丛状神经鞘瘤10例临床及组织病理分析  

Plexiform schwannoma:a clinical and histopathological study of 10 cases

作　　者：余斌[1,2] 陈凤鸣 王雷[1] YU Bin;CHEN Feng-ming;WANG Lei(Department of Dermatology,Xijing Hospital,Fourth Military MedicalUniversity,Xi′an 710032,China)

机构地区：[1]第四军医大学西京皮肤医院,陕西西安710032 [2]福建医科大学附属第二医院皮肤科,福建泉州362000

出　　处：《临床皮肤科杂志》2023年第2期71-74,共4页Journal of Clinical Dermatology

摘　　要：目的:探讨丛状神经鞘瘤(PS)临床及组织病理学特点。方法:回顾性分析10例PS患者临床表现及组织病理学特点,并进行文献复习。结果:10例患者中女7例,男3例;发病年龄1~58岁。皮损单发者9例,多发者1例。皮损位于躯干者7例,上肢者2例,躯干及四肢均累及者1例。所有患者皮损表现为单发光滑的丘疹(2例)、多个光滑丘疹融合成的斑块(2例)、外生性结节(2例)、褐色丘疹(3例)及褐色斑块(1例)。1例并发神经纤维瘤病Ⅰ型(NF1),1例并发神经纤维瘤病Ⅱ型(NF2)。所有患者皮损组织病理均示真皮或皮下可见丛状分布的具有包膜的肿瘤团块,肿瘤团块内细胞形态以Antoni A型为主,可见特征性的Verocay小体,可见个别核分裂象或轻度异形性,肿瘤团块内或间质中可有黏液。免疫组化示肿瘤团块内S-100蛋白弥漫性阳性,神经丝蛋白(NF)阴性。结论:PS是神经鞘瘤中罕见的类型,好发于躯干及双上肢,其临床表现缺乏特征性,诊断主要依靠皮损组织病理及免疫组化检查。Objective:To analyze the clinical and histopathological features of plexiform schwannoma(PS).Methods:The clinical manifestations and histopathological features of 10 patients with PS were analyzed retrospectively,and the literature was reviewed.Results:Seven patients were female and three patients were male.The age of disease onset was 1 to 58 years.Nine cases had one lesion,and one case had multiple lesions.PS were located on the trunk(seven cases),upper limb(two cases),as well as both trunk and limb(one case).The clinical presentation revealed solitary papule with smooth surface(two cases),multiple papules with smooth surface fused to form plaques(two cases),exophytic nodules(two cases),brown papules(three cases)or brown plaques(one case).One patient accompanied by neurofibromatosis typeⅠ(NF1),and one patient accompanied by neurofibromatosis typeⅡ(NF2).Histopathological examination showed a well encapsulated nodular pro liferation of spindle cells arranged in plexiform patterns involving dermis and subcutis.The tumor cells showed a predominantly Antoni A pattern and nuclear palisading forming Verocay bodies found in all cases.Slight atypia and few mitoses were observed.Mucin deposits were found in the lesion.Immunohistochemistry showed the tumor cells were diffusely positive for S-100 pro tein,but negative for NF.Conclusion:PS is a rare type of schwannoma and is mainly located on trunk and upper limbs.The clinical manifestations of PS are not specific,and histopathologic and immunohistochemical studies are the key to diagnosis.

关 键 词：丛状神经鞘瘤 神经鞘瘤 神经纤维瘤病Ⅰ型 神经纤维瘤病Ⅱ型 

分 类 号：R739.5[医药卫生—肿瘤]