PERIPHERAL PRIMITIVE NEUROECTODERMAL TUMOR AND EXTRA-OSSEOUS EWINGS-SARCOMA - A HISTOLOGICAL, IMMUNOHISTOCHEMICAL AND DNA FLOW CYTOMETRIC STUDY
PERIPHERAL PRIMITIVE NEUROECTODERMAL TUMOR AND EXTRA-OSSEOUS EWINGS-SARCOMA - A HISTOLOGICAL, IMMUNOHISTOCHEMICAL AND DNA FLOW CYTOMETRIC STUDY
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DOI：
10.1007/bf00199351
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发表时间：
1995-01-01
期刊：
影响因子：
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通讯作者：
MEIJER, CJLM
中科院分区：
文献类型：
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作者：
BRINKHUIS, M;WIJNAENDTS, LCD;MEIJER, CJLM
虽然周围原始神经外胚层肿瘤（pPNET）和骨外尤文氏肉瘤（EES）被认为是密切相关的肿瘤，但它们的临床行为有很大不同。为了确定区分pPNET和EES的Schmidt分类方案的临床相关性，我们对20例低分化圆形细胞肿瘤标本进行了评估。此外，还评估了几种神经标志物的诊断价值和定量形态学变量（DNA倍性、s期分数和有丝分裂活性）的预后价值。9个肿瘤中存在霍默-莱特玫瑰花结。神经元特异性烯醇化酶（NSE）在11个肿瘤中表达，其中8个肿瘤表达第二神经标志物（CD57、S100或神经丝）。根据Schmidt分类，区分出11个pPNET和5个EES。HBA-71仅在pPNET和EES中表达。其余肿瘤被分类为未明确的肉瘤（n = 2）、横纹肌肉瘤（n = 1）和分化分化的结缔组织增生瘤（n = 1）。EES611患者的5年生存率明显优于pPNET患者（100% vs 42%）。12个评估直方图（9个pPNET和3个EES）评估的DNA倍性和s期分数，以及有丝分裂活性都不能提供额外的预后价值信息。根据本研究和Schmidt分类方案，可以得出结论，如果EES和pPNET的诊断是基于光镜（Homer-Wright玫瑰花）和/或免疫组织化学（至少两种神经标记物，即NSE， S-100， CD57和神经丝），则该分类提供了重要的临床信息。此外，hb -71阳性有助于将pPNET和EES与其他小圆细胞肿瘤区分开来。
Although peripheral primitive neuroectodermal tumour (pPNET) and extra-osseous Ewing's sarcoma (EES) are thought to be closely related neoplasms, their clinical behaviour differs considerably. To determine the clinical relevance of the Schmidt classification scheme for differentiating pPNET and EES, 20 tumour specimens of poorly differentiated round cell tumours were evaluated. In addition, the diagnostic value of several neural markers and the prognostic value of quantitative morphological variables (DNA ploidy, S-phase fraction, and the mitotic activity) were assessed. Homer-Wright rosettes were present in 9 tumours. Neuron specific enolase (NSE) was expressed in 11 tumours, 8 of which expressed a second neural marker (CD57, S100, or neurofilament). According to the Schmidt classification, 11 pPNET and 5 EES were distinguished. HBA-71 was exclusively expressed in pPNET and EES. The remaining tumours were classified as sarcoma not otherwise specified (n = 2), rhabdomyosarcoma (n = 1), and desmoplastic tumour with divergent differentiation (n = 1). EES611 patients fared significantly better than the pPNET patients (100% versus 42% 5-year survival). Neither DNA ploidy nor S-phase fraction assessed in 12 evaluative histograms (9 pPNET and 3 EES), nor mitotic activity yielded information of additional prognostic value. On the basis of this study and the Schmidt classification scheme, it can be concluded that if the diagnosis of EES and pPNET is based on light microscopy (Homer-Wright rosettes) and/or immunohistochemistry (at least two neural markers, i.e. NSE, S-100, CD57, and neurofilament), the classification provides important clinical information. Furthermore, positivity for HBA-71 is helpful in differentiating pPNET and EES from all other small round cell tumours.