Hukin Juliette, Siffert Joao, Velasquez Linda, Zagzag David, Allen Jeffrey
Division of Neurology, Department of Pediatrics, Children's and Women's Hospital, Vancouver, British Columbia, V6H3V4 Canada.
Neuro Oncol. 2002 Oct;4(4):253-60. doi: 10.1093/neuonc/4.4.253.
Our purpose is to describe the incidence and clinical features of leptomeningeal dissemination (LM) in children with progressive low-grade neuroepithelial tumor (LGN). We have continuously tracked all patients with primary CNS tumors since 1986. Satisfactorily followed data were obtained on 427 of the 588 patients with localized LGN at diagnosis between 1986 and 1998, 177 (42%) of whom developed progressive or recurrent disease. LM was identified in 13/177 (7%). The median age at initial diagnosis was 5 years and at LM diagnosis was 8.5 years. The primary tumor sites were diencephalon (6), brainstem (3), cerebellum (2), cerebrum (1), and spinal cord (1). The histologies were pilocytic astrocytoma (4), ganglioglioma (4), fibrillary astrocytoma (3), mixed glioma (1), and glioneurofibroma (1). Management included chemotherapy (2) or radiotherapy (3) or both (7); 1 patient received only radical resections of symptomatic lesions. The 5-year progression-free survival rates for patients with localized versus LM disease at recurrence were 22% (95% confidence interval [CI], 13%-25%) versus 15% (95% CI, 0.1%-36%), respectively ( P = 0.28). The 5- and 10-year overall survival rates for patients with localized disease versus LM were 87% (95% CI, 82%-92%) and 83% (95% CI, 77%-89%) versus 68% (95% CI, 39%-91%) and 68% (95% CI, 39%-91%), respectively ( P = 0.05). The 7% incidence of LM is a low estimate because patients were not routinely staged at recurrence. Tumors arising from the diencephalon appeared to predispose to LM; no other predisposing features were identified. We strongly urge that for optimum treatment planning all patients with recurrent LGN be staged with an enhanced spine and brain MRI before adjuvant therapy is initiated. The good survival of patients with LGN and LM reflects a more indolent disease than malignant CNS tumors with LM.
我们的目的是描述进展性低级别神经上皮肿瘤（LGN）患儿软脑膜播散（LM）的发生率及临床特征。自1986年以来，我们持续追踪了所有原发性中枢神经系统肿瘤患者。在1986年至1998年间确诊的588例局限性LGN患者中，427例获得了满意的随访数据，其中177例（42%）出现疾病进展或复发。177例中有13例（7%）确诊为LM。初次诊断时的中位年龄为5岁，LM诊断时为8.5岁。原发肿瘤部位为间脑（6例）、脑干（3例）、小脑（2例）、大脑（1例）和脊髓（1例）。组织学类型为毛细胞型星形细胞瘤（4例）、神经节胶质瘤（4例）、纤维型星形细胞瘤（3例）、混合性胶质瘤（1例）和神经胶质纤维瘤（1例）。治疗包括化疗（2例）、放疗（3例）或两者联合（7例）；1例患者仅接受了有症状病变的根治性切除。复发时局限性疾病患者与LM疾病患者的5年无进展生存率分别为22%（95%置信区间[CI]，13% - 25%）和15%（95% CI，0.1% - 36%）（P = 0.28）。局限性疾病患者与LM患者的5年和10年总生存率分别为87%（95% CI，82% - 92%）和87%（95% CI，77% - 89%）与68%（95% CI，39% - 91%）和68%（95% CI，39% - 91%）（P = 0.05）。7%的LM发生率是一个低估，因为患者复发时未常规进行分期。起源于间脑的肿瘤似乎易发生LM；未发现其他易感特征。我们强烈敦促，为了制定最佳治疗方案，所有复发LGN患者在开始辅助治疗前应进行增强脊柱和脑部MRI分期。LGN合并LM患者的良好生存率反映出该疾病比伴有LM的恶性中枢神经系统肿瘤更为惰性。
