Zelger B G, Zelger B
Institut für Pathologie, Universitätsklinik für Dermatologie, Universität Innsbruck, Osterreich.
Verh Dtsch Ges Pathol. 1998;82:239-45.
The present series describes 15 cellular neurothekeomas whose clinicopathologic features indicate a close relationship to dermatofibroma.
Retrospective clinicopathologic study.
Lesions preferentially occurred in adolescents to young adults on the upper half of the body, often clinically diagnosed as some kind of fibrohistiocytic tissue response. Besides characteristic whorled nests to fascicles of palely eosinophilic epithelioid cells all lesions showed variable clues pointing to dermatofibroma: acanthosis, ill-defined storiform periphery, peripherally accentuated prominent sclerosis and lymphocytic demarcation/infiltration. All cases were positive with NK1C3 (CD 57), Ki-M1p and proliferating cell nuclear antigen, 7 for neuron specific enolase, 5 for factor XIIIa, 6 for smooth muscle actin and 3 with E9, an anti-metallothionein marker. These findings are similar to other types of dermatofibromas, the variability of the profile being best explained by time cycle and function dependent changes. Ultrastructurally, two cases showed microfilaments, attachment plaques, prominent pinocytosis and focal remnants of basal lamina. A careful study of data and microphotographs from the literature reveals that in many cases similar conclusions can be reached. Obvious discrepancies are most likely due to the confusion with myxoid neurothekeoma, a well circumscribed, more spindly and myxoid, S 100 positive lesion of Schwannian origin.
According to our results cellular neurothekeoma seems to be a whorled-nested to plexiform epithelioid variant of dermatofibroma.
本系列报道了15例细胞性神经鞘黏液瘤，其临床病理特征表明与皮肤纤维瘤关系密切。
回顾性临床病理研究。
病变好发于青少年至青年的上半身，临床上常被诊断为某种纤维组织细胞性组织反应。除了具有特征性的淡嗜酸性上皮样细胞呈漩涡状巢状至束状排列外，所有病变均显示出指向皮肤纤维瘤的不同线索：棘层肥厚、边界不清的席纹状周边、周边显著硬化以及淋巴细胞分界/浸润。所有病例NK1C3（CD 57）、Ki-M1p和增殖细胞核抗原均呈阳性，7例神经元特异性烯醇化酶阳性，5例凝血因子ⅩⅢa阳性，6例平滑肌肌动蛋白阳性，3例E9（一种抗金属硫蛋白标志物）阳性。这些发现与其他类型的皮肤纤维瘤相似，其特征的变异性最好用时间周期和功能依赖性变化来解释。超微结构上，2例显示有微丝、附着斑、明显的胞饮作用和基底膜的局灶性残余。对文献中的数据和显微照片进行仔细研究发现，在许多情况下可以得出类似的结论。明显的差异很可能是由于与黏液样神经鞘黏液瘤混淆所致，黏液样神经鞘黏液瘤是一种界限清楚、更呈梭形且黏液样、起源于施万细胞的S 100阳性病变。
根据我们的结果，细胞性神经鞘黏液瘤似乎是皮肤纤维瘤的一种漩涡状巢状至丛状上皮样变体。
