Participation of perineurial cells in peripheral nerve sheath tumors and FISH ananysis of their chromosomal abnormalities

为了阐明神经束膜细胞在周围神经鞘瘤（PNST）中的作用，进行了以下三项研究。本文报道了一种新的神经束膜恶性细胞瘤（malignant perimenurial cell tumor，MPNST），并对硬化性神经束膜瘤进行了病理学观察，同时对神经纤维瘤中的神经束膜细胞进行了免疫组化染色。选择23例梭形细胞瘤，其长突起呈螺旋状或故事状排列，具有良性神经束膜瘤的典型特征。免疫组化（EMA + / S-100 -）显示23例肿瘤中有5例神经束膜MPNST。因此，4%的MPNST（121例中的5例）显示神经束膜细胞分化。一个NE-1协会尚未被描述。神经受累不常见。其免疫表型（EMA + / S-100 -）常提示 ...更多信息 超微结构神经束膜分化。石蜡包埋标本FISH检测未见着丝粒信号。2.硬化性神经束膜瘤硬化性神经束膜瘤是一种新的软组织神经束膜瘤亚型，好发于手部真皮和皮下组织。显微镜下，肿瘤由致密间质中的梭形或上皮样细胞组成，常呈索状或簇状排列。大多数肿瘤细胞EMA阳性，而它们缺乏S-100蛋白的免疫反应性。超微结构研究清楚地显示了神经束膜细胞的特征。3.不同类型周围神经鞘瘤中的神经束膜细胞为了证实神经束膜细胞参与了PNST，我们应用了一种高度敏感的免疫组化方法--催化信号放大（CSA）法，对不同类型的肿瘤进行了研究。结果发现神经纤维瘤中有许多EMA阳性的梭形细胞，表明神经束膜细胞是这类肿瘤的主要成分之一。神经鞘瘤被EMA阳性的神经束膜包围。少

To clarify the role of perineurial cells in peripheral nerve sheath tumors (PNST), the following three studies were carried out. A new entity, malignant perineurial cell tumor, was proposed ; scierosing perineurioma was pathologically characterized ; and the presence of perineurial cells was immunohistochemically revealed in neurofibromas.1.Perineurial malignant peripheral nerve sheath tumor (MPNST)To determine the clinicopathologic, immunohistochemical and ultrastructural characteristics of perineurial MPNST, 121 MPNST were examined ; 23 spindle cell tumors with long processes disposed in whorls or storiform patterns, features typical of benign perineurioma, were selected. On the basis of immunohistochemistry (EMA + / S-100 -), 5 perineurial MPNST were found in 23 tumors selected. Therefore, 4% of MPNST (5 of 121) show perineurial cell differentiation. An NE-1 association has yet to be described. Nerve involvement is infrequent. Their immunophenotype (EMA + / S-100 -) frequently indic … More ates ultrastructural perineurial differentiation. The prognosis of perineurial MPNST appears to be more favorable than that of conventional MPNST.FISH analyses using paraffin embedded materials failed to show centromere signals.2.Sclerosing perineuriomaScierosirig perineurioma, a new subtype of soft part perineurioma, frequently arises in the dermis and subcutis of the hands. Microscopically, tumors consisted of spindle-shaped or epithelioid cells in a dense stroma, often arranged in cords or clusters. Most tumor cells were positive for EMA, whereas they lacked immunoreactivity for S-100 protein. Ultrastructural studies clearly showed perineunal cell characteristics.3.Perineurial cells in varied peripheral nerve sheath tumorsTo demonstrate the participation of perineurial cells in PNST, the catalyzed signal amplification (CSA) method, a highly sensitive immunohistochemical method, was applied to varied tumors. In result, many EMA-positive spindle-shaped cells were identified in neurofibromas, indicating that the perineurial cells are one of principal constituents of this type of tumor. Schwannomas were surrounded by EMA-positive perineurium. Less