Kim Chan-Seop, Choi Chi-Hoon, Yi Kyung Sik, Kim Yook, Lee Jisun, Woo Chang Gok, Jeon Young Hun
Department of Radiology, Chungbuk National University Hospital, Chungbuk National University College of Medicine, Cheongju-si 28644, Chungcheongbuk-do, South Korea.
Department of Pathology, Chungbuk National University Hospital, Chungbuk National University College of Medicine, Cheongju-si 28644, Chungcheongbuk-do, South Korea.
World J Clin Cases. 2024 Jan 16;12(2):374-382. doi: 10.12998/wjcc.v12.i2.374.
Primary central nervous system lymphoma (PCNSL) is a non-Hodgkin lymphoma that originates in the central nervous system (CNS) and is exclusively limited to the CNS. Although most PCNSLs are diffuse large B-cell lymphomas, primary CNS T-cell lymphomas (PCNSTLs) are rare. PCNSTLs typically demonstrate some degree of enhancement on contrast-enhanced magnetic resonance imaging (MRI). To the best of our knowledge, non-enhancing PCNSTL has not been reported previously.
A 69-year-old male presented to the neurology department with complaints of mild cognitive impairment and gradual onset of left lower leg weakness over a span of two weeks. Initial MRI showed asymmetric T2-hyperintense lesions within the brain. No enhancement was observed on the contrast-enhanced T1 image. The initial diagnosis was neuro-Behçet's disease. Despite high-dose steroid therapy, no alterations in the lesions were identified on initial MRI. The patient's symptoms deteriorated further. An MRI performed one month after the initial scan revealed an increased lesion extent. Subsequently, brain biopsy confirmed the diagnosis of PCNSTL. The patient underwent definitive combined chemo-radiotherapy. However, the patient developed bacteremia and died of septic shock approximately three months after diagnosis.
The absence of enhancement in the lesion did not rule out PCNSTL. A biopsy approach is advisable for pathological confirmation.
原发性中枢神经系统淋巴瘤（PCNSL）是一种起源于中枢神经系统（CNS）且仅局限于CNS的非霍奇金淋巴瘤。尽管大多数PCNSL为弥漫性大B细胞淋巴瘤，但原发性中枢神经系统T细胞淋巴瘤（PCNSTL）较为罕见。PCNSTL在对比增强磁共振成像（MRI）上通常表现出一定程度的强化。据我们所知，此前尚未报道过无强化的PCNSTL。
一名69岁男性因轻度认知障碍以及在两周内逐渐出现左下肢无力症状就诊于神经科。最初的MRI显示脑内不对称T2高信号病变。在对比增强T1图像上未观察到强化。初步诊断为神经白塞病。尽管给予了大剂量类固醇治疗，但在最初的MRI上未发现病变有变化。患者症状进一步恶化。初次扫描后1个月进行的MRI显示病变范围扩大。随后，脑活检确诊为PCNSTL。患者接受了确定性联合放化疗。然而，患者发生了菌血症，并在诊断后约3个月死于感染性休克。
病变无强化并不能排除PCNSTL。建议采用活检方法进行病理确诊。
