Yerleşim yeri biyolojiyle buluştuğunda: primer santral sinir sistemi lenfomada kombine risk paternleri sağkalımı belirler

Öz

Amaç: Primer santral sinir sistemi lenfoması (PSSSL), nadir görülen ve agresif seyirli bir ekstranodal lenfoma alt tipidir. Özellikle immün sistemi sağlam hastalarda yapılan gerçek yaşam verileri sınırlıdır. Prognostik faktörler net olarak tanımlanmamıştır ve küçük retrospektif serilerde sonuçlar çelişkilidir. Gereç ve Yöntemler: Bu tek merkezli retrospektif çalışmaya, Haziran 2002 ile Mart 2022 tarihleri arasında PSSSL tanısı almış 19 hasta dahil edildi. Klinik, radyolojik, patolojik ve tedaviyle ilişkili veriler değerlendirildi. Genel sağkalım (OS) analiz edildi. Prognostik faktörleri belirlemek amacıyla univaryant analiz yapıldı. Bulgular: Hastaların medyan yaşı 49,1 olup %57,9’u erkekti. En sık başvuru semptomu fokal nörolojik defisitti. Derin beyin yerleşimi, parietal lob tutulumu, bilateral veya multifokal lezyonlar ve germinal merkez B hücreli (GCB) alt tip varlığı ile sağkalımın belirgin şekilde kötü olduğu gözlendi. GCB alt tipli tüm hastalar relaps gösterip yaşamını yitirirken, non-GCB hastaların çoğu hastalıksız olarak yaşamını sürdürmekteydi. Memorial Sloan Kettering Kanser Merkezi (MSKCC) prognostik skoru, risk gruplarını başarılı şekilde ayırt etti. Medyan genel sağkalım 13,6 aydı. Prognostik faktörlerin aynı hastalarda kümelenmesi nedeniyle çok değişkenli analiz yapılmadı. Sonuç: Bu çalışma, PSSSL’de anatomik ve moleküler yüksek risk özelliklerinin sağkalım üzerindeki belirgin etkisini vurgulamakta ve az temsil edilen bir popülasyondan gerçek yaşam verileri sunarak literatüre katkı sağlamaktadır. Bulgular, bu nadir malignitede bireyselleştirilmiş tedavi yaklaşımlarının geliştirilmesine rehberlik edebilir.

Anahtar Kelimeler

• primer santral sinir sistemi lenfoması
• prognostik faktörler
• sağkalım analizi

When location meets biology: combined risk patterns drive outcomes in primary central nervous system lymphoma

Öz

Aim: Primary central nervous system lymphoma (PCNSL) is a rare and aggressive extranodal lymphoma. Despite therapeutic advances, data from immunocompetent patients in real-world cohorts remain limited, and prognostic factors are still not clearly defined- especially in small retrospective series. Material and Methods: We retrospectively analyzed 19 immunocompetent patients diagnosed with PCNSL between June 2002 and March 2022 at a single tertiary oncology center. Clinical, radiological, pathological, and treatment-related parameters were collected. Overall survival (OS) was assessed, and univariate analysis was conducted to explore potential prognostic factors. Results: The median age was 49.1 years, and 57.9% were male. Focal neurological deficits were the most common presenting symptom. Poor outcomes were observed in patients with deep brain involvement, parietal lobe lesions, bilateral or multifocal disease, and germinal center B-cell (GCB) subtype. All GCB patients experienced relapse and died, while most non-GCB patients remained alive and disease-free. The Memorial Sloan Kettering Cancer Center (MSKCC) score effectively stratified risk groups. Median OS was 13.6 months. Multivariate analysis was not feasible due to overlapping high-risk features, limiting statistical independence. Conclusion: This study emphasizes the prognostic impact of combined anatomical and molecular risk factors in PCNSL and contributes real-world data from an underrepresented population. These findings may support the development of risk-adapted therapeutic strategies for this rare malignancy.

Anahtar Kelimeler

• primary central nervous system lymphoma
• prognosis
• survival analysis

Kaynakça

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