Primary CNS Lymphoma: age as a prognostic biomarker

Abstract

Abstract Background Lymphomatous involvement of the CNS incidence has been increasing in recent years. However, the rarity of these diseases results in a scarcity of available data regarding their clinical presentation natural history and prognosis. We aimed to investigate neurological characteristics of uncommon lymphomatous involvements confined to the central nervous system (CNS) and identify key variables that could serve as predictive biomarkers for treatment outcomes. Methods We searched for patients presenting with neurological symptoms and diagnosed with CNS-restricted lymphomatous involvement between 2005 and 2023. Results We identified 44 cases (41 with primary central nervous system lymphoma diagnosis, 93%; 3 intravascular lymphoma 7%). Overall median time from the onset of symptoms onset to diagnosis was 47 days: range (6-573 days) and was not statistically different comparing patients > 60 years old, p = 0.22. The median follow-up time was 1144 days (27-3501 days). Cognitive deterioration was the most common presenting symptom in 19/44 (43%). Brain MRI revealed lobar lesions as the most frequent location of pathology (24/44, 55%). Death was reported in 30 patients (68%) by the end of the study period with a median survival of 666 days; (median range 17-3291 days) and was significantly more common in the group experiencing relapses p = 0.04(CI: 0.99,0.03). Patients with relapses had a 4 times higher chance of death p = 0.04 (95% CI: 0.03–0.99) HR = 4.1 (CI 1.01–16.09). Time to diagnosis was associated with higher mortality, p = 0.04 (95% CI: 0.02–0.97) and survival p = 0.02 (95% CI: 0.005–0.54) as well with Eastern Cooperative Oncology Group at the last follow-up p = 0.006 (95% CI: 0.0012–0.62). Conclusions Patients had significant delays in diagnosis, affecting patient outcomes. Cognitive deterioration and lobar lesions were prominent clinical and radiological features. Mortality was notably higher in older patients, relapses, and a longer time to diagnosis.