Division of Neurology.
Division of Oncology, Children's Hospital of Philadelphia.
J Pediatr Hematol Oncol. 2023 Apr 1;45(3):152-154. doi: 10.1097/MPH.0000000000002643. Epub 2023 Feb 20.
Opsoclonus myoclonus ataxia syndrome (OMAS) is a rare disorder that causes significant neurodevelopmental sequelae in children. Approximately half of pediatric OMAS cases are paraneoplastic, typically associated with localized neuroblastic tumors. Since early persistence or relapse of OMAS symptoms is common even after tumor resection, OMAS relapses may not routinely prompt reevaluation for recurrent tumors. We report a 12-year-old girl with neuroblastic tumor recurrence associated with OMAS relapse a decade after initial treatment. Providers should be aware of tumor recurrence as a trigger for distant OMAS relapse, raising intriguing questions about the role of immune surveillance and control of neuroblastic tumors.
眼震-肌阵挛-共济失调综合征(OMAS)是一种罕见的疾病,会导致儿童出现严重的神经发育后遗症。大约一半的小儿 OMAS 病例为副肿瘤性的,通常与局部神经母细胞瘤相关。由于即使在肿瘤切除后,OMAS 症状的早期持续存在或复发也很常见,因此 OMAS 的复发通常不会常规提示重新评估是否存在复发性肿瘤。我们报告了一例 12 岁女孩,在初始治疗 10 年后出现神经母细胞瘤复发和 OMAS 复发。提供者应该意识到肿瘤复发是 OMAS 远处复发的触发因素,这引发了关于免疫监视和神经母细胞瘤控制作用的有趣问题。