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目的 探讨混杂性神经鞘瘤/神经束膜瘤(HS/P)的临床病理特点、病理诊断及鉴别诊断.方法 收集复旦大学附属肿瘤医院病理科2010年10月至2017年8月间诊断的35例HS/P的临床病理资料,行形态学观察和免疫组织化学检测,并复习相关文献.结果 男性7例,女性28例(男∶女=1∶4).发病年龄3~81岁,平均年龄36岁.发生部位:头颈部11例,躯干10例,四肢9例,肠道4例,大阴唇1例.术前病程1个月至20年,大多表现为缓慢性生长的肿块,可伴疼痛,最大径0.8~6.0 cm,平均2.6 cm.光镜形态:大多数肿瘤边界清楚,无明显包膜.低倍镜下,肿瘤多位于真皮或皮下,少数位于黏膜下层,主要由交织状、席纹状或旋涡状排列的梭形细胞组成;高倍镜下,肿瘤主要由胖梭形细胞组成,核肥胖而饱满,于胖梭形细胞之间夹杂核和胞质细长的纤细梭形细胞.瘤细胞无明显异型性,核分裂象罕见.部分病例可见核大深染的退变细胞.免疫组织化学标记:胖梭形细胞表达S?100蛋白(35/35)和SOX10(8/9);纤细梭形细胞不同程度表达上皮细胞膜抗原(EMA,31/35)、CD34(32/33)、Claudin?1(15/15)和GLUT?1(8/8),Ki?67阳性指数均<5%.16例获得随访资料,随访时间4~72个月(平46个月),仅1例发生局部复发,其余无瘤生存.结论 HS/P是一种好发于真皮及皮下的良性神经鞘膜肿瘤,部分可发生于胃肠道、鼻腔和外生殖区等少见部位;肿瘤由相互混杂的胖梭形施万细胞和细梭形神经束膜细胞组成,免疫表型上显示S?100蛋白和SOX10,及EMA、CD34、Claudin?1和GLUT?1双向分化.需注意与隆突性皮纤维肉瘤和孤立性纤维性肿瘤等鉴别.

Objective To investigate the clinical pathological features, pathological diagnosis and differential diagnosis of hybrid schwannoma/perineurioma. Methods The clinicopathological data of 35 cases were collected at Fudan University Shanghai Cancer Center, from October 2010 to August 2017;morphological observation and immunohistochemical staining were performed, and the literatures were also reviewed. Results There were 7 males and 28 females (male∶female=1∶4), patients with onset age ranging from 3 to 81 years(mean=36 years). Of 35 tumors, 11 cases occurred in the head and neck, 10 in the extremities, 9 in the trunk, 4 in the intestine, and 1 in the labiamajora, respectively. Clinically, most patients presented as a slowly growing dermal nodule, sometimes associated with pain. The duration of symptoms ranged from 1 month to 20 years before excision. Tumor size ranged from 0.8 cm to 6.0 cm (mean=2.6 cm). Microscopically, the tumors were usually well circumscribed but unencapsulated. At low power, most tumors were located in the dermis or subcutis, and several cases in the submucosal tissues. The tumors were composed of fascicular, storiform or whorled growth of closely intermixed plump spindle cells and slender spindle cells. The plump spindle cells had ill?defined eosinophilic cytoplasm with larger tapered or wavy nuclei, whereas the slender spindle cells had comparatively delicate nuclei with elongated cytoplasmic processes. Tumor cells had no obvious atypia, and mitoses were rare. Scattered large cells with degenerative nuclear atypia were seen in some cases. By immunohistochemistry, most of plump spindle cells showed strong staining of S?100 protein(35/35) and SOX10(8/9), whereas slender spindle cells stained variably for epithelial membrane antigen(31/35), CD34(32/33), Claudin?1 (15/15) and GLUT?1(8/8). Ki?67 proliferation index were all less than 5%. Follow?up data available in 16 patients (range 4 to 72 months; mean=46 months) were all free of disease, and one case developed local recurrence. Conclusions Hybrid schwannoma / perineuriomaisa benign nerve sheath tumor that typically manifests as a dermaland subcutaneous tumor, less frequently may affect uncommon sites such as the nasal cavity, the gastro?intestinal tract, and the external genital areas. The tumors consisted of intimately admixed plump?spindled schwannian cells and slender?spindled perineurial cells showing dual differentiation of strong S?100 protein and SOX10 expression in the former component and variable immunoreactivity of epithelial membrane antigen, Claudin?1 and CD34 in the latter. It should be aware of the possibility of potentially misinterpretation of hybrid schwannoma/perineurioma as dermatofibrosarcoma protuberans and solitary fibrous tumor and so on.