Katenkamp D, Stiller D, Holzhausen H J
Zentralbl Allg Pathol. 1983;127(3-4):207-18.
The neuroblastoma is one of the most frequent malignant solid tumors in childhood and is thus of great practical importance. The origin of neuroblastoma cells from neural crest derivatives is generally accepted now, and this histogenesis explains some biochemical and morphological characteristics of the tumor. The cytological and histological features of neuroblastomas can be rather varying and, therefore, the diagnosis and differential diagnosis may be difficult. Our study presents the findings of 48 neuroblastomas after light microscopic examination and the ultrastructural characteristics of 8 neuroblastomas and 1 ganglioneuroma. At light microscopic level, completely undifferentiated neuroblastomas and tumors with variable degrees of differentiation were identified. The differentiation of the tumor tissue to ganglion cell-like elements was indicated by an increasing amount of cellular cytoplasm with development of a cytoplasmic process as well as an alteration of the picture of the nucleus (nuclear enlargement and a clearly visible nucleolus). Differentiation to Schwann cell-like elements was occasionally observed, too. Electron microscopically, in all tumors neurosecretory granules could be recognized, and in the better differentiated areas neurite-like cytoplasmic projections were detectable. Thus, the electron microscopy can be a valuable aid in establishing an unequivocal diagnosis. The histology of neuroblastomas is said to be of prognostic significance. Therefore, grading schemes of malignancy were developed. At present, the grading procedure after Hughes and coworkers is mostly used. The criteria of this grading system are presented and interpreted. Finally, those tumors are briefly characterized which play the main role in the differential diagnosis, i.e. juvenile rhabdomyosarcomas, Ewing's sarcoma (including the extraskeletal type), lymphoblastic lymphoma and histiocytic reticulosarcoma. The most important clinicopathologic differences in comparison to neuroblastomas are referred to. Using a large scale of morphologic methods as well as considering clinical and paraclinical parameters, the exact diagnosis of neuroblastoma should be possible in nearly every case.
神经母细胞瘤是儿童期最常见的恶性实体瘤之一，因此具有重要的实际意义。神经母细胞瘤细胞起源于神经嵴衍生物这一观点目前已被广泛接受，这种组织发生学解释了该肿瘤的一些生化和形态学特征。神经母细胞瘤的细胞学和组织学特征差异较大，因此诊断和鉴别诊断可能存在困难。我们的研究展示了48例神经母细胞瘤经光镜检查后的结果以及8例神经母细胞瘤和1例神经节神经瘤的超微结构特征。在光镜水平，识别出了完全未分化的神经母细胞瘤以及具有不同分化程度的肿瘤。肿瘤组织向神经节样细胞成分的分化表现为细胞质增多、出现细胞质突起以及细胞核形态改变（核增大且核仁清晰可见）。偶尔也观察到向施万细胞样成分的分化。在电镜下，所有肿瘤中均可识别出神经分泌颗粒，在分化较好的区域可检测到神经突样细胞质突起。因此，电镜检查有助于明确诊断。据说神经母细胞瘤的组织学具有预后意义。因此，制定了恶性程度分级方案。目前，大多采用休斯及其同事提出的分级方法。介绍并解释了该分级系统的标准。最后，简要描述了在鉴别诊断中起主要作用的那些肿瘤，即青少年横纹肌肉瘤、尤因肉瘤（包括骨外型）、淋巴细胞性淋巴瘤和组织细胞性网状肉瘤。提及了与神经母细胞瘤相比最重要的临床病理差异。运用多种形态学方法并结合临床和副临床参数，几乎在每种情况下都应能准确诊断神经母细胞瘤。
