Kinoshita Hisanori, Yamakado Hodaka, Kitano Toshiyuki, Kitamura Akihiro, Yamashita Hirofumi, Miyamoto Masakazu, Hitomi Takefumi, Okada Tomohisa, Nakamoto Yuji, Sawamoto Nobukatsu, Takaori-Kondo Akifumi, Takahashi Ryosuke
Department of Neurology, Kyoto University Graduate School of Medicine, 54 Shogoin-Kawahara-cho, Sakyo-ku, Kyoto, 606-8507, Japan.
Department of Hematology and Oncology, Kyoto University Graduate School of Medicine, Kyoto, Japan.
J Neurol. 2016 Sep;263(9):1719-26. doi: 10.1007/s00415-016-8190-4. Epub 2016 Jun 10.
Neurolymphomatosis (NL) is a rare condition involving the infiltration of lymphoma cells into the peripheral nervous system. NL can be primary or secondary in the setting of an unknown or known hematologic malignancy, respectively. Here, we report five cases in which F-18 2-fluoro-2-deoxy-glucose positron emission tomography/computed tomography (F-18 FDG-PET/CT) had great value for diagnosing NL. Two cases were rare primary NL, and the other three were secondary NL. Clinical presentations were asymmetric sensorimotor disturbances in the extremities with or without involvement of cranial nerves. Furthermore, all patients experienced spontaneous pain in the face or affected extremities. Cerebrospinal fluid analysis was cytologically negative in two of five cases. Gadolinium (Gd)-enhanced magnetic resonance imaging (MRI) detected abnormalities in the cranial nerves, nerve roots, and cauda equina in all cases except case 1 and the recurrent stage of case 2. F-18 FDG-PET/CT showed clear visualization of almost all the lymphomatous involvement of peripheral nerves and other tissues in all patients. Furthermore, F-18 FDG-PET/CT detected abnormalities including asymptomatic lesions that were not detected with MRI, and also identified the appropriate lesion for diagnostic biopsy. However, as in case 3, the lesions in the left oculomotor nerve and the cauda equina were detected only with Gd-enhanced MRI, which has superior spatial resolution. In conclusion, F-18 FDG-PET/CT is a sensitive modality that can suggest the presence of malignancy and identify appropriate places for diagnostic biopsies. It is especially useful when combined with Gd-enhanced MRI, even in patients with primary NL that is usually difficult to diagnose.
神经淋巴瘤(NL)是一种罕见的疾病,其特征为淋巴瘤细胞浸润至周围神经系统。NL 可分别为原发性(病因不明)或继发性(继发于已知的血液系统恶性肿瘤)。在此,我们报告 5 例 F-18 2-氟-2-脱氧葡萄糖正电子发射断层扫描/计算机断层扫描(F-18 FDG-PET/CT)对诊断 NL 具有重要价值的病例。其中 2 例为罕见的原发性 NL,另外 3 例为继发性 NL。临床表现为四肢不对称的感觉运动障碍,可伴有或不伴有颅神经受累。此外,所有患者均有面部或受累肢体的自发疼痛。5 例中有 2 例脑脊液分析细胞学检查为阴性。钆(Gd)增强磁共振成像(MRI)在除病例 1 和病例 2 复发阶段外的所有病例中均检测到颅神经、神经根和马尾神经的异常。F-18 FDG-PET/CT 在所有患者中均清晰显示了几乎所有周围神经及其他组织的淋巴瘤累及情况。此外,F-18 FDG-PET/CT 检测到包括 MRI 未发现的无症状病变在内的异常情况,还确定了适合诊断性活检的病变部位。然而,如病例 3 所示,仅通过具有更高空间分辨率的 Gd 增强 MRI 检测到左侧动眼神经和马尾神经的病变。总之,F-18 FDG-PET/CT 是一种敏感的检查方法,可提示恶性肿瘤的存在并确定适合诊断性活检的部位。即使在通常难以诊断的原发性 NL 患者中,与 Gd 增强 MRI 联合使用时也特别有用。