Smith K J, Mezebish D, Williams J, Elgart M L, Skelton H G
Departments of Dermatology and Pathology, National Naval Medical Center, Bethesda, MD, USA.
Ann Diagn Pathol. 1998 Aug;2(4):213-23. doi: 10.1016/s1092-9134(98)80010-5.
Neurocristic cutaneous hamartomas result from aberrant development of the neuromesenchyme. Thus, the elements within these tumors reflect the spectrum of differentiation that results from migration of neural crest-derived cells. We present three cases, in addition to routine hematoxylin-eosin staining, a battery of immunohistochemical staining, including S-100 protein, HMB-45, EMA, CD34, and neurofilament-protein stains, was performed on each specimen. A dermal melanocytic component was the dominant feature of two of these lesions, while neurosusenticular and neuromesenchymal components dominated in one tumor. Both tumors that developed on the scalp showed effects on the overlying epidermis and/or on adnexal development. The melanocytic component was positive for S-100 protein and HMB-45. The surrounding stroma showed tactoid bodies and increased CD34 staining. Neurocristic cutaneous hamartomas represent dysplastic development of neural crest-derived cells. Although melanocytic cells have been previously reported to be the dominant cell population, neurosusentacular and neuromesenchymal cells also may be the principal component. In cephalic areas, the neuromesenchyme may not only be an important component of the tumor, but may also effect the development of the overlying epithelium and adnexal structures. Although none of these cases presents evidence of malignant transformation, identification of these tumors could be important if malignant transformation results in the development of tumors with a distinctive biologic behavior.
神经嵴性皮肤错构瘤由神经间充质的异常发育引起。因此，这些肿瘤中的成分反映了神经嵴衍生细胞迁移所导致的分化谱。我们报告三例病例，除常规苏木精-伊红染色外，对每个标本进行了一系列免疫组织化学染色，包括S-100蛋白、HMB-45、EMA、CD34和神经丝蛋白染色。真皮黑素细胞成分是其中两个病变的主要特征，而神经感觉和神经间充质成分在一个肿瘤中占主导。发生在头皮上的两个肿瘤均显示对上方表皮和/或附属器发育有影响。黑素细胞成分对S-100蛋白和HMB-45呈阳性。周围间质显示触觉小体和CD34染色增加。神经嵴性皮肤错构瘤代表神经嵴衍生细胞的发育异常。尽管先前有报道黑素细胞是主要细胞群，但神经感觉细胞和神经间充质细胞也可能是主要成分。在头部区域，神经间充质不仅可能是肿瘤的重要成分，还可能影响上方上皮和附属器结构的发育。尽管这些病例均未呈现恶性转化的证据，但如果恶性转化导致具有独特生物学行为的肿瘤发生，识别这些肿瘤可能很重要。
