Department of Neurosurgery, The Aga Khan University, Karachi, Pakistan.
Department of Oncology, Ziauddin University Hospital, Karachi, Pakistan.
J Pak Med Assoc. 2024 Mar;74(3 (Supple-3)):S168-S176. doi: 10.47391/JPMA.S3.GNO-20.
Primary lymphoma of the central nervous system (PCNSL) is a rare and aggressive form of extranodal non-Hodgkin lymphoma primarily involving the brain, spinal cord, cerebrospinal fluid, and eyes. The role of surgical intervention in PCNSL is currently limited to biopsy and decompression of critical structures if needed - extended resection is debated. Chemotherapy is the mainstay of treatment. In lower and middle-income countries (LMICs), issues like delayed diagnosis and resource constraints are widespread. These guidelines provide a framework for addressing PCNSL in LMICs, emphasizing the importance of early diagnosis, tailored treatment approaches, and ongoing patient monitoring to improve outcomes for this rare and aggressive disease.
原发性中枢神经系统淋巴瘤（PCNSL）是一种罕见且侵袭性较强的结外非霍奇金淋巴瘤，主要累及大脑、脊髓、脑脊液和眼睛。手术干预在 PCNSL 中的作用目前仅限于活检和必要时对关键结构进行减压——广泛切除仍存在争议。化疗是治疗的主要手段。在中低收入国家（LMICs），普遍存在诊断延迟和资源受限等问题。这些指南为解决 LMICs 中的 PCNSL 提供了一个框架，强调了早期诊断、量身定制的治疗方法以及对患者的持续监测的重要性，以改善这种罕见且侵袭性疾病的预后。
