Michal Michael, Kazakov Dmitry V, Agaimy Abbas, Hosova Marta, Michalova Kvetoslava, Grossmann Petr, Steiner Petr, Skenderi Faruk, Vranic Semir, Michal Michal
Department of Pathology, Faculty of Medicine in Pilsen, Charles University, Alej Svobody 80, 304 60 Pilsen, Czech Republic; Biomedical Center of the Faculty of Medicine in Pilsen, Alej Svobody 80, 304 60 Pilsen, Czech Republic.
Department of Pathology, Faculty of Medicine in Pilsen, Charles University, Alej Svobody 80, 304 60 Pilsen, Czech Republic.
Ann Diagn Pathol. 2017 Apr;27:74-78. doi: 10.1016/j.anndiagpath.2017.02.001. Epub 2017 Feb 2.
The authors present a distinctive perineurioma (PN) variant which morphologically strongly resembles monophasic fibrous synovial sarcoma (MSS). The patients were 3 males and 1 female. The age ranged from 15 to 61years (mean: 44years). Locations included the sole, lower jaw, palm and foot. The tumor size ranged from 1.3cm to 2.5cm in the largest dimension (mean 1.8cm). Morphologically, all tumors had an identical, monotonous appearance. The perineurial cells were closely packed and created a confluent cellular whorls and/or sheets in a scarce stroma, with only focally discernible long, slender cytoplasmic processes typical for perineurial differentiation. The nuclei were rounded or slightly elongated to tapered, without nuclear atypia. Mitoses were rare to completely absent. Atypical mitoses, hemorrhage, necrosis or calcifications were not present. The proliferative index (Ki-67) was 1-3%. All analyzed tumors were positive for EMA, Claudin-1, GLUT-1 and negative with S100 protein, CD34, OSCAR, CK7 and TLE-1. Two cases were tested by fluorescence in situ hybridization and neither showed alterations of the SYT gene. One case studied by electron microscopy showed characteristic features of perineurial differentiation. Follow-up was available for two patients both of which showed no evidence of disease at 8years and 6months, respectively. Based on their bland morphology, perineurial features and presumably benign clinical outcome we propose the term "whorling cellular perineurioma" for these tumors, which may represent an extremely cellular variant of sclerosing PN. Awareness of this PN subtype and its distinction from MSS is of utmost clinical significance.
作者报告了一种独特的神经束膜瘤（PN）变体，其形态学上与单相纤维性滑膜肉瘤（MSS）极为相似。患者为3名男性和1名女性。年龄范围为15至61岁（平均44岁）。肿瘤部位包括足底、下颌、手掌和足部。肿瘤最大径为1.3cm至2.5cm（平均1.8cm）。形态学上，所有肿瘤外观相同，表现单一。神经束膜细胞紧密排列，在稀少的间质中形成融合的细胞漩涡和/或细胞片，仅局灶性可见典型的神经束膜分化的细长胞质突起。细胞核圆形或略呈细长形至锥形，无核异型性。有丝分裂罕见或完全缺如。未见非典型有丝分裂、出血、坏死或钙化。增殖指数（Ki-67）为1%-3%。所有分析的肿瘤EMA、Claudin-1、GLUT-1均呈阳性，而S100蛋白、CD34、OSCAR、CK7和TLE-1均为阴性。2例患者进行了荧光原位杂交检测，均未显示SYT基因改变。1例患者进行了电子显微镜检查，显示出神经束膜分化的特征性表现。2例患者有随访资料，分别在8年和6个月时均无疾病证据。基于其温和的形态学、神经束膜特征以及可能的良性临床转归，我们建议将这些肿瘤命名为“漩涡状细胞神经束膜瘤”，其可能代表硬化性PN的一种细胞极度丰富的变体。认识到这种PN亚型及其与MSS的鉴别具有极其重要的临床意义。
