背景:Dinutuximab是一种靶向GD2抗原的单克隆抗体,用于治疗高危神经母细胞瘤。Dinutuximab相关的菱形脑炎和脊髓炎是一种罕见的,类固醇反应,严肃,而是可逆的病理学.迄今为止,已经报道了3例横贯性脊髓炎和1例因dinutuximab引起的菱形脑炎。此外,最近发表的一篇文章确定了5例炎症性中枢神经系统脱髓鞘病例(4例脊髓炎和1例菱形脑炎).我们介绍了一名5岁的患者,在使用丁妥昔单抗-β治疗后患有菱形脑炎和脊髓炎。
方法:一名5岁患者,左侧腹膜后肿块浸润左肾,多发性溶解性骨病变,经腹部肿块经皮活检诊断为神经母细胞瘤。在腹部CT上检测到明显的治疗反应后进行手术。对腹部进行放射治疗。当她还在接受13-顺式视黄酸的维持治疗时,间碘苄基胍(MIBG)扫描检测到新的骨病变,脑MRG确定了厚膜受累。开始新的化疗方案,在所有先前的骨病变中均发现MIBG摄取降低。然而,在随后的MIBG扫描中发现了新发展的第八肋骨转移。进行自体干细胞移植。不久之后,丁妥昔单抗-β,再加上替莫唑胺和伊立替康,已启动。第三个周期低血压后,嗜睡,轻瘫,单侧固定散瞳。之后,观察到偏瘫样不规则肢体运动。体检研究并不引人注目,除了脑CT上脑干的低密度。MRI显示脑干和脊髓的T2高强度从颈髓质交界处延伸到T7水平。此外,观察到不完全的对比增强和促进扩散。影像学发现提示脱髓鞘。开始类固醇和静脉注射免疫球蛋白(IVIG)治疗。影像学异常和临床症状在1个月时部分消失,在6个月时消失。
结论:了解dinutuximab毒性的放射学发现将导致及时的诊断和治疗。
Dinutuximab is a monoclonal antibody that targets the GD2 antigen used in the treatment of high-risk neuroblastoma.
Dinutuximab-associated rhombencephalitis and myelitis is a rare, steroid-responsive, serious, but reversible pathology. To date, three transverse myelitis cases and one rhombencephalitis case due to
dinutuximab have already been reported. Moreover, a recently published article identified five inflammatory CNS demyelination cases (four myelitis and one rhombencephalitis). We present a 5-year-old patient with rhombencephalitis and myelitis following
dinutuximab-beta treatment.
A 5-year-old patient with a left-sided retroperitoneal mass infiltrating the left kidney and multiple lytic bone lesions was diagnosed with neuroblastoma with a percutaneous biopsy from the abdominal mass. Surgery was performed after a prominent treatment response was detected on the abdominal CT. Radiotherapy was applied to the abdomen. While she was still undergoing maintenance treatment with 13-cis retinoic acid, a metaiodobenzylguanidine (MIBG) scan detected new bone lesions, and brain MRG identified pachymeningeal involvement. A new chemotherapy regimen was started and decreased MIBG uptake was seen in all previous bone lesions. However, newly developed eighth rib metastasis was seen in the following MIBG scan. Autologous stem cell transplantation was done. Soon after,
dinutuximab-beta, together with temozolomide and irinotecan, was initiated. Following the third cycle hypotension, somnolence, paraparesis, and unilateral fixed dilated pupil were developed. Afterward, hemiballismus-like irregular limb movements were observed. Work-up studies were unremarkable, except for hypodensity in the brain stem on the brain CT. MRI revealed T2 hyperintensity of the brainstem and spinal cord extending from the cervicomedullary junction to the T7 level. Moreover, incomplete contrast enhancement and facilitated diffusion were observed. Imaging findings suggested demyelination. Steroids and intravenous immune globulin (IVIG) treatment were initiated. Both imaging abnormalities and clinical symptoms resolved partially at one month and disappeared at six months.
Awareness of the radiological findings of dinutuximab toxicity will lead to prompt diagnosis and treatment.