Coelho Joana, Nunes Sofia, Salgado Duarte
Department of Pediatric, Hospital de Santa Maria, Lisbon, Portugal.
Department of Pediatric Neurology, Portuguese Oncology Institute of Lisbon, Portugal.
Child Neurol Open. 2015 Jan 30;2(1):2329048X14566813. doi: 10.1177/2329048X14566813. eCollection 2015 Jan-Mar.
Pilocytic astrocytoma is a slowly growing neoplasia that represents the most frequent cerebral tumor in pediatric age. Malignant transformation is rare and it is usually related to previous radiotherapy. The authors describe a case of a spontaneous malignant transformation of a pilocytic astrocytoma. A 3-year-old boy was diagnosed with a cerebellar hemisphere tumor. He was submitted to a complete excision of the lesion, and histological findings were consistent with pilocytic astrocytoma. It was negative for p53. Twelve years later he presented with a local recurrence. Histopathological diagnosis was glioblastoma and it was positive for p53. Death from disease progression occurred 16 months after the diagnosis of glioblastoma. This case suggests that patients with pilocytic astrocytoma need closer follow-up and further genotypic studies in order to provide clues to clinical behavior. Such understanding can allow us to stratify treatment accordingly and to proceed to more aggressive treatment when necessary.
毛细胞型星形细胞瘤是一种生长缓慢的肿瘤,是儿童期最常见的脑肿瘤。恶性转化罕见,通常与先前的放疗有关。作者描述了一例毛细胞型星形细胞瘤自发恶性转化的病例。一名3岁男孩被诊断为小脑半球肿瘤。他接受了病变的完全切除,组织学检查结果符合毛细胞型星形细胞瘤。p53检测为阴性。12年后,他出现局部复发。组织病理学诊断为胶质母细胞瘤,p53检测为阳性。胶质母细胞瘤诊断16个月后,患者因疾病进展死亡。该病例表明,毛细胞型星形细胞瘤患者需要更密切的随访和进一步的基因分型研究,以便为临床行为提供线索。这样的了解可以使我们据此对治疗进行分层,并在必要时采取更积极的治疗措施。