肺原发恶性黑色素瘤一例并文献回顾

Primary malignant melanoma of the lung:a case report and literature review

目的探讨并总结肺原发恶性黑色素瘤的临床特点、诊疗及预后。方法报道1例原发于肺的恶性黑色素瘤病例。并以"恶性黑色素瘤、肺脏、原发"为检索词检索中国知网、维普网和万方数据库,以"malignant melanoma、lung、primary"为检索词检索PubMed数据库中我国的肺原发恶性黑色素瘤患者的文献进行文献回顾。结果本例患者,男,65岁,因"咳嗽伴咯血2月余"入院。胸部CT显示右肺上叶占位,肿物穿刺活检病理结果为恶性黑色素瘤。免疫组化示:S-100(+),SOX-10(+),HMB45(+),MelanA(-),AE1/AE3(-),CK5/6(-),Ki-67(40%)。PET-CT提示多发脏器转移,经对症治疗后症状缓解,由于未接受进一步治疗,病情进展,于3个月后死亡。对36篇文献报道中的41例及本例患者资料进行回顾分析,患者多为中老年人,中位年龄55岁,肿瘤肺叶分布及症状无特异性,影像学绝大部分表现为单发肿物或结节,容易早期转移和复发,31例确诊后生存期范围为27 d至24个月,其中23例(74.2%)患者生存期<12个月。结论肺原发恶性黑色素瘤临床罕见,不易与肺癌等疾病相鉴别,需结合症状、影像学以及组织病理结果等综合诊断,手术、放化疗以及免疫治疗等可以改善患者预后,但由于恶性程度高,极易复发和转移,总体预后不良。

Objective To analyze and summarize the clinical features,diagnosis and treatment of primary malignant melanoma of the lung(PMML).Methods To report a confirmed case.And searched the keywords"malignant melanoma,primary,lung"from the databases of CNKI,Weipu,Wanfang and PubMed,then reviewed the relevant literature.Results A 65-year-old male admitted to the First Affiliated Hospital of Zhengzhou University with the complaint of frequent coughs with bloody sputum for 2 months.Computed tomography(CT)showed a large space-occupy ing lesion in the upper lobe of the right lung and to be a hyper metabolic tumor by positron emission tomography-CT.The pathological biopsy of the mass con-firmed a malignant tumor cell,immunohistochemical results showed S-100(+),SOX-10(+),HMB45(+),MelanA(-),AEI/AE3(-),CK5/6(-),Ki-67(40%).The patient died after 3 months of di-agnosis because of refusing any further therapy.There were 36 papers searched from mentioned databases reported 41 Chinese patients with PMML.And we analyzed the clinical data of 42 cases(include the pres-ent case)and discussed the diagnosis and treatment while referring to the existing literatures.Conclusions PMML is extremely rare,and difficult to differentiate from lung cancer,A diagnosis of PMML is based on.the combination of clinical,imaging characteristics and pathological outcomes.The choice of treatment is an.aggressive surgical approach,combined with radiation therapy and chemotherapy.Because of high degree of malignancy,powerful capacity for invasiveness and recurrence,the overall prognosis is poor.