Mertz Kirsten D, Mentzel Thomas, Grob Martin, Pfaltz Madeleine, Kempf Werner
Kempf & Pfaltz Histological Diagnostics, Zürich, Switzerland.
J Cutan Pathol. 2009 Nov;36(11):1210-4. doi: 10.1111/j.1600-0560.2009.01261.x.
Cellular neurothekeoma is a benign dermal lesion of uncertain differentiation. The atypical variant of cellular neurothekeoma, characterized by features such as large size, deep penetration, diffusely infiltrative borders, vascular invasion, high mitotic rate and marked cytologic pleomorphism, has been very rarely reported in the literature. We present the clinical, histopathological and immunohistochemical profile of a new case of atypical cellular neurothekeoma in a 68-year-old female patient who was diagnosed with ductal breast carcinoma in situ before. An erythematous painless papule arose in her right breast, and tentative clinical diagnosis was orientated toward skin metastasis of breast cancer or fibroma. Upon histological examination, the lesion showed the histological and immunohistochemical features of atypical variant of cellular neurothekeoma with high mitotic rate, marked cellular pleomorphism and penetration into subcutaneous fat. A further unusual feature in our patient was her age, because she is the oldest patient reported in the literature with diagnosis of atypical cellular neurothekeoma so far. Prognosis remains uncertain because only few cases of atypical cellular neurothekeoma have been reported in the literature, with a very limited follow-up time. Our report contributes to a better characterization of the clinical and morphologic features of atypical cellular neurothekeoma.
细胞性神经鞘黏液瘤是一种分化不明的良性皮肤病变。细胞性神经鞘黏液瘤的非典型变体，其特征为体积大、浸润深、边界弥漫性浸润、血管侵犯、高有丝分裂率和显著的细胞多形性等，在文献中报道极少。我们报告了一例68岁女性患者的非典型细胞性神经鞘黏液瘤新病例的临床、组织病理学和免疫组化特征，该患者之前被诊断为乳腺导管原位癌。她右乳出现一个无痛性红斑丘疹，初步临床诊断倾向于乳腺癌皮肤转移或纤维瘤。组织学检查显示，该病变具有细胞性神经鞘黏液瘤非典型变体的组织学和免疫组化特征，有高有丝分裂率、显著的细胞多形性并侵入皮下脂肪。我们患者的一个进一步不寻常特征是她的年龄，因为她是迄今为止文献报道中诊断为非典型细胞性神经鞘黏液瘤的最年长患者。由于文献中仅报道了少数非典型细胞性神经鞘黏液瘤病例，且随访时间非常有限，其预后仍不确定。我们的报告有助于更好地描述非典型细胞性神经鞘黏液瘤的临床和形态学特征。
