男，9个月，发现左前臂包块8个月入院。

术中所见：包块边界尚清，血供一般。

大体所见：带皮肤组织一块，2.5cmx1cmx1cm，切面暗红、实性。

也考虑神经束膜瘤

很像神经束膜瘤。

神经痣

这一例似乎应该考虑巨细胞血管母细胞瘤。结节状、中央为小血管，血管周围为同心圆状、洋葱皮样排列，可见多核细胞，年龄幼小。

学习了，谢谢

肌性周细胞瘤

病变由相对单形性的椭圆及梭形细胞构成，多层排列在小血管周围呈明显的同心圆状生长。

非常感谢TK1905老师的精彩点评，也敬佩老师的专业精神，从这例中学习了很多！TK1905老师的意见我会虚心接纳。

非常感谢TK1905老师的精彩点评，也敬佩老师的专业精神，从这例中学习了很多！TK1905老师的意见我会虚心接纳。

学习了

多积累病例，做足工作，注意随访，最后新的WHO分类里说不定就是您的观点。

Differential gene expression profiles of neurothekeomas and nerve sheath myxomas by microarray analysis

Abstract presented at the United States and Canadian Academy of Pathology 99th Annual Meeting, Washington, DC, 2010.

Sachiv Sheth¹, Xinmin Li¹, Scott Binder¹ and Sarah M Dry¹

¹Department of Pathology and Laboratory Medicine, David Geffen School of Medicine, University of California Los Angeles, Los Angeles, CA, USA

Abstract

Neurothekeomas and dermal nerve sheath myxomas have previously been considered related cutaneous neoplasms of peripheral nerve sheath origin based on light microscopic similarities. However, recent immunohistochemical and ultrastructural data indicate nerve sheath myxomas exhibit true nerve sheath differentiation, whereas no such compelling evidence exists for neurothekeomas. Although neurothekeomas lack a specific immunohistochemical profile, similar antigen expression and histopathologic patterns suggest neurothekeomas may be categorized as fibrohistiocytic tumors. To date, no known molecular studies have examined the histogenetic relationship of these tumors. We report the first microarray-based gene expression profile study of these entities on formalin-fixed paraffin-embedded tissues. Cases of dermal schwannomas, dermal nerve sheath myxomas, myxoid/mixed/cellular neurothekeomas, and cellular fibrous histiocytomas diagnosed in the past 3 years were identified in our database. Archival formalin-fixed paraffin-embedded tissue from 28 patients was selected for microarray analysis (seven schwannomas, five nerve sheath myxomas, nine myxoid/mixed/cellular neurothekeomas and seven cellular fibrous histiocytomas). Following tumor RNA isolation, amplification and labeling using commercially available kits, labeled targets were hybridized to the Affymetrix GeneChip Human Genome U133 Plus 2.0 Array (Santa Clara, CA, USA). Acquisition of array images and data analyses was performed using appropriate software. Hierarchical clustering and principal component analysis demonstrated discrete groups, which correlated with histopathologically identified diagnoses. Dermal nerve sheath myxomas demonstrate very similar molecular genetic signatures to dermal schwannomas, whereas neurothekeomas of all subtypes more closely resemble cellular fibrous histiocytomas. We are the first to report distinct gene expression profiles for nerve sheath myxomas and neurothekeomas, which further substantiates the argument that these are separate entities. Our molecular data confirms that dermal nerve sheath myxomas are of peripheral nerve sheath origin, and suggests that neurothekeomas may actually be a variant of fibrous histiocytomas.

 Weiss Enzinger soft tissue tumors:

Cellular neurothekeoma

In 1990 Barnhill and Mihm described the entity of “cellular neurothekeoma” and noted that, unlike classic neurothekeoma, it consistently lacked S-100 protein, although this point has recently been contested.Since that time, its similarity to classic neurothekeoma has been debated and some have considered it an epithelioid form of pilar leiomyoma. In our opinion, the lesions that have been described as cellular neurothekeoma may well represent more than a single entity. Some probably represent classic neurothekeomas which lack a myxoid background and therefore appear cellular, whereas others may represent other tumor(s) altogether. The recent report that MiTF and NK1/3 (melanocytic markers) are strongly expressed in cellular neurothekeomas in the absence of S-100 protein and with the occasional presence of actin has led to the interesting proposal that the lesion might represent a member of the PEComa family  

美国外科病理学杂志：2009年6月 33卷 6期 905-913页
神经鞘黏液瘤和丛状纤维组织细胞性肿瘤：两者仅是形态相似还是其组织发生有关联？

摘要：
       神经鞘黏液瘤（NTK）和丛状纤维组织细胞性肿瘤（PFHT）好发于皮肤，两者的临床特点和组织学特征有许多相同之处，但两者的组织发生仍具争议。我们分析了43例NTK和18例PFHT的形态特征和免疫表型，从而评价两者之间是否存在某种关系。基于黏液样基质的量，我们将这43例NTK分成：8例黏液样神经鞘黏液瘤（MyNTK）、15例混合性神经鞘黏液瘤（MiNTK）和20例富于细胞性神经鞘黏液瘤（CNTK）。MyNTK和MiNTK的病变界限很清楚，由梭形细胞组成。CNTK呈浸润性生长，主要由上皮样细胞组成，超过50%的病例可见多核巨细胞。这18例PFHT中的11例主要由上皮样细胞组成，1例由梭形细胞组成，另外6例由以上两种细胞等量混合而成。本研究中所有其他病例的形态特征都与CNTK有重叠，尤其是那些富于上皮样细胞的病例。免疫组化：8/8例MyNTK、4/15例MiNTK、1/19例CNTK和0/18例PFHT表达S100；0/8例MyNTK、6/15例MiNTK、9/17例CNTK和6 /11例PFHT表达CD68。这些结果表明，MyNTK和MiNTK存在连续性。此外，CNTK和PFHT的形态特征、免疫表型显著相似，说明这两者组织发生相同，而MyNTK和MiNTK的组织发生则不同。 

我找到的一些图片，很多是美国外科病理学杂志的：

向TK1905老师特别肯钻研的精神致敬！！！

Neurothekeoma（神经鞘瘤黏液瘤）分为经典型（即Nerve sheath myxoma）和细胞型（即Neurothekeoma）。经典型（即Nerve sheath myxoma）和 细胞型（即Neurothekeoma） 的电镜超微结构及免疫组化完全不同，前者是具有施万细胞分化特点，免疫组化S100阳性也支持是神经源性，故神经鞘黏液瘤应为Nerve sheath myxoma，而不应包括Neurothekeoma。细胞型（即Neurothekeoma）不论在电镜超微结构或是免疫组化均显示源于（肌）纤维母细胞，免疫组化不表达S100、PGP9.5及GFAP等，可表达NKI-C3、Mitf等，或许不久细胞型（即Neurothekeoma）就会从神经鞘黏液瘤中分出去，而成为单独的一种肿瘤。

今天在啃这个病看了好几篇文献，这个例子形态学不是很像，因为Celluar Neurothekeoma长得就像丛状纤维组织细胞瘤，而且现在最新的文献证实它们极有可能就是一种实体，至少在临床病理学及遗传学上都有很大的相似性，它不是神经鞘源性已经明确，这个名字可能不能再叫做神经鞘粘液瘤了，神经鞘粘液瘤应该由Nerve Sheath Myxoma专门担当。IHC做得不够，NKI-C3、SMA、CD68可以做下。有时间我可以上传一些典型图片，本例不除外其它诊断。纯学术交流，不是挑刺，望楼主见谅

承蒙TK1905老师的点评。Neurothekeoma小叶内的粘液含量明显少于Nerve sheath myxoma，部分肿瘤含有少量的粘液或基本上不含粘液，主要由上皮样细胞组成，瘤细胞可成小叶状或微小叶状分布，核多深染，此型也称富于细胞性神经鞘粘液瘤（cellular  Neurothekeoma）。

Neurothekeoma中的瘤细胞具有纤维母细胞分化，部分具有肌纤维母细胞分化，故不表达S-100蛋白、PGP9.5，但可表达NKI-C3、CD10、NSE。  而Nerve sheath myxoma中的瘤细胞具有施万细胞分化，故表达S-100蛋白。

真的是这个肿瘤吗？

Weiss%Enzinger软组织第5版把neurothekeoma分为2个，一个叫经典的，一个叫细胞性，结合本例S-100-，无明显多结节状粘液，应该归在细胞性神经鞘粘液瘤这个范畴里面。

没见过，学习，谢谢！

Neurothekeoma

神经鞘粘液瘤。谢谢！