Southern California Permanente Medical Group, Department of Pathology, Orange County-Anaheim Medical Center, Anaheim, California, USA.
Southern California Permanente Medical Group, Department of Pathology, Los Angeles Medical Center, Los Angeles, California, USA.
J Cutan Pathol. 2021 Jul;48(7):980-985. doi: 10.1111/cup.14025. Epub 2021 May 9.
Cellular neurothekeoma is a cutaneous tumor with a distinctive histopathologic appearance characterized by a dermal-based multinodular proliferation of epithelioid to spindled cells. Although the tumor may show varying amounts of myxoid stroma, extensive myxoid change is uncommon. The tumor typically presents as a solitary nodule with a predilection for the head and neck and upper limbs; examples of multiple cellular neurothekeomas are decidedly rare. The present report describes a unique case of multiple myxoid cellular neurothekeomas arising in a 60-year-old female with systemic lupus erythematosus. Two papular lesions were identified involving the skin inferior to the umbilicus and the left inguinal crease. Both lesions were histopathologically similar, forming a nodular mass composed of epithelioid cells in a prominent myxoid stroma. By immunohistochemistry the lesional cells expressed NKI/C3, microphthalmia transcription factor (MiTF), and CD68, with focal staining for PGP9.5, factor XIIIa, and CD10 also observed. The tumors were negative for S-100, SOX-10, epithelial membrane antigen, desmin, smooth muscle actin, glial fibrillary acid protein, and CD34. The present case confirms that cellular neurothekeoma can present clinically as multiple lesions and can have a predominantly myxoid appearance, potentially mimicking other cutaneous myxoid lesions.
细胞神经鞘瘤是一种具有独特组织病理学表现的皮肤肿瘤，其特征为真皮基底的多结节状上皮样至梭形细胞增生。尽管肿瘤可能表现出不同程度的黏液样基质，但广泛的黏液样改变并不常见。该肿瘤通常表现为单个结节，好发于头颈部和上肢；多灶性细胞神经鞘瘤的病例则非常罕见。本报告描述了一例 60 岁女性系统性红斑狼疮患者发生的多发性黏液样细胞神经鞘瘤。该患者存在两个丘疹性皮损，分别位于脐以下皮肤和左侧腹股沟褶皱。两个皮损的组织病理学表现相似，形成一个由上皮样细胞组成的结节状肿块，伴有明显的黏液样基质。免疫组化染色显示病变细胞表达 NKI/C3、小眼畸形转录因子（MiTF）和 CD68，也可见局灶性 PGP9.5、因子 XIIIa 和 CD10 染色。该肿瘤 S-100、SOX-10、上皮膜抗原、结蛋白、平滑肌肌动蛋白、胶质纤维酸性蛋白和 CD34 均为阴性。本病例证实，细胞神经鞘瘤可表现为多个临床病变，并可具有主要的黏液样外观，可能模拟其他皮肤黏液样病变。
