Exploration of Neuroscience

Table 2. Summary of recently reported cases of isolated PCNSL.

Article	Age (years)	Gender	Immune status	Site	Histology	CSF findings	Imaging	Treatment
Kim et al. [12]	53	Male	Immunocompetent	Thoracic epidural (T7–T9)	DLBCL, CD20⁺	Lymphoma cells present	MRI: epidural mass, T7–T9	Decompressive laminectomy, chemo, RT
Chen et al. [13]	65	Female	Immunocompetent	Leptomeningeal (thoracolumbar)	DLBCL, CD20⁺	WBC‚ Üë, lymphoma cells	MRI: meningeal enhancement T11‚ ÄìL3	High-dose MTX + rituximab
Lobban et al. [14]	67	Female	Immunocompetent	Cranial nerves (trigeminal, facial)	DLBCL, CD20⁺, BCL2⁺, BCL6⁺	Pleocytosis, inconclusive	MRI: cranial nerve enhancement	Steroid, rituximab, MTX, WBRT
Khanna et al. [15]	85	Male	Immunocompetent	Intraventricular	DLBCL, CD20⁺	Negative	MRI: intraventricular masses	Rituximab-MTX ×8 cycles
Zayed et al. [10]	64	Female	Immunosuppressed (post-LT)	Right frontal lobe	DLBCL, CD20⁺	Negative	MRI: frontal lesion, PET negative	R-MP chemo
Schuster Bruce and Grammatopoulou [11]	66	Female	Immunocompetent	Skull base (sphenoid, Meckel‚ Äôs cave)	DLBCL, CD20⁺, BCL6⁺, MIB1 high	Not reported	MRI/PET: skull base, dura	MATRIX regimen
Sheng et al. [16]	63	Female	Immunocompetent	Cranial nerves, neurolymphomatosis	DLBCL, CD20⁺	Pleocytosis, no malignant cells	MRI: cranial nerves	CHOP ×2 cycles
Xue et al. [17]	44	Female	Immunocompetent	Frontal, parietal, periventricular	DLBCL, non-GCB	Negative	MRI: periventricular lesions	CHOP ×2 cycles
Cheng et al. [18]	35–69 (mean 55)	2 female, 6 male	Immunocompetent	Intraventricular system (lateral and fourth ventricles)	DLBCL	Not specified (pathology diagnosis)	Solitary, cluster-like, or diffuse ventricular lesions with hydrocephalus	Surgical resection in all; 3 received high-dose methotrexate-based chemotherapy and radiotherapy; others declined adjuvant therapy

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BCL2: B-cell lymphoma 2; DLBCL: diffuse large B-cell lymphoma; MRI: magnetic resonance imaging; PCNSL: primary central nervous system lymphoma.

Haldorsen IS, Espeland A, Larsson EM. Central nervous system lymphoma: characteristic findings on traditional and advanced imaging. AJNR Am J Neuroradiol. 2011;32:984–92. [DOI] [PubMed] [PMC]

Shah T, Venur VA. Central Nervous System Lymphoma. Semin Neurol. 2023;43:825–32. [DOI] [PubMed]

Hoang-Xuan K, Deckert M, Ferreri AJM, Furtner J, Gallego Perez-Larraya J, Henriksson R, et al. European Association of Neuro-Oncology (EANO) guidelines for treatment of primary central nervous system lymphoma (PCNSL). Neuro Oncol. 2023;25:37–53. [DOI] [PubMed] [PMC]

Lauw MIS, Lucas CG, Ohgami RS, Wen KW. Primary Central Nervous System Lymphomas: A Diagnostic Overview of Key Histomorphologic, Immunophenotypic, and Genetic Features. Diagnostics (Basel). 2020;10:1076. [DOI] [PubMed] [PMC]

van der Sanden GA, Schouten LJ, van Dijck JA, van Andel JP, van der Maazen RW, Coebergh JW; Working Group of Specialists in Neuro-Oncology in the Southern and Eastern Netherlands. Primary central nervous system lymphomas: incidence and survival in the Southern and Eastern Netherlands. Cancer. 2002;94:1548–56. [DOI] [PubMed]

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;105:1414–8. [DOI] [PubMed] [PMC]

Ferreri AJM, Calimeri T, Cwynarski K, Dietrich J, Grommes C, Hoang-Xuan K, et al. Primary central nervous system lymphoma. Nat Rev Dis Primers. 2023;9:29. [DOI] [PubMed] [PMC]

DeAngelis LM. Primary central nervous system lymphoma. J Neurol Neurosurg Psychiatry. 1999;66:699–701. [DOI] [PubMed] [PMC]

de Koning ME, Hof JJ, Jansen C, Doorduijn JK, Bromberg JEC, van der Meulen M. Primary central nervous system lymphoma. J Neurol. 2024;271:2906–13. [DOI] [PubMed]

10.

Zayed A, Abdoh Q, Sarama A, Shubietah A. Isolated primary CNS lymphoma after liver transplantation for autoimmune hepatitis: a case report. BMC Neurol. 2024;24:305. [DOI] [PubMed] [PMC]

11.

Schuster Bruce A, Grammatopoulou V. Primary Skull Base Lymphoma Presenting With Ipsilateral Abducens Nerve Palsy: A Case Report. Cureus. 2024;16:e73108. [DOI] [PubMed] [PMC]

12.

Kim H, Kim D, Kim S. Primary Malignant Lymphoma Presenting as an Isolated Epidural Mass. Diagnostics (Basel). 2022;12:2462. [DOI] [PubMed] [PMC]

13.

Chen X, Huang M, Zhang Z, Jing H, Zou Y, Bu H. Primary meningeal central nervous system lymphoma: A case report and literature review. Medicine (Baltimore). 2022;101:e32567. [DOI] [PubMed] [PMC]

14.

Lobban GB, Luke AJ, Basta P, Laziuk K, Kalra A, Kulhari A. Primary Central Nervous System Lymphoma Presenting as Isolated Multiple Cranial Neuropathies: An Extremely Rare Case. Cureus. 2023;15:e41561. [DOI] [PubMed] [PMC]

15.

Khanna G, Ahlawat S, Garg N, Gupta R, Patir R. A Rare Case of Isolated Intraventricular Primary Central Nervous System Lymphoma in an 85-Year-Old Man. Asian J Neurosurg. 2021;16:623–5. [DOI] [PubMed] [PMC]

16.

Sheng S, Sharma R, Samant R, Yuan J, Nalleballe K, Kapoor N. Isolated primary neurolymphomatosis with cranial multineuritis: a case presentation. Neurol Sci. 2021;42:1223–6. [DOI] [PubMed]

17.

Xue Y, Liu CC, Xiang YY, Liu XH. Primary central nervous system lymphoma initially manifesting as cerebral white matter lesions followed by isolated extracerebral relapse: a case report. Neurol Sci. 2021;42:4825–8. [DOI] [PubMed]

18.

Cheng L, Zhu H, Wang J, Wang G, Ma X, Zhao K, et al. Clinical Features, Diagnosis, and Treatment of Primary Intraventricular Lymphoma: Insights From a Monocentric Case Series. Front Neurol. 2022;13:920505. [DOI] [PubMed] [PMC]

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