van den Akker Machiel, Northcott Paul, Taylor Michael D, Halliday William, Bartels Ute, Bouffet Eric
Division of Haematology/Oncology, Hospital for Sick Children, University of Toronto, Ontario, Canada.
J Neurosurg Pediatr. 2012 Jul;10(1):21-4. doi: 10.3171/2012.3.PEDS11152. Epub 2012 Jun 15.
A 9-year-old boy with known Duchenne type muscular dystrophy (DMD) presented with signs of increased intracranial pressure. Radiological investigations revealed a lesion in the midline of the posterior fossa. Subtotal resection was performed. Pathology findings were consistent with the diagnosis of anaplastic medulloblastoma. The postoperative lumbar CSF was positive for malignant cells. Postoperatively, the patient showed severe neurological deterioration and lost his capacity to walk. He was treated with craniospinal radiation followed by nonintensive chemotherapy. At 30 months postsurgery, he was still in complete remission but had not recovered his walking ability. This is the second report of a malignant brain tumor in a boy with DMD. The possible link between the 2 conditions is discussed, as are ethical considerations regarding the management of medulloblastoma in children with DMD.
一名患有杜氏型肌营养不良症(DMD)的9岁男孩出现颅内压升高的症状。影像学检查发现后颅窝中线有一个病变。进行了次全切除。病理结果与间变性髓母细胞瘤的诊断一致。术后腰椎脑脊液中恶性细胞呈阳性。术后,患者出现严重的神经功能恶化,失去了行走能力。他接受了颅脊髓放疗,随后进行了非强化化疗。术后30个月,他仍处于完全缓解状态,但尚未恢复行走能力。这是第二例关于患有DMD的男孩发生恶性脑肿瘤的报告。讨论了这两种情况之间可能的联系,以及关于DMD患儿髓母细胞瘤治疗的伦理考量。