肛管直肠恶性黑色素瘤临床诊治及预后  

Clinical Diagnosis,Treatment and Prognosis of Anorectal Malignant Melanoma

作　　者：时立瞳 王文佳 秦艳茹[1] SHI Litong;WANG Wenjia;QIN Yanru(Department of Oncology,the First Affiliated Hospital of Zhengzhou University,Zhengzhou 450052,China)

机构地区：[1]郑州大学第一附属医院肿瘤科,河南郑州450052

出　　处：《河南医学研究》2022年第6期978-983,共6页Henan Medical Research

摘　　要：目的分析肛管直肠恶性黑色素瘤(ARMM)患者的生物学特性、临床表现、诊治方法及与预后的关系,以提高ARMM的临床诊治水平。方法回顾性分析2012年5月至2020年10月郑州大学第一附属医院病理检查确诊为ARMM的38例患者的临床资料并随访。采用Kaplan-Meier法绘制生存曲线并采用log-rank检验进行比较。结果38例ARMM患者中男10例,女28例,中位发病年龄为60岁。临床症状缺乏特异性,21例(55.26%)首发症状为便血。内镜表现多为发生在肛管直肠的无蒂、质硬脆、有色素沉着、表面溃烂充血、伴有肠腔狭窄的隆起型肿块。磁共振成像(MRI)和电子计算机断层扫描(CT)异常检出率为100%和82.61%。免疫组织化学S-100、HMB-45、Melan-A、Vimentin和Ki-67阳性率分别为93.94%、93.94%、96.88%、100%和10%~90%。中位总生存期为25.97个月,1、3、5a生存率分别为75.64%、39.97%和27.41%。治疗无特效手段,33例患者行手术治疗,术后远处转移19例,复发5例。患者总生存期与肿瘤直径、浸润深度和临床分期有关(P<0.05))。结论ARMM临床罕见、预后差、误诊率高,需结合病理及免疫组化结果明确诊断。肿瘤直径、浸润深度和临床分期是ARMM重要的预后影响因素。早期首选手术治疗,晚期行以手术为主的综合治疗,免疫联合靶向治疗是ARMM未来治疗的突破口。Objective To analyze the biological characteristics,clinical manifestations,diagnosis and treatment methods and their relationship with prognosis of patients with anorectal malignant melanoma(ARMM).Methods The clinical data of 38 patients who were pathologically diagnosed as ARMM from May 2012 to October 2020 in the the First Afiliated Hospital of Zhengzhou University were retrospectively analyzed and followed up.The survival curve was drawn using the Kaplan-Meier method and compared with the log-rank test.Results Among 38 patients with ARMM,10 were male and 28 were female.The median age of onset was 60 years old.The clinical symptoms lacked specificity,of which 21 cases(55.26%)had blood in the stool as the first symptom.Endoscopy were mostly characterized by bulging lumps of untidy,hard and brittle,pigmentation,surface festering congestion,accompanied by narrow intestines in the anorectal.The abnormal detection rates of magnetic resonance imaging(MRI)and computed tomography(CT)were 100%and 82.61%.The positive rates ol immunohistochemistry for S-100,HMB-45,Melan-A,Vimentin and Ki-67 were 93.94%,93.94%,96.88%,100%and 10%-90%,respectively.The median overall survival time was 25.97 months.The 1,3 and 5 year survival rates were 75.64%,39.97%and 27.41%,respectively.There was no special effect to treatment,33 patients underwent surgical treatment,19 cases were transferred at a distance after surgery and 5 cases were relapsed.The overall survival of patients was related to tumor diameter,depth of invasion and clinical stage(P<0.05).Conclusion ARMM is a clinical rare disease with poor prognosis and high misdiagnosis rate.The diagnosis should be confirmed based on pathological and immunohistochemical results.Tumor diameter,depth of invasion and clinical stage are important prognostic factors for ARMM.Surgical treatment is the first choice for treatment in the early stage,and comprehensive treatment based on surgery in the late stage.The combination of immune and targeted therapy is a breakthrough in ARMM's future treatmen

关 键 词：肛管直肠恶性黑色素瘤 预后 临床特征 

分 类 号：R735.38[医药卫生—肿瘤]