Chelliah Daniel, Mensah Sarfo-Poku Christian, Stea Baldassarre D, Gardetto Jennifer, Zumwalt Jonathan
School of Medicine, Loma Linda University, Loma Linda, CA, USA.
Pediatr Neurosurg. 2010;46(5):381-4. doi: 10.1159/000322896. Epub 2011 Mar 9.
We present a report of a 12-year-old boy diagnosed with medulloblastoma at 22 months of age. A gross total resection was performed followed by adjuvant systemic chemotherapy due to his young age; however, the tumor recurred locally in the posterior fossa 7 months later. The recurrent tumor was excised and he received craniospinal radiation with a boost given to the posterior fossa followed by high-dose chemotherapy. He remained disease free for approximately 10 years without major neurologic deficit and only mild cognitive impairment. A routine follow-up MRI of the brain revealed an enhancing mass. The patient underwent surgical debulking and pathological examination revealed no residual immature medulloblastoma cells but instead mature ganglion cells, consistent with a gangliocytoma. The apparent maturation of primitive medulloblastoma cells is a rare phenomenon, which may have ensued from the long-term effects of adjuvant therapies inducing advanced cellular maturation.
我们报告一例12岁男孩，其在22个月大时被诊断为髓母细胞瘤。由于年龄较小，进行了全切除手术，随后进行辅助全身化疗；然而，7个月后肿瘤在后颅窝局部复发。复发性肿瘤被切除，他接受了颅脊髓放疗，并对后颅窝进行了强化放疗，随后进行了高剂量化疗。他在大约10年的时间里一直无病生存，没有严重的神经功能缺损，仅有轻度认知障碍。脑部的常规随访MRI显示有一个强化肿块。患者接受了手术减瘤，病理检查未发现残留的未成熟髓母细胞瘤细胞，而是成熟的神经节细胞，符合神经节细胞瘤。原始髓母细胞瘤细胞的明显成熟是一种罕见现象，可能是由于辅助治疗的长期作用导致细胞晚期成熟所致。
