Orbital Neurolymphomatosis in Patient with CNS Lymphoma

Neurolymphomatosis (NL) is a rare manifestation of hematologic malignancies, characterized by a neoplastic infiltration of the peripheral nervous system and cranial nerves (CNs). Non-Hodgkin lymphomas (NHLs) account for 90% of NL cases, while acute leukemia represents 10% of the cases. NL can occur...

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Main Authors: Tara Shooshani, Michael Han, Jeremiah P. Tao, Samuel J. Spiegel, Maria Del Valle Estopinal
Format: Article
Language:English
Published: MDPI AG 2025-03-01
Series:Diagnostics
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Online Access:https://www.mdpi.com/2075-4418/15/6/780
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author Tara Shooshani
Michael Han
Jeremiah P. Tao
Samuel J. Spiegel
Maria Del Valle Estopinal
author_facet Tara Shooshani
Michael Han
Jeremiah P. Tao
Samuel J. Spiegel
Maria Del Valle Estopinal
author_sort Tara Shooshani
collection DOAJ
description Neurolymphomatosis (NL) is a rare manifestation of hematologic malignancies, characterized by a neoplastic infiltration of the peripheral nervous system and cranial nerves (CNs). Non-Hodgkin lymphomas (NHLs) account for 90% of NL cases, while acute leukemia represents 10% of the cases. NL can occur as the first manifestation of a malignancy (primary), or as a relapse or progression of a previously treated disease (secondary). Herein, we report a unique case of NL involving the left orbit and CNs in a 74-year-old female with primary central nervous system (CNS) diffuse large B-cell lymphoma (DLBCL). Our patient developed secondary neurolymphomatosis involving the left orbit and CNs II, III, V, and VI, supported by clinical, radiologic, and histologic findings. The lacrimal gland enhancement was histopathologically proven to be caused by the direct spread of CNS DLBCL to the lacrimal nerve, a branch of CN V, identifying NL as one of the conditions that can affect this organ. The lacrimal gland could be considered as a more accessible biopsy site when the involvement of CN V is suspected.
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spelling doaj-art-5b2ae63b931d486d923971097b37f2f12025-08-20T03:43:37ZengMDPI AGDiagnostics2075-44182025-03-0115678010.3390/diagnostics15060780Orbital Neurolymphomatosis in Patient with CNS LymphomaTara Shooshani0Michael Han1Jeremiah P. Tao2Samuel J. Spiegel3Maria Del Valle Estopinal4School of Medicine, University of California, Irvine, CA 92697, USADepartment of Ophthalmology, University of California, Irvine, CA 92697, USADepartment of Ophthalmology, University of California, Irvine, CA 92697, USADepartment of Ophthalmology, University of California, Irvine, CA 92697, USADepartment of Ophthalmology, University of California, Irvine, CA 92697, USANeurolymphomatosis (NL) is a rare manifestation of hematologic malignancies, characterized by a neoplastic infiltration of the peripheral nervous system and cranial nerves (CNs). Non-Hodgkin lymphomas (NHLs) account for 90% of NL cases, while acute leukemia represents 10% of the cases. NL can occur as the first manifestation of a malignancy (primary), or as a relapse or progression of a previously treated disease (secondary). Herein, we report a unique case of NL involving the left orbit and CNs in a 74-year-old female with primary central nervous system (CNS) diffuse large B-cell lymphoma (DLBCL). Our patient developed secondary neurolymphomatosis involving the left orbit and CNs II, III, V, and VI, supported by clinical, radiologic, and histologic findings. The lacrimal gland enhancement was histopathologically proven to be caused by the direct spread of CNS DLBCL to the lacrimal nerve, a branch of CN V, identifying NL as one of the conditions that can affect this organ. The lacrimal gland could be considered as a more accessible biopsy site when the involvement of CN V is suspected.https://www.mdpi.com/2075-4418/15/6/780neurolymphomatosisCNS lymphomahistopathology
spellingShingle Tara Shooshani
Michael Han
Jeremiah P. Tao
Samuel J. Spiegel
Maria Del Valle Estopinal
Orbital Neurolymphomatosis in Patient with CNS Lymphoma
Diagnostics
neurolymphomatosis
CNS lymphoma
histopathology
title Orbital Neurolymphomatosis in Patient with CNS Lymphoma
title_full Orbital Neurolymphomatosis in Patient with CNS Lymphoma
title_fullStr Orbital Neurolymphomatosis in Patient with CNS Lymphoma
title_full_unstemmed Orbital Neurolymphomatosis in Patient with CNS Lymphoma
title_short Orbital Neurolymphomatosis in Patient with CNS Lymphoma
title_sort orbital neurolymphomatosis in patient with cns lymphoma
topic neurolymphomatosis
CNS lymphoma
histopathology
url https://www.mdpi.com/2075-4418/15/6/780
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AT michaelhan orbitalneurolymphomatosisinpatientwithcnslymphoma
AT jeremiahptao orbitalneurolymphomatosisinpatientwithcnslymphoma
AT samueljspiegel orbitalneurolymphomatosisinpatientwithcnslymphoma
AT mariadelvalleestopinal orbitalneurolymphomatosisinpatientwithcnslymphoma