Prognostic importance of cellular differentiation in medulloblastoma of childhood.
Prognostic importance of cellular differentiation in medulloblastoma of childhood.
复制标题
儿童髓母细胞瘤细胞分化的预后重要性。
DOI：
10.3171/jns.1984.61.2.0296
复制
发表时间：
1984
影响因子：
4.1
通讯作者：
L. Schut
中科院分区：
文献类型：
--
作者：
R. Packer;L. Sutton;L. Rorke;P. Littman;R. Sposto;J. Rosenstock;D. Bruce;L. Schut
髓母细胞瘤是儿童期最常见的颅内原始神经外胚层恶性肿瘤。某些参数可以预测儿童髓母细胞瘤的生存率，然而，肿瘤组织学的预后价值尚不清楚。采用Rorke提出的由原始神经上皮细胞组成的中枢神经系统（CNS）肿瘤的分类系统，对38例新诊断的髓母细胞瘤进行了回顾性分析。这种分类是基于这样一个概念，即髓母细胞瘤不是小脑所特有的，而是类似于原始神经上皮细胞的肿瘤转化后可能在CNS其他部位发生的肿瘤。形成肿瘤的细胞可以保持在未分化状态，或者它们可以沿沿着神经胶质和/或室管膜和/或神经元线表现出分化。为简化起见，将病例分为两大组：未显示细胞分化证据的原始神经外胚层肿瘤（PNET）（PNET-U）和分化的原始神经外胚层肿瘤（PNET-D）。PNET-U组20例，PNET-D组18例。PNET-U的4年生存率为70%，而PNET-D为32%（p = 0.004）。10名PNET-D患儿中只有1名沿着一个以上细胞系分化，存活。其他因素，包括诊断时的年龄，肿瘤转移（TM）阶段，手术切除的程度，进行了分析，并预后的重要性，但组织学特征仍然在每个亚组的统计学意义。
Medulloblastoma is the most common intracranial primitive neuroectodermal malignancy of childhood. Certain parameters are predictive of survival in children with medulloblastoma; however, tumor histology is of unclear prognostic value. A classification system, proposed by Rorke for all central nervous system (CNS) neoplasms composed of primitive neuroepithelial cells, was utilized in a review of 38 consecutive patients with newly diagnosed medulloblastoma. The classification is based on the concept that medulloblastoma is not unique to the cerebellum but is similar to tumors that may arise elsewhere in the CNS consequent to neoplastic transformation of primitive neuroepithelial cells. Cells forming the tumors may remain in the undifferentiated state or they may exhibit differentiation along glial, and/or ependymal, and/or neuronal lines. For purposes of simplification, the cases were divided into two major groups: those primitive neuroectodermal tumors (PNET's) which showed no evidence of cellular differentiation (PNET-U) and those that were differentiated (PNET-D). There were 20 cases in the PNET-U group and 18 in the PNET-D group. The 4-year survival rate was 70% for PNET-U, compared to 32% for PNET-D (p = 0.004). Only one of 10 children with PNET-D with differentiation along more than one cell line survived. Other factors, including age at diagnosis, tumor metastasis (TM) stage, and extent of surgical resection, were analyzed and were of prognostic importance; but histological features remained statistically significant within each subgroup.
利用现代技术提高髓母细胞瘤的无复发生存率。
影响因子：
4.8
作者：
Norris,DG;Bruce,DA;Byrd,RL;Schut,L;Littman,P;Bilaniuk,LT;Zimmerman,RA;Capp,R
通讯作者：
Capp,R