隆突性皮肤纤维肉瘤55例临床病理分析  

Pathologic and clinical analysis of 55 cases of dermatofibrosarcoma protuberans

作　　者：顾婷婷 姚丽倩[1] 王雨潇 徐松[1] 高小姣 GU Tingting;YAO Liqian;WANG Yuxiao;XU Song;GAO Xiaojiao(Department of Pathology,Affiliated Kunshan Hospital of Jiangsu University,the First People′s Hospital of Kunshan,Jiangsu Province,Kunshan 215300;Department of Pathology,Traditional Chinese Medicine Hospital of Kunshan,Jiangsu Province)

机构地区：[1]江苏大学附属昆山医院,江苏省昆山市第一人民医院病理科,昆山215300 [2]江苏省昆山市中医院病理科

出　　处：《南通大学学报（医学版）》2023年第4期346-350,共5页Journal of Nantong University(Medical sciences)

基　　金：昆山市重点研发计划(社会发展)项目(KSF202133);苏州市重点扶持学科(SZFCXK202140)。

摘　　要：目的:分析55例隆突性皮肤纤维肉瘤(dermatofibrosarcoma protuberans,DFSP)患者的临床表现和病理,讨论其诊断和鉴别诊断要点,提高对该肿瘤的认识。方法:回顾性分析昆山地区2007年1月—2021年1月期间诊断的55例DFSP患者的临床表现、病理组织学特点、治疗及预后。结果:DFSP临床多表现皮肤瘢痕或萎缩性斑块结节,表面可有破溃,肿块直径平均(3.31±1.04)cm。发病部位主要为躯干、头颈肩部,也可发生于乳房、腹股沟等少见部位。病变多位于真皮层,单发结节,偶为多发,切面呈褐色、粉红色、灰白色,与周围组织界限相对较清,可浸润皮下脂肪及横纹肌。组织形态大多为梭形、短梭形,可呈轮辐状、漩涡状或花边样排列,可伴有黏液变性,间质硬化,以及出现色素细胞分布。免疫组织化学染色显示肿瘤细胞主要表达CD34和Vimentin,也可在胞质表达BCL-2和β-Catenin。4例行FISH荧光原位杂交分子检测,结果均存在血小板衍生的生长因子B链基因重排。55例DFSP中行局部扩大切除术14例,行局部切除术41例。随访的30例患者中6例术后局部复发。结论:DFSP是易发生于真皮的低度恶性软组织肿瘤,掌握DFSP的临床病理特点,通过免疫组织化学染色标记及分子检测能有效地诊断,避免与其他皮肤梭形细胞肿瘤混淆;主要的治疗方式是外科手术扩大切除,局部切除易复发。Objective:To investigate the pathological features and clinical manifestation of 55 cases of dermatofibrosarcoma protuberans(DFSP),and to discuss the diagnosis of DFSP in order to raise the awareness of the disease.Methods:A retrospective analysis of 55 patients with DFSP diagnosed in Kunshan area since January 2007 to January 2021,including the histopathological features,clinical manifestations,treatment and prognosis.Results:The review of the histologic results of the 55 DFSP cases revealed that the clinical manifestation included nodules,skin patches average diameter of(3.31±1.04)cm.The main sites of the disease are the chest,abdomen,back,head and shoulders,and they can also occur in the breast,groin and other rare parts.The tumors grew in the dermis and subcutaneous tissues was single and multiple nodules with the section appear to be brown,pink,and grayish-white,tumor composed of uniform infiltrative spindle cells arranged in a cartwheel,storiform or lace-like pattern,and accompanied by mucous degeneration,stromal hardening,and the distribution of pigment cells.Immunohistochemical staining showed that the tumor cells were positive for CD34,Vimentin always,and positive for BCL-2 andβ-Catenin in cytoplasm sometimes.4 cases showed platelet-derived growth factor subunit B gene rearrangement by FISH molecular detection.14 patients underwent wide excision and 41 patients underwent local excision.Of the 30 patients followed up,6 cases had local recurrence after surgery.Conclusion:DFSP is a soft tissue tumor with low malignancy by multiplex clinical symptoms.Histopathological,immunohistochemical staining and molecular detection is particularly important to avoid confusion with other skin spindle cell tumors and make the correct diagnosis.Although surgical operation is the best treatment option,patients require extensive excision,as the local excision often has a high recurrence rate.

关 键 词：隆突性皮肤纤维肉瘤 免疫组织化学染色 FISH 血小板衍生的生长因子B链基因重排 临床病理 

分 类 号：R739.5[医药卫生—肿瘤]