Jakacki R I, Zeltzer P M, Boyett J M, Albright A L, Allen J C, Geyer J R, Rorke L B, Stanley P, Stevens K R, Wisoff J
Riley Hospital for Children, Indianapolis, IN, USA.
J Clin Oncol. 1995 Jun;13(6):1377-83. doi: 10.1200/JCO.1995.13.6.1377.
To describe the biologic and clinical features of children with primitive neuroectodermal tumors (PNETs) arising in the pineal region (pineoblastomas) and evaluate prospectively the efficacy of radiation therapy (RT) and/or chemotherapy.
Between 1986 and 1992, 25 children with PNETs of the pineal region were treated as part of a Childrens Cancer Group study. Eight infants less than 18 months of age were nonrandomly treated with eight-drugs-in-1-day chemotherapy without RT. The remaining 17 patients were treated with craniospinal RT and randomized to receive either vincristine, lomustine (CCNU), and prednisone or the eight-drugs-in-1-day regimen.
Of 24 completely staged patients, 20 (83%) had localized disease at diagnosis. All infants developed progressive disease a median of 4 months from the start of treatment. Of the 17 older patients treated with RT and chemotherapy, the Kaplan-Meier estimate of progression-free survival (PFS) at 3 years is 61% +/- 13%. This is superior to the PFS of children with other supratentorial PNETs (P = .026). Following RT, 12 of 17 patients (70.6%) had a residual pineal region mass, which persisted for as long as 5 years before resolving; only four subsequently developed progressive disease.
(1) Eight-in-1 chemotherapy without RT appears to be ineffective therapy for young children with PNETs of the pineal region. (2) For children more than 18 months of age at diagnosis treated with craniospinal RT and chemotherapy, the PFS is superior to that of children with other supratentorial PNETs. (3) A residual enhancing mass following RT is not predictive of treatment failure.
描述松果体区原始神经外胚层肿瘤（PNETs，松果体母细胞瘤）患儿的生物学和临床特征，并前瞻性评估放射治疗（RT）和/或化疗的疗效。
1986年至1992年间，25例松果体区PNETs患儿作为儿童癌症研究组研究的一部分接受治疗。8例18个月以下婴儿接受了1天8药联合化疗，未接受放疗，治疗方式为非随机分配。其余17例患者接受了全脑全脊髓放疗，并随机接受长春新碱、洛莫司汀（CCNU）和泼尼松或1天8药联合方案治疗。
在24例分期完整的患者中，20例（83%）在诊断时为局限性疾病。所有婴儿在治疗开始后中位4个月出现疾病进展。在17例接受放疗和化疗的年长患者中，3年无进展生存期（PFS）的Kaplan-Meier估计值为61%±13%。这优于其他幕上PNETs患儿的PFS（P = 0.026）。放疗后，17例患者中有12例（70.6%）松果体区有残留肿块，该肿块持续长达5年才消退；只有4例随后出现疾病进展。
（1）对于松果体区PNETs幼儿，单纯1天8药联合化疗似乎无效。（2）对于诊断时年龄超过18个月且接受全脑全脊髓放疗和化疗的患儿，其PFS优于其他幕上PNETs患儿。（3）放疗后残留强化肿块并非治疗失败的预测指标。
