# Diagnostic Challenge of Extranodal Marginal Zone Lymphoma of Extraocular Muscles: A Case Report

**Authors:** Vaia-Aikaterini Alexoudi, Dimitris Tatsis, Christos Varelas, Konstantinos Vaxtsevanos, Aggeliki Cheva

PMC · DOI: 10.3390/reports9010055 · Reports - Clinical Practice and Surgical Cases · 2026-02-09

## TL;DR

This case report describes a rare lymphoma affecting extraocular muscles and emphasizes the importance of early diagnosis and advanced imaging techniques.

## Contribution

The paper presents a unique case of EMZL in extraocular muscles and highlights the role of navigation systems in accurate diagnosis.

## Key findings

- Extranodal marginal zone lymphoma can present atypically in extraocular muscles.
- Advanced visualization techniques aid in obtaining adequate biopsy samples from delicate orbital tissue.
- Early clinical suspicion and multidisciplinary teamwork improve diagnostic accuracy and patient outcomes.

## Abstract

Background and clinical significance: The current report examines an unusual case of extranodal marginal zone lymphoma (EMZL) affecting only the extraocular muscles. The diagnostic challenges connected to such atypical manifestations are highlighted. The resemblance to inflammatory or infiltrative processes may lead to diagnostic delays; therefore, therapy administration may be withheld. Case presentation: A 77-year-old male was admitted to our hospital with periorbital edema accompanied with vision abnormalities affecting his left eye. The complete diagnostic workup and surgical treatment is presented using a navigation system. Adequate biopsy samples from the delicate orbital tissue can be obtained by utilizing contemporary visualization techniques, particularly navigation systems. The pathology report proved indispensable, with ample raw muscle tissue providing sufficient material from whicha definitive diagnosis was made. The indicated therapy was administered without delay. Conclusions: The uncommon, isolated affection of extraocular muscles by extranodal marginal zone lymphoma is exemplified in this case, highlighting the need for early clinical suspicion in order to differentiate this condition from other more prevalent inflammatory pathologies. The implementation of advanced technologies, such as image-guided navigation systems, alongside a highly skilled multidisciplinary medical team, ensures optimal patient results.

## Full-text entities

- **Genes:** CRP (C-reactive protein) [NCBI Gene 1401] {aka PTX1}, MME (membrane metalloendopeptidase) [NCBI Gene 4311] {aka CALLA, CD10, CMT2T, NEP, SCA43, SFE}, BCL2 (BCL2 apoptosis regulator) [NCBI Gene 596] {aka Bcl-2, PPP1R50}, BCL6 (BCL6 transcription repressor) [NCBI Gene 604] {aka BCL5, BCL6A, LAZ3, ZBTB27, ZNF51}, BTK (Bruton tyrosine kinase) [NCBI Gene 695] {aka AGMX1, AT, ATK, BPK, IGHD3, IMD1}, FCER2 (Fc epsilon receptor II) [NCBI Gene 2208] {aka BLAST-2, CD23, CD23A, CLEC4J, FCE2, FCErII}, CCND1 (cyclin D1) [NCBI Gene 595] {aka BCL1, D11S287E, PRAD1, U21B31}, CD5 (CD5 molecule) [NCBI Gene 921] {aka LEU1, T1}, KRT20 (keratin 20) [NCBI Gene 54474] {aka CD20, CK-20, CK20, K20, KRT21}, LEF1 (lymphoid enhancer binding factor 1) [NCBI Gene 51176] {aka ECTD1, ECTD17, LEF-1, TCF10, TCF1ALPHA, TCF7L3}, ANXA1 (annexin A1) [NCBI Gene 301] {aka ANX1, LPC1}, CR2 (complement C3d receptor 2) [NCBI Gene 1380] {aka C3DR, CD21, CR, CVID7, SLEB9}
- **Diseases:** orbital myositis (MESH:D055622), follicular lymphoma (MESH:D008224), eye movement impairment (MESH:D015835), hypertension (MESH:D006973), diplopia (MESH:D004172), sarcoidosis (MESH:D012507), infections (MESH:D007239), Extranodal marginal zone lymphoma (MESH:D018442), toxicity (MESH:D064420), B-cell lymphoma (MESH:D016393), amyloidosis (MESH:D000686), cataract (MESH:D002386), lymphoma (MESH:D008223), bacterial infections (MESH:D001424), non-Hodgkin lymphoma (MESH:D008228), orbital (MESH:D009916), mantle cell lymphoma (MESH:D020522), ocular disorders (MESH:D005128), gastritis (MESH:D005756), diffuse large B-cell lymphoma (MESH:D016403), EOMLs (MESH:C580012), chronic (MESH:D002908), tumor infiltrative disease (MESH:D010524), Chlamydia psittaci infection (MESH:D002690), hyperlipidemia (MESH:D006949), injury to (MESH:D014947), mydriasis (MESH:D015878), chronic inflammation (MESH:D007249), chronic lymphocytic leukemia (MESH:D015451), afferent pupillary defect (MESH:D011681), vision abnormalities (MESH:D014786), malignancy (MESH:D009369), thyroid eye disease (MESH:D049970), Periorbital edema (MESH:D004487), inflammatory pseudotumor (MESH:D006104), conjunctival hyperemia (MESH:D003229), hairy cell leukemia (MESH:D007943), myositis (MESH:D009220), fever (MESH:D005334), IgG4-related disease (MESH:D000077733), proptosis (MESH:D005094), systemic disease (MESH:D034721), Orbital lymphomas (MESH:C537131)
- **Chemicals:** Hematoxylin-eosin (-), rituximab (MESH:D000069283), Congo red (MESH:D003224), bendamustine (MESH:D000069461), formalin (MESH:D005557), doxycycline (MESH:D004318), paraffin (MESH:D010232), chlorambucil (MESH:D002699)
- **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/PMC12921922/full.md

## References

18 references — full list in the complete paper: https://tomesphere.com/paper/PMC12921922/full.md

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Source: https://tomesphere.com/paper/PMC12921922