摘要
目的 分析总结中国老年人原发性食管黑色素瘤(PMME)的临床表现、病理特点、治疗及转归等,加强临床医生对老年人PMME的认识.方法 报道1例中日友好医院消化科收治的老年PMME患者,同时收集截至2023年7月国内外报道的中国老年人PMME的文献及病例资料,对该疾病的流行病学特征、内镜下表现、临床表现、诊断、治疗及预后进行总结分析.结果 截至2023年7月文献报道中国老年PMME患者,联合我院病例,共计114例老年PMME患者,男性68例(59.6％)、女性46例(40.4％);年龄60～81岁,中位年龄65岁.主要症状为吞咽困难、进食哽咽;其次为胸痛、胸骨后烧灼感等;病变位置主要位于食管中段、下段;多为向腔内突出肿物,有2例(1.8％)患者仅发现食管黏膜色素沉着.HMB45(+)74 例(64.9％)、HMB45(-)3 例(2.6％);S-100(+)66例(57.9％)、S-100(-)2例(1.8％),部分患者未记录.45例(39.5％)患者有淋巴结或远处转移;38例(33.3％)患者肿瘤局限于食管,无淋巴结或远处转移,31例患者未描述肿瘤转移情况.61例(53.5％)患者随访时间0.3～39个月,中位随访时间6.75个月.随访期间存活的患者共30例(26.3％),随访时间1～39个月,中位随访时间7.5个月.在死亡患者中,自就诊至死亡时间0.3～31个月.结论 中国老年人PMME起病隐匿,症状不典型,发病时多有转移,恶性程度高,预后较差.
Abstract
Objective To analyze and summarize the clinical manifestations,pathological characteristics,treatment,and outcomes of primary malignant melanoma of the esophagus(PMME)in elderly Chinese patients.Methods A case study of an elderly patient with PMME was conducted at the Department of Gastroenterology in the China-Japan Friendship Hospital.Additionally,literature and case data on elderly PMME cases reported in China up to July 2023 were gathered and analyzed to summarize the epidemiological characteristics,endoscopic manifestations,clinical presentations,diagnosis,treatment,and prognosis of the disease.Results A comprehensive review of the literature up to July 2023 documented a total of 114 cases of elderly patients with PMME in China,which also included cases from our hospital.Among these cases,there were 68 male patients(59.6％)and 46 female patients(40.4％),ranging in age from 60 to 81 years,with a median age of 65 years.The predominant clinical manifestations observed were dysphagia and choking while eating,followed by chest pain and retrosternal burning sensation.The majority of the lesions were found in the middle and lower segments of the esophagus,predominantly protruding into the lumen,with only 2 cases(1.8％)displaying esophageal mucosal pigmentation.Immunohistochemical analysis revealed that HMB45 was positive in 74 cases(64.9％)and negative in 3 cases(2.6％),while S-100 was positive in 66 cases(57.9％)and negative in 2 cases(1.8％),although data for some patients were not available.Lymph node or distant metastases were present in 45 cases(39.5％),while 38 patients(33.3％)had tumors confined to the esophagus without metastases.Of the 114 patients,61(53.5％)had a follow-up period ranging from 0.3 to 39 months,with a median follow-up time of 6.75 months.Among the patients who survived during the follow-up period,there were 30 cases(26.3％),with a follow-up time of 1 to 39 months and a median follow-up time of 7.5 months.For the deceased patients,the time from consultation to death ranged from 0.3 to 31 months.Conclusions Elderly individuals with PMME in China typically present with a gradual onset,nonspecific symptoms,frequent metastasis upon diagnosis,aggressive behavior,and unfavorable outcomes.
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