肺原发性恶性黑色素瘤二例并文献复习

Two cases of primary malignant melanoma of the lung and literature review

目的分析肺原发性恶性黑色素瘤(PMML)的临床特征、诊断、治疗,以提高临床医师对该病的认识。方法回顾性分析南京医科大学第一附属医院近4年来通过病理确诊的2例PMML患者的临床资料,并复习近16年的相关文献,总结其临床特征、诊断与治疗。结果2例患者均为女性,年龄分别为48、69岁。其症状和体征无特异性。胸部CT检查提示肺部占位。1例经气管镜淋巴结活检术获得组织,免疫组织化学示HMB45(++),S-100(++);另外1例通过CT定位经皮肺穿刺活检,免疫组织化学示S-100(++),HMB45(个别+),Melan-A(++)。确诊后3个月死亡。结论PMML是一种较为罕见的疾病。其临床表现以及影像学缺乏特异性,确诊仍基于病理活检。目前手术仍是治疗首选,化疗及靶向免疫等综合治疗可以改善预后,但总体预后较差。

Objective To analyze the clinical features, diagnosis and treatment of primary malignant melanoma of the lung (PMML), and improve the understanding of the disease. Methods The clinical data of two PMML patients were retrospectively analyzed, and the literatures in recent fifteen years were reviewed.The two cases were pathologically diagnosed in the First Affiliated Hospital of Nanjing Medical University.The clinical characteristics, diagnosis and treatment were summarized. Results Two cases were both elder women and admitted to our department with complaints of chest tightness without any other specific signs and symptoms.Chest CT revealed lung mass.One patient underwent transbronchial needle aspiration which showed a malignant tumor cell, immunohistochemical result showed HMB45(+ + ), S100(+ + ). Another patient underwent CT-guided fine needle aspiration biopsy of the lung mass, which showed small round cell malignant tumor, immunohistochemical result showed S-100(+ + ), HMB45(several + ), and Melan-A(+ + ). Both cases were diagnosed as PMML and died after three months of diagnosis. Conclusions PMML is a relatively rare disease, of which clinical symptoms and CT images are lack of specificity.Diagnosis is based on pathological biopsy.Surgery is still the preferred treatment for PMML.Chemotherapy and targeted immunotherapy can also improve prognosis, but the overall prognosis is extremely poor.