Primary diffuse large B-cell lymphoma of the CNS, with a “Lymphomatosis cerebri” pattern

Authors

DOI:

https://doi.org/10.4322/acr.2021.250

Keywords:

Central Nervous System Neoplasm, Lymphomatosis cerebri, Non-Hodgkin lymphoma, Dementia

Abstract

We describe an unusual case of lymphomatosis cerebri in a middle-aged lady presenting with rapid-onset dementia. The lymphomatous infiltrate, instead of forming mass lesions, percolated throughout the brain parenchyma, which is often missed on a stereotactic biopsy and hence warrants caution and awareness about this entity. The nonspecific symptoms at presentation and a variable picture at imaging make this entity diagnostically challenging.

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References

Ostrom QT, Gittleman H, Fulop J, Liu M, Blanda R, Kromer C, Wolinsky Y, Kruchko C, Barnholtz-Sloan JS. CBTRUS Statistical Report: Primary Brain and Central Nervous System Tumors Diagnosed in the United States in 2008-2012. Neuro Oncol. 2015 Oct;17(4 Suppl):iv1-iv62.

Ferreri AJ, Marturano E. Primary CNS lymphoma. Best Pract Res Clin Haematol. 2012;25(1):119-30. http://dx.doi.org/10.1016/j.beha.2011.12.001. PMid:22409828.

Chihara D, Fowler NH, Oki Y, et al. Impact of histologic subtypes and treatment modality among patients with primary central nervous system lymphoma: a SEER database analysis. Oncotarget. 2018;9(48):28897-902. http://dx.doi.org/10.18632/oncotarget.25622. PMid:29988979.

Bakshi R, Mazziotta JC, Mischel PS, Jahan R, Seligson DB, Vinters HV. Lymphomatosis cerebri presenting as a rapidly progressive dementia: Clinical, neuroimaging and pathologic findings. Dement Geriatr Cogn Disord. 1999;10(2):152-7. http://dx.doi.org/10.1159/000017116. PMid:10026390.

Gupta K, Gupta V, Radotra BD, Tewari MK. “Slow and Steady” infiltrates the brain: an autopsy report of Lymphomatosis Cerebri. Neurol India. 2019;67(6):1504-8. http://dx.doi.org/10.4103/0028-3886.273648. PMid:31857546.

Sugie M, Ishihara K, Kato H, Nakano I, Kawamura M. Primary central nervous system lymphoma initially mimicking lymphomatosis cerebri: an autopsy case report. Neuropathology. 2009;29(6):704-7. http://dx.doi.org/10.1111/j.1440-1789.2009.01004.x. PMid:19389079.

Carlson BA. Rapidly progressive dementia caused by nonenhancing primary lymphoma of the central nervous system. AJNR Am J Neuroradiol. 1996;17(9):1695-7. PMid:8896625.

de Toledo M, López-Valdés E, Ferreiro M, et al. Linfomatosis cerebral como causa de leucoencefalopatía. Rev Neurol. 2008;46(11):667-70. http://dx.doi.org/10.33588/rn.4611.2007660. PMid:18509825.

Keswani A, Bigio E, Grimm S. Lymphomatosis cerebri presenting with orthostatic hypotension, anorexia, and paraparesis. J Neurooncol. 2012;109(3):581-6. http://dx.doi.org/10.1007/s11060-012-0931-3. PMid:22806340.

Kitai R, Hashimoto N, Yamate K, et al. Lymphomatosis cerebri: clinical characteristics, neuroimaging, and pathological findings. Brain Tumor Pathol. 2012;29(1):47-53. http://dx.doi.org/10.1007/s10014-011-0064-y. PMid:21927864.

Leschziner G, Rudge P, Lucas S, Andrews T. Lymphomatosis cerebri presenting as a rapidly progressive dementia with a high methylmalonic acid. J Neurol. 2011;258(8):1489-93. http://dx.doi.org/10.1007/s00415-011-5965-5. PMid:21365456.

Kim EY, Kim SS. Magnetic resonance findings of primary central nervous system T-cell lymphoma in immunocompetent patients. Acta Radiol. 2005;46(2):187-92. http://dx.doi.org/10.1080/02841850510022714. PMid:15902895.

Villano JL, Koshy M, Shaikh H, Dolecek TA, McCarthy BJ. Age, gender, and racial differences in incidence and survival in primary CNS lymphoma. Br J Cancer. 2011;105(9):1414-8. http://dx.doi.org/10.1038/bjc.2011.357. PMid:21915121.

Haldorsen IS, Espeland A, Larsson EM. Central nervous system lymphoma: characteristic findings on traditional and advanced imaging. AJNR Am J Neuroradiol. 2011;32(6):984-92. http://dx.doi.org/10.3174/ajnr.A2171. PMid:20616176.

Izquierdo C, Velasco R, Vidal N, et al. Lymphomatosis cerebri: A rare form of primary central nervous system lymphoma. Analysis of 7 cases and systematic review of the literature. Neuro-oncol. 2016;18(5):707-15. http://dx.doi.org/10.1093/neuonc/nov197. PMid:26415875.

Yamamoto A, Kikuchi Y, Homma K, O’uchi T, Furui S. Characteristics of Intravascular Large B-Cell Lymphoma on Cerebral MR Imaging. AJNR Am J Neuroradiol. 2012;33(2):292-6. http://dx.doi.org/10.3174/ajnr.A2770. PMid:22173763.

Paulus W, Jellinger K. Comparison of integrin adhesion molecules expressed by primary brain lymphomas and nodal lymphomas. Acta Neuropathol. 1993;86(4):360-4. http://dx.doi.org/10.1007/BF00369448. PMid:8256586.

Sugita Y, Muta H, Ohshima K, et al. Primary central nervous system lymphomas and related diseases: pathological characteristics and discussion of the differential diagnosis. Neuropathology. 2016;36(4):313-24. http://dx.doi.org/10.1111/neup.12276. PMid:26607855.

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Published

2021-04-20

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Section

Article / Autopsy Case Report

How to Cite

Thirunavukkarasu, B., Gupta, K., Shree, R., Prabhakar, A., Takkar Kapila, A., Lal, V., & Radotra, B. (2021). Primary diffuse large B-cell lymphoma of the CNS, with a “Lymphomatosis cerebri” pattern. Autopsy and Case Reports, 11, e2021250. https://doi.org/10.4322/acr.2021.250