摘要
目的探讨软组织血管纤维瘤（AFST）的临床病理特点、分子遗传学特征、病理诊断和鉴别诊断。方法回顾性复习复旦大学附属肿瘤医院2011至2017年24例AFST的临床病理学资料，行形态学观察、免疫组织化学染色和荧光原位杂交并复习相关文献。结果24例患者，男性15例，女性9例（男∶女＝1.7∶1.0）。发病年龄8～68岁，平均年龄45岁。发生部位：四肢14例（上肢3例，下肢11例），躯干7例，颞部、腹膜后和肝脏各1例。临床上多表现为缓慢生长的无痛性肿块，直径0.8～14.0 cm，平均4.6 cm。镜下观察：多数肿瘤境界清楚，有纤维性包膜，少数病例局部浸润邻近的纤维脂肪组织。肿瘤主要由稀疏分布的短梭形细胞和丰富的薄壁分支状血管组成，分布于黏液样、纤维黏液样或胶原性间质中。周边均可见圆形或不规则形扩张的中等大血管。免疫组织化学标记：短梭形细胞主要表达波形蛋白（5/5），不同程度表达上皮细胞膜抗原（2/4）、雌激素受体（1/2）、孕激素受体（2/3）、α-平滑肌肌动蛋白（1/18）和结蛋白（1/10），Ki-67阳性指数均小于5%。CD34、CD31和ERG清晰显示肿瘤内血管的轮廓和分布。4例行荧光原位杂交检测，其中3例存在NCOA2基因相关易位。17例获得随访资料，随访时间3～69个月（平均30个月），均无瘤生存。结论AFST是一种良性纤维性肿瘤的新类型，以含有大量薄壁分支状小血管为特征，并显示特征性的NCOA2基因相关易位，需注意与血管性肿瘤及其他富含黏液样基质的软组织肿瘤相鉴别。
ObjectiveTo investigate the clinicopathologic and genetic features, pathologic diagnosis and differential diagnosis of angiofibroma of soft tissue（AFST）.MethodsThe clinicopathologic characteristics of 24 cases diagnosed at Fudan University Shanghai Cancer Center from 2011 to 2017 were analyzed; immunohistochemical staining and interphase fluorescence in situ hybridization （FISH） were performed, and the literatures were also reviewed.ResultsThere were 15 male and 9 female （male∶female=1.7∶1.0） patients with age of onset ranging from 8 to 68 years （mean, 45 years）. Fourteen cases occurred in extremities, including upper limbs （n=3） and lower limbs （n=11）; seven cases were in the trunk, and 1 case each was in the temporal region, retroperitoneum and liver, respectively. Clinically, the tumors usually presented as a slowly growing painless mass. Tumor sizes ranged from 0.8 to 14 cm （mean 4.6 cm）. Microscopically, most lesions were well-circumscribed, with fibrous capsules. Few cases infiltrated the surrounding fibrofatty tissue focally. The tumors were mainly composed of sparse short spindle cells and numerous small, branching, thin-walled blood vessels distributed in amyxoid, fibromyxoid or collagenous matrix, often accompanied by medium-sized, round or irregular and ecstatic vessels at the tumor periphery.By immunohistochemistry, all tested cases expressed vimentin （5/5）, and showed variable positivity for EMA （2/4）, ER （1/2）, PR （2/3）, α-SMA （1/18）and desmin （1/10）. Ki-67 proliferation index were all less than 5%. CD34, CD31 and ERG staining clearly outlined the contours of blood vessels in the stroma. Four cases were tested for NCOA2 gene rearrangement by FISH, of which three were positive. Follow-up data was available in 17 patients （range, 3 to 69 months; mean, 30 months） were all free of disease.ConclusionsSoft tissue angiofibroma is a benign fibroblastic neoplasm characterized by a prominent and complex vasculature set in a myxoid-to-collagenous str
作者
许晓琳
刘尽国
孙蒙
喻林
刘绮颖
柏乾明
吴丽静
王坚
Xu Xiaolin;Liu Jinguo;Sun Meng;Yu Lin;Lao;Bai Qianming;Wu Lijing;Wang Jian(Department of Pathology,Fudan University Shanghai Cancer Cente;Department of Oncology,Shanghai Medical College,Fudan University,Shanghai 200032,China;Department of Pulmonary Medicine,Zhongshan Hospital,Fudan University and Shanghai Respiratory Research Institute,Shanghai 200032,China)
出处
《中华病理学杂志》
CAS
CSCD
北大核心
2018年第8期616-621,共6页
Chinese Journal of Pathology
基金
国家自然科学基金青年基金项目（81202083）
上海市自然科学基金（15ZR1407500）
上海市卫生计生系统重要薄弱学科（2015ZB0201）
上海申康医院发展中心新兴前沿项目（HDC12014105）
关键词
血管纤维瘤
免疫组织化学
原位杂交
荧光
诊断
鉴别
Angiofibroma
Immunohistoehemistry
In situ hybridization
fluorescence
Diagnosis
differential
