Nguyen-Them L, Alentorn A, Ahle G, Soussain C, Mathon B, Le Garff Tavernier M, Houillier C, Hoang-Xuan K
Centre Hospitalier Saint Jean, 20 Avenue du Languedoc, 66000 Perpignan, France; Hôpital Pitié Salpêtrière, APHP, Sorbonne Université, 47-83 Boulevard de l'Hôpital, 75013 Paris, France.
Hôpital Pitié Salpêtrière, APHP, Sorbonne Université, 47-83 Boulevard de l'Hôpital, 75013 Paris, France.
Rev Neurol (Paris). 2023 Mar;179(3):141-149. doi: 10.1016/j.neurol.2022.06.014. Epub 2022 Nov 3.
PCNSL is a non-Hodgkin lymphoma (NHL) affecting brain, spinal cord, eyes and leptomeninges. In the past two decades, its prognosis significantly improved due to therapeutic advances but it remains a highly aggressive tumor and early diagnosis is necessary for optimal management. Diagnosis relies on the identification of lymphoma cells in brain tissue obtained by stereotactic biopsy. Alternatively, lymphoma cells may be found in CSF through lumbar puncture (LP) or by a vitrectomy. For several reasons, the diagnosis of PCNSL may be challenging. Misleading radiological presentations are frequent. Dramatic response to steroids may bias histological analysis and deep brain location or frail health status can contraindicate brain biopsy. In the follow-up of patients who have been previously treated, differential diagnosis between tumor relapse and post-treatment may be also difficult. Therefore, the development of complementary reliable diagnostic tools is needed. This review will summarize several diagnostic or prognostic CSF biomarkers which have been proposed in PCNSL, their interests and limits.
原发性中枢神经系统淋巴瘤(PCNSL)是一种非霍奇金淋巴瘤(NHL),可累及脑、脊髓、眼和软脑膜。在过去二十年中,由于治疗进展,其预后有了显著改善,但它仍然是一种高度侵袭性的肿瘤,早期诊断对于最佳治疗管理至关重要。诊断依赖于通过立体定向活检获取的脑组织中淋巴瘤细胞的鉴定。另外,也可通过腰椎穿刺(LP)或玻璃体切除术在脑脊液中发现淋巴瘤细胞。由于多种原因,PCNSL的诊断可能具有挑战性。误导性的影像学表现很常见。对类固醇的显著反应可能会影响组织学分析,而脑深部位置或健康状况不佳可能会成为脑活检的禁忌证。在先前接受过治疗的患者的随访中,肿瘤复发与治疗后情况之间的鉴别诊断也可能很困难。因此,需要开发可靠的补充诊断工具。本综述将总结几种在PCNSL中提出的诊断或预后脑脊液生物标志物、它们的优势和局限性。