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

为了阐明周围神经鞘细胞在周围神经鞘肿瘤（PNST）中的作用，我们进行了以下三项研究。提出了一种新的肿瘤类型：恶性神经周围细胞瘤；横切性神经周围瘤具有病理特征；神经纤维瘤免疫组织化学显示神经周围细胞的存在。为了解恶性周围神经鞘瘤（MPNST）的临床病理、免疫组织化学及超微结构特征，对121例MPNST进行了检查；选择23例梭形细胞瘤，其长突呈螺旋状或故事状，具有良性会阴瘤的典型特征。经免疫组化（EMA + / S-100 -）检查，23例肿瘤中发现5例神经周围MPNST。因此，4%的MPNST（121例中的5例）显示神经周围细胞分化。NE-1的关联尚未被描述。神经受累是罕见的。他们的免疫表型（EMA + / S-100 -）经常显示神经周围的超微结构分化。神经周围MPNST的预后似乎比常规MPNST更有利。使用石蜡包埋材料的FISH分析未能显示着丝粒信号。硬化性骨膜瘤是一种新的软部骨膜瘤亚型，常见于手部真皮和皮下。显微镜下，肿瘤由密集基质中的梭形或上皮样细胞组成，通常呈索状或簇状排列。大多数肿瘤细胞对EMA呈阳性，而对S-100蛋白缺乏免疫反应性。超微结构检查清楚显示会阴细胞的特征。为了证明周围神经鞘肿瘤中有周围神经细胞参与PNST，我们采用高灵敏度的免疫组织化学方法——催化信号放大（CSA）法对多种肿瘤进行了检测。结果，在神经纤维瘤中发现了许多ema阳性的纺锤形细胞，表明神经周围细胞是这类肿瘤的主要成分之一。神经鞘瘤周围为ema阳性的神经膜。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