Primary central nervous system lymphoma in a patient with systemic lupus erythematosus mimicking high-grade glioma: A case report and review of literature.
Primary central nervous system lymphoma in a patient with systemic lupus erythematosus mimicking high-grade glioma: A case report and review of literature.
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DOI：
10.1097/md.0000000000011072
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发表时间：
2018-06
期刊：
影响因子：
1.6
通讯作者：
Lao M
中科院分区：
文献类型：
--
作者：
Su L;Ding M;Chen L;Li C;Lao M
原发性中枢神经系统淋巴瘤是一种罕见的疾病。风湿性疾病患者的PCNSL研究缺乏。临床症状和影像学表现均不是PCNSL所特有的。因此，它可能被误诊为其他疾病，如脑肿瘤。化疗是PCNSL的主要治疗方法，而手术的作用仍然存在争议。我们报告一位39岁女性系统性红斑狼疮（SLE）患者，在接受多种免疫抑制剂治疗15年后发生PCNSL。头颅磁共振成像（MRI）显示右半球多灶性病变，增强后呈环状增强，影像学上与胶质瘤相似。由于颅内高压症状严重，进行了大体肿瘤切除术。病理学检查显示血管周围浸润的非典型淋巴细胞，CD20和EB病毒（EBV）编码的RNA（EREB）阳性。患者被诊断为弥漫性大B细胞淋巴瘤（DLBCL）。患者随后接受了6个周期的化疗和自体干细胞移植（ASCT）。在撰写本文之前，患者一直保持完全缓解。免疫功能低下者的PCNSL可表现为不均匀性增强，应与其他疾病特别是高级别胶质瘤相鉴别。
Primary central nervous system lymphoma (PCNSL) is a rare disease. Studies of PCNSL in patients with rheumatic diseases are lacking. Neither clinical symptoms nor radiographic manifestation is specific to PCNSL. Therefore, it could be misdiagnosed with other diseases such as brain tumors. Chemotherapy is the primary treatment for PCNSL, while the role of surgery remains controversial. We reported a 39-year-old woman with systemic lupus erythematosus (SLE) developed PCNSL after 15-year treatment with multiple immunosuppressants. Cranial magnetic resonance imaging (MRI) showed multi-focal lesions with ring-like enhancement post-contrast in the right hemisphere, which mimicked glioma radiographically. Owing to the severe symptoms of intracranial hypertension, gross tumor resection was performed. Pathological exam showed perivascular infiltration of atypical lymphoid cells with CD20 and Epstein-Barr virus (EBV) -encoded RNA (EREB) positive. The patient was diagnosed with diffuse large B-cell lymphoma (DLBCL). The patient received six cycles of chemotherapy and autologous stem cell transplantation (ASCT) subsequently. The patient remained complete remission until this article was written. PCNSL in immunocompromised hosts may present heterogeneous contrast enhancement, which should be differentiated from other diseases especially high-grade glioma.