, CD23A, CLEC4J, FCE2, FCErII}, CD5 (CD5 molecule) [NCBI Gene 921] {aka LEU1, T1}, CCND1 (cyclin D1) [NCBI Gene 595] {aka BCL1, D11S287E, PRAD1, U21B31}, TNFRSF8 (TNF receptor superfamily member 8) [NCBI Gene 943] {aka CD30, D1S166E, Ki-1}, KRT20 (keratin 20) [NCBI Gene 54474] {aka CD20, CK-20, CK20, K20, KRT21}, MME (membrane metalloendopeptidase) [NCBI Gene 4311] {aka CALLA, CD10, CMT2T, NEP, SCA43, SFE}, PWWP3A (PWWP domain containing 3A, DNA repair factor) [NCBI Gene 84939] {aka EXPAND1, HSPC211, MUM-1, MUM1}, BCL2 (BCL2 apoptosis regulator) [NCBI Gene 596] {aka Bcl-2, PPP1R50}, BCL6 (BCL6 transcription repressor) [NCBI Gene 604] {aka BCL5, BCL6A, LAZ3, ZBTB27, ZNF51}, CD19 (CD19 molecule) [NCBI Gene 930] {aka B4, CVID3}
- **Diseases:** inflammatory disease (MESH:D007249), cancer (MESH:D009369), intracranial tumours (MESH:D001932), IgG4 (MESH:D000077733), lymphoma (MESH:D008223), neurological deficits (MESH:D009461), edema (MESH:D004487), meningiomatous lesions (MESH:D009059), chloroma (MESH:D023981), MALT lymphoma (MESH:D018442), non-Hodgkin's lymphoma (MESH:D008228), dural metastases (MESH:D009362), forehead swelling (MESH:D006259), hypertrophic pachymeningitis (MESH:D014390), neurosarcoidosis (MESH:C535814), bone defect (MESH:D001847), intracranial meningioma (MESH:D008579), Erdheim-Chester disease (MESH:D031249), systemic disorders (MESH:D009422), haemorrhage (MESH:D006470), hygroma (MESH:D018191), dural lesions (MESH:D020785), extraaxial epidural tumour lesion (MESH:D015174), headaches (MESH:D006261)
- **Chemicals:** Rituximab (MESH:D000069283), fludeoxyglucose-18 (-)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

5 figures with captions in the complete paper: https://tomesphere.com/paper/PMC12308279/full.md

## References

15 references — full list in the complete paper: https://tomesphere.com/paper/PMC12308279/full.md

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Source: https://tomesphere.com/paper/PMC12308279