Primary Orbital Marginal Zone B-cell Lymphoma: A Rare Case Report

Orbital Lymphomas (OL) account for 1 to 2% of Non Hodgkin Lymphomas (NHLs) and 2 to 11% of all orbital tumours. The most common primary OL is low-grade marginal zone B-cell lymphoma. This is a case of primary orbital NHL mimicking an orbital pseudotumour. A 67-year-old male presented with a swolle...

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Main Authors: Ranjana Giri, Nageswar Sahu, Prita Pradhan, Dhanasree Surendran Vellikal, Goutami Das Nayak
Format: Article
Language:English
Published: JCDR Research and Publications Private Limited 2025-02-01
Series:Journal of Clinical and Diagnostic Research
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Online Access:https://jcdr.net/articles/PDF/20594/74703_CE[Ra1]_F(SHU)_QC(PS_SS)_PF1(AG_SS)_PFA(IS)_PB(AG_IS)_PN(IS).pdf
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author Ranjana Giri
Nageswar Sahu
Prita Pradhan
Dhanasree Surendran Vellikal
Goutami Das Nayak
author_facet Ranjana Giri
Nageswar Sahu
Prita Pradhan
Dhanasree Surendran Vellikal
Goutami Das Nayak
author_sort Ranjana Giri
collection DOAJ
description Orbital Lymphomas (OL) account for 1 to 2% of Non Hodgkin Lymphomas (NHLs) and 2 to 11% of all orbital tumours. The most common primary OL is low-grade marginal zone B-cell lymphoma. This is a case of primary orbital NHL mimicking an orbital pseudotumour. A 67-year-old male presented with a swollen right eye associated with lacrimation for two months. On examination, there was right eye proptosis. Thyroid, liver and renal function tests were within normal limits. Contrast Enhanced Computed Tomography (CECT) revealed a homogenously enhancing intraconal mass measuring 4.1×3.2×2.8 cm, with features favouring a benign aetiology. Cytosmears revealed features suggestive of inflammatory pseudotumour of the orbit. Histopathological findings suggested low-grade NHL. Immunohistochemistry (IHC) showed positivity for CD20, CD79a, PAX5, and Bcl2, and negativity for CD3, CD5, CD10, CD23, Bcl6, and Cyclin-D1. Hence, a final diagnosis of marginal zone B-cell lymphoma was rendered. Detailed clinical and radiological evaluations did not reveal lymphadenopathy anywhere else or any other focus of tumour, indicating the primary origin of this tumour in the orbit. Primary NHL of the orbit is rare. It can occur in the orbit without any systemic features. It must be diagnosed early and can be treated with surgery and chemotherapy.
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spelling doaj-art-bfa1c1b71db8422dbc29af2431e9502c2025-08-20T02:08:57ZengJCDR Research and Publications Private LimitedJournal of Clinical and Diagnostic Research2249-782X0973-709X2025-02-01040610.7860/JCDR/2025/74703.20594Primary Orbital Marginal Zone B-cell Lymphoma: A Rare Case ReportRanjana Giri0 Nageswar Sahu1Prita Pradhan2Dhanasree Surendran Vellikal3Goutami Das Nayak4Professor and Head, Department of Pathology, Kalinga Institute of Medical Sciences, KIIT University, Bhubaneswar, Odisha, India.Professor, Department of Pathology, Kalinga Institute of Medical Sciences, KIIT University, Bhubaneswar, Odisha, India.Associate Professor, Department of Pathology, Kalinga Institute of Medical Sciences, KIIT University, Bhubaneswar, Odisha, India.Postgraduate Resident, Department of Pathology, Kalinga Institute of Medical Sciences, KIIT University, Bhubaneswar, Odisha, India.Assistant Professor, Department of Pathology, SCB Medical College, Cuttack, Odisha, India.Orbital Lymphomas (OL) account for 1 to 2% of Non Hodgkin Lymphomas (NHLs) and 2 to 11% of all orbital tumours. The most common primary OL is low-grade marginal zone B-cell lymphoma. This is a case of primary orbital NHL mimicking an orbital pseudotumour. A 67-year-old male presented with a swollen right eye associated with lacrimation for two months. On examination, there was right eye proptosis. Thyroid, liver and renal function tests were within normal limits. Contrast Enhanced Computed Tomography (CECT) revealed a homogenously enhancing intraconal mass measuring 4.1×3.2×2.8 cm, with features favouring a benign aetiology. Cytosmears revealed features suggestive of inflammatory pseudotumour of the orbit. Histopathological findings suggested low-grade NHL. Immunohistochemistry (IHC) showed positivity for CD20, CD79a, PAX5, and Bcl2, and negativity for CD3, CD5, CD10, CD23, Bcl6, and Cyclin-D1. Hence, a final diagnosis of marginal zone B-cell lymphoma was rendered. Detailed clinical and radiological evaluations did not reveal lymphadenopathy anywhere else or any other focus of tumour, indicating the primary origin of this tumour in the orbit. Primary NHL of the orbit is rare. It can occur in the orbit without any systemic features. It must be diagnosed early and can be treated with surgery and chemotherapy.https://jcdr.net/articles/PDF/20594/74703_CE[Ra1]_F(SHU)_QC(PS_SS)_PF1(AG_SS)_PFA(IS)_PB(AG_IS)_PN(IS).pdfimmunohistochemistrynon hodgkin lymphomaspseudotumour
spellingShingle Ranjana Giri
Nageswar Sahu
Prita Pradhan
Dhanasree Surendran Vellikal
Goutami Das Nayak
Primary Orbital Marginal Zone B-cell Lymphoma: A Rare Case Report
Journal of Clinical and Diagnostic Research
immunohistochemistry
non hodgkin lymphomas
pseudotumour
title Primary Orbital Marginal Zone B-cell Lymphoma: A Rare Case Report
title_full Primary Orbital Marginal Zone B-cell Lymphoma: A Rare Case Report
title_fullStr Primary Orbital Marginal Zone B-cell Lymphoma: A Rare Case Report
title_full_unstemmed Primary Orbital Marginal Zone B-cell Lymphoma: A Rare Case Report
title_short Primary Orbital Marginal Zone B-cell Lymphoma: A Rare Case Report
title_sort primary orbital marginal zone b cell lymphoma a rare case report
topic immunohistochemistry
non hodgkin lymphomas
pseudotumour
url https://jcdr.net/articles/PDF/20594/74703_CE[Ra1]_F(SHU)_QC(PS_SS)_PF1(AG_SS)_PFA(IS)_PB(AG_IS)_PN(IS).pdf
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AT dhanasreesurendranvellikal primaryorbitalmarginalzonebcelllymphomaararecasereport
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