Barnhill R L, Mihm M C
Dermatopathology Division, Massachusetts General Hospital, Boston 02114.
Am J Surg Pathol. 1990 Feb;14(2):113-20. doi: 10.1097/00000478-199002000-00002.
We describe the clinical, histopathologic, and immunohistochemical characteristics of five examples of a distinctive subtype of neurothekeoma we term "cellular neurothekeoma" (CNT). These lesions are nondescript papules or nodules primarily involving the head and neck areas of young adults. Histopathologically, CNT are fairly well-defined proliferations involving the reticular dermis; they consist of fascicles of polygonal and spindle cells with eosinophilic or pale-staining cytoplasm and neuroid characteristics. Low-grade cytologic atypia and mitotic activity are common. All immunohistochemical markers--including S-100 protein, myelin basic protein, epithelial membrane antigen, and histiocytic antigens--have failed to show positivity in our laboratory. Separation from myxomatous variants of neurothekeoma is based on greater cellularity, less myxomatous change, and less pronounced plexiform compartmentalization by fibrous septae, which resemble perineurium. The differential diagnosis usually includes spindle and epithelioid cell (Spitz) nevus, malignant melanoma (particularly desmoplastic-neurotropic melanoma), cellular blue nevus, and fibrohistiocytic proliferations. The recognition of CNT and its differentiation from melanoma are important so that overly aggressive therapy is avoided.
我们描述了一种独特的神经鞘黏液瘤亚型——我们称之为“细胞性神经鞘黏液瘤”（CNT）的5个病例的临床、组织病理学和免疫组化特征。这些病变为无特征性丘疹或结节，主要累及年轻成人的头颈部区域。组织病理学上，CNT是累及网状真皮的边界相对清晰的增生性病变；由多边形和梭形细胞束组成，细胞质呈嗜酸性或淡染，具有神经样特征。低度细胞异型性和有丝分裂活性常见。在我们实验室，所有免疫组化标记物——包括S-100蛋白、髓磷脂碱性蛋白、上皮膜抗原和组织细胞抗原——均未显示阳性。与黏液瘤样神经鞘黏液瘤的鉴别基于细胞更丰富、黏液样改变更少以及由类似神经束膜的纤维间隔形成的丛状分隔不明显。鉴别诊断通常包括梭形和上皮样细胞（斯皮茨）痣、恶性黑色素瘤（尤其是促纤维组织增生性神经侵袭性黑色素瘤）、细胞性蓝色痣以及纤维组织细胞增生。认识CNT并将其与黑色素瘤区分开来很重要，这样可以避免过度积极的治疗。
