Harms D, Leuschner I, Krams M, Pilgrim T B, Treuner J
Institut für Pathologie, Christian-Albrechts-Universität zu Kiel.
Verh Dtsch Ges Pathol. 1998;82:83-98.
Rhabdomyosarcomas (RMS) and extraosseous Ewing's sarcomas (EOE) including malignant peripheral neuroectodermal tumors (MPNT) are the most frequent soft tissue malignancies of childhood. They account for 60.2% of 2.350 cases collected in the files of the Kiel Pediatric Tumor Registry. RMS: It is absolutely necessary to distinguish between embryonal (e) and alveolar (a) RMS, since these are two distinct tumor entities with significant differences in clinical presentation, morphology, molecular biology, cytogenetics and prognosis (Botryoid and spindle cell RMS are special variants of eRMS). The overall proportion of eRMS: aRMS is 2.4:1. Most cases of eRMS develop in the first 10 years of life (77.3%) while the age distribution of aRMS is almost constant in childhood and adolescence. Embryonal RMS exhibit a significantly higher proportion of male patients than aRMS (m:f = 1.72:1 vs. 1.06:1). A higher percentage of aRMS cases (25%) shows metastatic disease at the time of diagnosis than eRMS (8%), and the overall survival rates of aRMS are significantly lower even in localised disease (stage I-III) than in eRMS (59% vs. 76%; p < 0.002) (Data from the Cooperative Soft Tissue Sarcoma Study CWS). EOE: Despite proven histogenetic relationship (identical chromosomal rearrangements and fusion genes) the members of the Ewing's sarcoma (ES) family, classic ES and MPNT, whether osseous or extraosseous display significant differences in location, morphology and prognosis. Morphologically, MPNT and classic ES can be considered to be the extremes of a spectrum with overt neurodifferentiation in the former and lack of neural differentiation in the latter. Matched-pairs analysis of CWS EOE and MPNT cases show dramatically more unfavorable overall survival rates in MPNT than in EOE (45% vs. 67%).
横纹肌肉瘤（RMS）和骨外尤文肉瘤（EOE），包括恶性外周神经外胚层肿瘤（MPNT），是儿童期最常见的软组织恶性肿瘤。它们占基尔儿科肿瘤登记处档案中收集的2350例病例的60.2%。RMS：区分胚胎型（e）和肺泡型（a）RMS是绝对必要的，因为这是两种不同的肿瘤实体，在临床表现、形态学、分子生物学、细胞遗传学和预后方面存在显著差异（葡萄状和梭形细胞RMS是eRMS的特殊变体）。eRMS与aRMS的总体比例为2.4:1。大多数eRMS病例在生命的前10年发病（77.3%），而aRMS的年龄分布在儿童期和青少年期几乎是恒定的。胚胎型RMS的男性患者比例明显高于肺泡型RMS（男:女 = 1.72:1对1.06:1）。与eRMS（8%）相比，更高比例的aRMS病例（25%）在诊断时出现转移性疾病，即使在局限性疾病（I - III期）中，aRMS的总生存率也明显低于eRMS（59%对76%；p < 0.002）（数据来自软组织肉瘤协作研究CWS）。EOE：尽管已证实尤因肉瘤（ES）家族的成员，即经典ES和MPNT，无论骨内还是骨外，在组织发生学上存在关联（相同的染色体重排和融合基因），但它们在位置、形态学和预后方面仍存在显著差异。形态学上，MPNT和经典ES可被视为一个连续谱的两端，前者有明显的神经分化，后者缺乏神经分化。对CWS的EOE和MPNT病例进行配对分析显示，MPNT的总生存率明显低于EOE（45%对67%）。
