Toussaint-Caire Sonia, Aguilar-Donis Adriana, Torres-Guerrero Edoardo, Asz-Sigall Daniel, Vega-Memije María Elisa, Lacy-Niebla Rosa María, Domínguez-Cherit Judith, Alfaro Ledesma Patricia, Ortiz-Hidalgo Carlos
Servicio de Dermatología y Dermatopatología, Hospital Dr. Manuel Gea González, México, D.F., México.
Servicio de Dermatología, Hospital Dr. Manuel Gea González, México, D.F., México.
Gac Med Mex. 2015 May-Jun;151(3):299-305.
Perineurioma is an infrequent and benign cutaneous neoplasm characterized by proliferation of perineurial cells. It is classified into two main types: intraneural and the extraneural or soft tissue perineurioma, in which the sclerosing variant is included. Sclerosing perineurioma is more frequently found on acral skin. Clinically, they are well-circumscribed,skin colored, nodular tumors.
Describe and communicate clinicopathologic findings from a case series of sclerosing acral perineurioma.
This is a clinical, morphological and immunohistologic case study of eight patients with the diagnosis of sclerosing perineurioma.
It included five men and five women, with ages ranging between nine and 66 years. All of them had lesion on acral skin. At microscopy study, the lesions showed a proliferation of epithelioid and spindle-shaped perineurial cells, arranged in small aggregates and short fascicles between thickened collagen bundles. Immunohistochemistry studies revealed that the proliferating cells expressed EMA, Claudin-1 and Glut-1, and were negative for S-100 protein.
It is important to report these infrequent skin tumors, so they can be taken into account in the differential diagnoses of acral lesions.
神经束膜瘤是一种罕见的良性皮肤肿瘤，其特征为神经束膜细胞增殖。它主要分为两种类型：神经内型和神经外型或软组织神经束膜瘤，后者包括硬化型。硬化型神经束膜瘤更常见于手足部皮肤。临床上，它们是边界清晰、肤色的结节状肿瘤。
描述并交流一组硬化型手足部神经束膜瘤病例的临床病理特征。
这是一项对8例诊断为硬化型神经束膜瘤患者的临床、形态学及免疫组织化学病例研究。
其中男性5例，女性5例，年龄在9岁至66岁之间。所有患者的手足部皮肤均有病变。显微镜检查显示，病变表现为上皮样和梭形神经束膜细胞增殖，呈小巢状排列，并在增厚的胶原束之间形成短束状结构。免疫组织化学研究显示，增殖细胞表达上皮膜抗原（EMA）、闭合蛋白-1（Claudin-1）和葡萄糖转运蛋白-1（Glut-1），而S-100蛋白呈阴性。
报告这些罕见的皮肤肿瘤很重要，以便在手足部病变的鉴别诊断中予以考虑。
