Primary CNS Lymphoma

C71WHO Vol. 6 (CNS5, 2021)

Central Nervous System (CNS)

Prognosis

This section is being prepared by our editorial process.

🔬 Histological Types

📚 Latest Research

2026-09-09

Anti-CD94 Monoclonal Antibody Dibotatug-Induced Response in a Refractory Primary CNS T-Cell Lymphoma.

Grommes C, et al

A patient with refractory primary CNS T-cell lymphoma achieved a clinical response following treatment with dibotatug, an anti-CD94 monoclonal antibody, marking a notable outcome in a disease with very limited therapeutic options. Primary CNS T-cell lymphoma is an exceptionally rare and aggressive malignancy that typically fails standard immunochemotherapy regimens, leaving patients with a dire prognosis. The anti-CD94 target is expressed on subsets of T cells and NK cells, and this case report published in JCO Precision Oncology suggests that targeting CD94 with dibotatug may represent a viable salvage strategy for this difficult-to-treat entity. These findings support further investigation of anti-CD94 immunotherapy in refractory T-cell lymphomas affecting the central nervous system.

JCO precision oncology

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2026-09-07

Elevated Expression of Caveolin-1 Reflects a Poor Prognosis in Patients With Primary Central Nervous System Lymphomas.

Sugita Y, et al

A study of 39 patients with primary central nervous system lymphomas (PCNSLs, all CNS diffuse large B-cell lymphomas) found that high Caveolin-1 (Cav-1) expression — defined as an H-score ≥ 20 — is significantly associated with markedly lower patient survival compared to those with no or low H-scores (p < 0.01), establishing Cav-1 overexpression as a poor prognostic marker in this aggressive brain tumor. Using immunostaining and histoscoring (H-score range 0–90, median 20; 12 cases scored 0, 11 cases low, 16 cases high), researchers also showed that patients with high numbers of Cav-1-positive tumor vessels had significantly worse survival than those with low vessel counts (p < 0.05). Cav-1 positivity in tumor cells correlated with the Ki-67 proliferation index and the number of Cav-1-positive vessels, implicating this membrane protein in both tumor cell proliferation and angiogenesis in PCNSL. These findings suggest that Cav-1 could serve as a clinically actionable biomarker to identify PCNSL patients at highest risk of rapid disease progression.

Neuropathology : official journal of the Japanese Society of Neuropathology

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2026-09-03

Primary central nervous system extranodal NK/T-cell lymphoma predominantly composed of anaplastic cells: A case report.

Chen H, et al

Researchers describe what is believed to be the first reported case of a primary central nervous system extranodal NK/T-cell lymphoma (ENKTL) exhibiting a predominantly anaplastic morphology, occurring in a 79-year-old woman who presented with episodic seizures, left-sided limb weakness, altered mental status, and dysarthria lasting more than one month. Imaging revealed a solitary frontal-lobe mass that was surgically resected, and histopathology showed diffuse large anaplastic cells with reniform, horseshoe, and doughnut-like nuclei; immunohistochemistry demonstrated diffuse positivity for CD3, CD30, Granzyme B, and TIA1 alongside negativity for ALK and CD56. The case nearly received a misdiagnosis of ALK-negative anaplastic large cell lymphoma until cytotoxic marker positivity and EBER in-situ hybridisation confirmed ENKTL, underscoring a significant diagnostic pitfall for pathologists evaluating aggressive CNS lymphomas with anaplastic features.

Indian journal of pathology & microbiology

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