Winters J L, Geil J D, O'Connor W N
Department of Pathology and Laboratory Medicine, College of Medicine, University of Kentucky, Lexington 40536, USA.
Ann Clin Lab Sci. 1995 Jan-Feb;25(1):66-78.
Malignancies of childhood include a well-defined spectrum of hematolymphoid, organ specific (adrenal, kidney, liver), soft tissue, bone, and nervous system (central and peripheral) neoplasms with variable biology. Small round cell neoplasms, a subset of childhood malignancies, are histologically similar but differ markedly in their histogenesis, therapy, and prognosis. Traditionally, clinical information and light microscopy, with the aid of histochemistry and ultrastructural evaluation, establish a diagnosis or at least narrow the differential diagnosis. Additionally, immunohistology, cytogenetics, and molecular studies have become important in diagnosis and in defining phenotype/genotype, patient treatment modalities, and prognosis in specific cases. The 11;22 chromosomal translocation typifies Ewing's sarcoma, primitive neuroectodermal tumor, and Askin's tumor, as does the resultant chimeric transcript, while expression and amplification of N-myc oncogene are predictive of the prognosis in neuroblastoma. Furthermore, studies of genes and gene products are elucidating mechanisms of oncogenesis and tumor progression.
儿童恶性肿瘤包括一系列明确的血液淋巴系统、器官特异性（肾上腺、肾脏、肝脏）、软组织、骨骼和神经系统（中枢和外周）肿瘤，其生物学特性各异。小圆细胞肿瘤是儿童恶性肿瘤的一个子集，在组织学上相似，但在组织发生、治疗和预后方面有显著差异。传统上，临床信息和借助组织化学及超微结构评估的光学显微镜检查可做出诊断，或至少缩小鉴别诊断范围。此外，免疫组织化学、细胞遗传学和分子研究在特定病例的诊断、定义表型/基因型、患者治疗方式及预后方面已变得至关重要。11；22染色体易位是尤因肉瘤、原始神经外胚层肿瘤和阿斯金肿瘤的典型特征，由此产生的嵌合转录本也是如此，而N - myc癌基因的表达和扩增可预测神经母细胞瘤的预后。此外，对基因和基因产物的研究正在阐明肿瘤发生和进展的机制。
