Barnhill R L, Dickersin G R, Nickeleit V, Bhan A K, Muhlbauer J E, Phillips M E, Mihm M C
Dermatopathology Division, Massachusetts General Hospital, Boston.
J Am Acad Dermatol. 1991 Jul;25(1 Pt 1):80-8. doi: 10.1016/0190-9622(91)70177-4.
The clinical, histopathologic, and immunohistochemical features of 11 cases of neurothekeoma are reported. One case was examined by electron microscopy. The mean age of the patients was 27.1 years; the study comprised eight female and three male patients. Most lesions were nondescript papules and located on the upper part of the body, seven cases of neurothekeoma on the head. Eight cases were classified as cellular neurothekeoma on the basis of a striking fascicular pattern and three cases as myxomatous neurothekeoma because of prominent myxoid stromal change. All cellular neurothekeomas failed to express S-100 protein, whereas the three myxomatous types were strongly positive for this marker. Other than vimentin, there was no significant immunoreactivity with other immunohistochemical markers. Ultrastructural study of one case of cellular neurothekeoma was inconclusive for cell type although a perineurial origin could not be excluded. On the basis of these results, we conclude that cellular neurothekeoma differs from myxomatous neurothekeoma not only by clinical and histologic findings but also by immunoreactivity with S-100 protein. These findings also suggest the existence of two distinct subtypes of neurothekeoma and possible origin of the two variants of neurothekeoma from different cell types or at least variation in phenotypic expression of a common cell type. On the other hand, it cannot be excluded that these two variants are different stages in the natural history of neurothekeoma.
报告了11例神经鞘黏液瘤的临床、组织病理学和免疫组化特征。1例进行了电镜检查。患者的平均年龄为27.1岁；该研究包括8例女性和3例男性患者。大多数病变为无特征性丘疹，位于身体上部，7例神经鞘黏液瘤位于头部。8例根据显著的束状模式被分类为细胞性神经鞘黏液瘤，3例因显著的黏液样基质改变而被分类为黏液样神经鞘黏液瘤。所有细胞性神经鞘黏液瘤均未表达S-100蛋白，而3例黏液样类型对此标志物呈强阳性。除波形蛋白外，与其他免疫组化标志物无明显免疫反应性。1例细胞性神经鞘黏液瘤的超微结构研究虽不能排除神经束膜起源，但对细胞类型的结论不明确。基于这些结果，我们得出结论，细胞性神经鞘黏液瘤与黏液样神经鞘黏液瘤的不同不仅在于临床和组织学表现，还在于与S-100蛋白的免疫反应性。这些发现还提示神经鞘黏液瘤存在两种不同的亚型，且两种神经鞘黏液瘤变体可能起源于不同的细胞类型，或至少是同一细胞类型表型表达的差异。另一方面，不能排除这两种变体是神经鞘黏液瘤自然病程中的不同阶段。
