Case Report: Diffuse cerebral lymphomatosis with superimposed multifocal primary CNS lymphoma
DescriptionCerebral lymphomatosis (CL) is a rare subtype of primary central nervous system lymphoma (PCNSL). In CL, atypical lymphoid cells diffusely infiltrate the cerebral parenchyma without forming a discrete mass as seen with PCNSL. We report a case of a 66-year-old woman with diffuse CL and sup...
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Frontiers Media S.A.
2024-11-01
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| Series: | Frontiers in Radiology |
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| Online Access: | https://www.frontiersin.org/articles/10.3389/fradi.2024.1479282/full |
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| author | Elizabeth Huai-Feng Li Claire Davila Connor Zuraski Jennifer Chang Vanessa Goodwill Nikdokht Farid |
| author_facet | Elizabeth Huai-Feng Li Claire Davila Connor Zuraski Jennifer Chang Vanessa Goodwill Nikdokht Farid |
| author_sort | Elizabeth Huai-Feng Li |
| collection | DOAJ |
| description | DescriptionCerebral lymphomatosis (CL) is a rare subtype of primary central nervous system lymphoma (PCNSL). In CL, atypical lymphoid cells diffusely infiltrate the cerebral parenchyma without forming a discrete mass as seen with PCNSL. We report a case of a 66-year-old woman with diffuse CL and superimposed areas of PCNSL. She presented with subacute cognitive decline and weakness. CSF studies showed lymphocytosis and IL-10 elevation. She became increasingly somnolent despite steroid and intravenous immunoglobulin trials, and she succumbed to the disease four months after symptom onset.Radiologic findingsHer initial non-contrast head CT showed ill-defined hypodensities in the periventricular and subcortical white matter, bilateral basal ganglia, and central pons, which corresponded to diffuse T2/FLAIR hyperintensities on brain MRI. No abnormal enhancement, diffusion restriction, or discrete mass was present initially. Subsequently, MR spectroscopy demonstrated abnormally elevated choline:creatine and decreased NAA peaks, suggesting a hypercellular process. One month later, MRI revealed increasingly confluent T2/FLAIR hyperintensities with new diffusion restriction in the right caudate and left hippocampus, as well as new hyperperfusion in the right caudate. Again, no mass or enhancement was identified in these areas. On autopsy, parenchymal pathology was mostly consistent with CL. However, there were two areas of frank PCNSL in the right caudate and left hippocampus, which corresponded to the new areas of abnormality on her last MRI despite lacking the typical radiologic features of PCNSL.Novel aspectsThis is a unique case of CL with concurrent areas of PCNSL. Although CL is thought to be a distinct subtype of PCNSL, our case demonstrates that PCNSL may develop on a background of diffuse CL. In patients with subacute neurologic decline and MRI findings of diffuse leukoencephalopathy, diffuse CL should be considered. |
| format | Article |
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| institution | OA Journals |
| issn | 2673-8740 |
| language | English |
| publishDate | 2024-11-01 |
| publisher | Frontiers Media S.A. |
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| series | Frontiers in Radiology |
| spelling | doaj-art-ad069f4ae63340468deb9e2dd8df3b6a2025-08-20T02:22:36ZengFrontiers Media S.A.Frontiers in Radiology2673-87402024-11-01410.3389/fradi.2024.14792821479282Case Report: Diffuse cerebral lymphomatosis with superimposed multifocal primary CNS lymphomaElizabeth Huai-Feng Li0Claire Davila1Connor Zuraski2Jennifer Chang3Vanessa Goodwill4Nikdokht Farid5School of Medicine, UC San Diego, San Diego, CA, United StatesDepartment of Neurology, UC San Diego, San Diego, CA, United StatesDepartment of Pathology, UC San Diego, San Diego, CA, United StatesDepartment of Radiology, UC San Diego, San Diego, CA, United StatesDepartment of Pathology, UC San Diego, San Diego, CA, United StatesDepartment of Radiology, UC San Diego, San Diego, CA, United StatesDescriptionCerebral lymphomatosis (CL) is a rare subtype of primary central nervous system lymphoma (PCNSL). In CL, atypical lymphoid cells diffusely infiltrate the cerebral parenchyma without forming a discrete mass as seen with PCNSL. We report a case of a 66-year-old woman with diffuse CL and superimposed areas of PCNSL. She presented with subacute cognitive decline and weakness. CSF studies showed lymphocytosis and IL-10 elevation. She became increasingly somnolent despite steroid and intravenous immunoglobulin trials, and she succumbed to the disease four months after symptom onset.Radiologic findingsHer initial non-contrast head CT showed ill-defined hypodensities in the periventricular and subcortical white matter, bilateral basal ganglia, and central pons, which corresponded to diffuse T2/FLAIR hyperintensities on brain MRI. No abnormal enhancement, diffusion restriction, or discrete mass was present initially. Subsequently, MR spectroscopy demonstrated abnormally elevated choline:creatine and decreased NAA peaks, suggesting a hypercellular process. One month later, MRI revealed increasingly confluent T2/FLAIR hyperintensities with new diffusion restriction in the right caudate and left hippocampus, as well as new hyperperfusion in the right caudate. Again, no mass or enhancement was identified in these areas. On autopsy, parenchymal pathology was mostly consistent with CL. However, there were two areas of frank PCNSL in the right caudate and left hippocampus, which corresponded to the new areas of abnormality on her last MRI despite lacking the typical radiologic features of PCNSL.Novel aspectsThis is a unique case of CL with concurrent areas of PCNSL. Although CL is thought to be a distinct subtype of PCNSL, our case demonstrates that PCNSL may develop on a background of diffuse CL. In patients with subacute neurologic decline and MRI findings of diffuse leukoencephalopathy, diffuse CL should be considered.https://www.frontiersin.org/articles/10.3389/fradi.2024.1479282/fullcerebral lymphomatosislymphomatosis cerebriprimary CNS lymphoma (PCNSL)diffuse leukoencephalopathydiffuse tumorous infiltrationmagnetic resonace imaging (MRI) |
| spellingShingle | Elizabeth Huai-Feng Li Claire Davila Connor Zuraski Jennifer Chang Vanessa Goodwill Nikdokht Farid Case Report: Diffuse cerebral lymphomatosis with superimposed multifocal primary CNS lymphoma Frontiers in Radiology cerebral lymphomatosis lymphomatosis cerebri primary CNS lymphoma (PCNSL) diffuse leukoencephalopathy diffuse tumorous infiltration magnetic resonace imaging (MRI) |
| title | Case Report: Diffuse cerebral lymphomatosis with superimposed multifocal primary CNS lymphoma |
| title_full | Case Report: Diffuse cerebral lymphomatosis with superimposed multifocal primary CNS lymphoma |
| title_fullStr | Case Report: Diffuse cerebral lymphomatosis with superimposed multifocal primary CNS lymphoma |
| title_full_unstemmed | Case Report: Diffuse cerebral lymphomatosis with superimposed multifocal primary CNS lymphoma |
| title_short | Case Report: Diffuse cerebral lymphomatosis with superimposed multifocal primary CNS lymphoma |
| title_sort | case report diffuse cerebral lymphomatosis with superimposed multifocal primary cns lymphoma |
| topic | cerebral lymphomatosis lymphomatosis cerebri primary CNS lymphoma (PCNSL) diffuse leukoencephalopathy diffuse tumorous infiltration magnetic resonace imaging (MRI) |
| url | https://www.frontiersin.org/articles/10.3389/fradi.2024.1479282/full |
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