Zelger B G, Steiner H, Kutzner H, Maier H, Zelger B
Department of Pathology, University of Innsbruck, Austria.
Histopathology. 1998 May;32(5):414-22. doi: 10.1046/j.1365-2559.1998.00406.x.
Cellular neurothekeoma is a rare benign cutaneous neoplasm with conflicting opinions regarding its histogenetic origin (nerve sheath, smooth muscle, myofibroblasts) as well as its relation to myxoid neurothekeoma (nerve sheath myxoma). The present series describes 15 cases whose clinicopathological features indicate a relationship to dermatofibroma.
In this retrospective clinicopathological study, the 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. Immunohistochemically, all cases were positive with NK1C3 (CD57), KiM1p and proliferating cell nuclear antigen, seven were positive for neurone specific enolase, five for factor XIIIa, six for smooth muscle specific actin and three for E9, an antimetallothionein marker. These findings are similar to those of conventional 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 the data and photomicrographs from the literature reveals that in many cases similar conclusions could be reached. Obvious discrepancies are most likely due to the confusion with myxoid neurothekeoma, a well circumscribed, more spindly and myxoid, S100 positive lesion of Schwannian origin.
The appearance of dermatofibromas is markedly influenced by architectural, e.g. in deep penetrating dermatofibroma, and/or cellular/stromal criteria, e.g. in epithelioid cell histiocytoma or sclerosing dermatofibroma. Cellular neurothekeoma seems to be a variant of dermatofibromas with both architectural and cellular/stromal peculiarities, i.e. plexiform pattern, epithelioid cytology and stromal sclerosis.
细胞性神经鞘黏液瘤是一种罕见的皮肤良性肿瘤，关于其组织发生起源（神经鞘、平滑肌、肌成纤维细胞）以及与黏液样神经鞘黏液瘤（神经鞘黏液瘤）的关系存在相互矛盾的观点。本系列描述了15例病例，其临床病理特征表明与皮肤纤维瘤有关。
在这项回顾性临床病理研究中，病变好发于青少年至年轻成年人的上半身，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各种线索：棘层肥厚、边界不清的席纹状周边、周边明显硬化以及淋巴细胞分界/浸润。免疫组化方面，所有病例NK1C3（CD57）、KiM1p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例因子XIIIa阳性，6例平滑肌特异性肌动蛋白阳性，3例E9（一种抗金属硫蛋白标志物）阳性。这些发现与传统皮肤纤维瘤相似，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边界清楚、更呈梭形且黏液样、起源于施万细胞的S100阳性病变。
皮肤纤维瘤的外观明显受结构标准（如深部穿透性皮肤纤维瘤）和/或细胞/间质标准（如上皮样细胞组织细胞瘤或硬化性皮肤纤维瘤）的影响。细胞性神经鞘黏液瘤似乎是皮肤纤维瘤的一种变体，具有结构和细胞/间质方面的特殊性，即丛状模式、上皮样细胞学特征和间质硬化。
