Primer Santral Sinir Sistemi Lenfomaları
Özet
Primer Santral Sinir Sistemi Lenfomaları (PSSSL), sistemik tutulum olmaksızın beyin, göz, omurilik ve leptomeninkslerde gelişen agresif bir Non-Hodgkin lenfoma türüdür. Tüm beyin tümörlerinin %3'ünü oluşturan bu hastalık, çoğunlukla diffüz büyük B hücreli alt tipten köken alır. Multidisipliner yönetim gerektiren hastalıktır. Kontrastlı MR en iyi görüntüleme seçeneğiyken, tanı ve evrelemede F-18 FDG PET ve CXCR4 hedefli yeni ajanlar kullanılmaktadır. İnvaziv cerrahi rezeksiyon yerine nörolojik hasar riski düşük olan stereotaktik biyopsi tercih edilir. Tedavi planlamasında IELSG ve MSKCC skorlama sistemleri ile hastanın yaşı ve performans durumu belirleyicidir. Birinci basamak tedavi, yüksek doz metotreksat bazlı indüksiyon kemoterapisi ve ardından genç hastalarda otolog kök hücre nakliyle yapılan konsolidasyondan oluşur. Yaşlı hastalarda radyoterapinin ciddi nörotoksik etkileri nedeniyle kemoterapi ön plandadır. Tedavi sonrası ilk iki yılda nüks sıktır. Relaps ve refrakter vakalarda metotreksat ile yeniden indüksiyon, ifosfamidli rejimler veya ibrutinib, temsirolimus ve immünomodülatörler (lenalidomid) gibi hedefe yönelik yeni ajanlar araştırılmaktadır.
Primary Central Nervous System Lymphoma (PCNSL) is an aggressive form of Non-Hodgkin lymphoma that develops in the brain, eyes, spinal cord, and leptomeninges without systemic involvement. Accounting for approximately 3% of all brain tumors, it predominantly originates from the diffuse large B-cell subtype. Multidisciplinary management is essential. While contrast-enhanced MRI is the optimal imaging modality, F-18 FDG PET and novel CXCR4-targeted agents are utilized for staging. Stereotactic biopsy is preferred over invasive surgical resection due to a lower risk of neurological deficits. Treatment planning relies on the IELSG and MSKCC scoring systems, alongside the patient's age and performance status. First-line therapy consists of high-dose methotrexate-based induction chemotherapy, followed by consolidation with autologous stem cell transplantation in eligible younger patients. Due to the severe neurotoxic effects of consolidative radiotherapy, chemotherapy is prioritized in elderly patients. Relapse is common within the first two years. In relapsed or refractory cases, re-induction with methotrexate, ifosfamide-containing regimens, or novel targeted agents such as ibrutinib, temsirolimus, and immunomodulatory drugs (lenalidomide) are being investigated.
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