Calonje E, Wilson-Jones E, Smith N P, Fletcher C D
Department of Histopathology, St Thomas's Hospital (UMDS), London, UK.
Histopathology. 1992 May;20(5):397-404. doi: 10.1111/j.1365-2559.1992.tb01009.x.
Cellular neurothekeoma is a recently recognized benign cutaneous neoplasm, which is currently regarded as being of nerve sheath origin and is thought to represent a variant of conventional neurothekeoma (dermal nerve sheath myxoma). Nine new cases presenting predominantly in adolescents or young adults are described. Morphologically they were characterized by short fascicles or small nests of palely eosinophilic epithelioid or spindle-shaped cells which ramified in an ill-defined manner between dermal collagen bundles. Myxoid matrix was absent or sparse. Scattered normal mitoses and multinucleate giant cells were often present. Immunohistochemically all nine cases were strongly NK1/C3 positive, seven were weakly NSE positive and three were smooth muscle actin positive. Staining for S-100 protein, PGP 9.5, epithelial membrane antigen and desmin was negative in all cases. In view of its distinctive architecture and immunophenotype, both of which are totally different from conventional neurothekeoma, it is proposed that cellular 'neurothekeoma' is a separate discrete entity which may represent an epithelioid variant of pilar leiomyoma.
细胞性神经鞘黏液瘤是一种最近才被认识的良性皮肤肿瘤，目前被认为起源于神经鞘，被认为是传统神经鞘黏液瘤（真皮神经鞘黏液瘤）的一种变体。本文描述了9例主要发生在青少年或年轻成年人中的新病例。其形态学特征为短束状或小巢状淡嗜酸性上皮样或梭形细胞，在真皮胶原束之间呈不明确的分支状。黏液样基质缺乏或稀少。常可见散在的正常有丝分裂象和多核巨细胞。免疫组化显示，所有9例均为NK1/C3强阳性，7例为NSE弱阳性，3例为平滑肌肌动蛋白阳性。所有病例S-100蛋白、PGP 9.5、上皮膜抗原和结蛋白染色均为阴性。鉴于其独特的结构和免疫表型，两者均与传统神经鞘黏液瘤完全不同，因此提出细胞性“神经鞘黏液瘤”是一个独立的实体，可能代表毛发平滑肌瘤的上皮样变体。
