原发性肺睾丸核蛋白中线癌1例并文献复习

Primary pulmonary NUT midline carcinoma： a case report and literatures review

目的 探讨原发性肺睾丸核蛋白中线癌的临床特点。方法 回顾性分析1例原发性肺睾丸核蛋白中线癌患者的临床特点。以 “睾丸核蛋白”“中线癌”“肺”为检索词在万方数据库和中国期刊全文数据库进行检索,以 “nuclear protein in testis”“midline carcinoma”“pulmonary”为检索词在PubMed数据库进行检索,检索时间截止至2017年4月。结果 患者男,28岁,因 “双下肢乏力17d,胸痛5d”入院,影像学检查示右肺下叶背段恶性肿瘤伴区域多组淋巴结、全身多发骨转移。肺穿刺病理检查示睾丸核蛋白抗体阳性,诊断为原发性肺睾丸核蛋白中线癌,给予化疗及对症支持治疗,胸痛缓解,肿块较前缩小,目前仍在随访中。文献检索经筛选后共有24篇入选,报道31例患者,男16例,女15例,年龄5-71岁,临床表现无特异性,影像学多表现为近肺门处肿块影,多伴远处转移,脑转移罕见,中位生存期为4.1个月。结论 原发性肺睾丸核蛋白中线癌临床罕见,恶性程度高,缺乏有效治疗手段,预后差;对于年轻、无吸烟史、临床病情进展快的中央型低分化或未分化肺癌患者,应警惕该病可能。

Objective To analyze the clinical features of primary pulmonary nuclear protein in testis （NUT） midline carcinoma. Methods The clinical features, auxiliary examinations and diagnosis of a patient with primary pulmonary NUT midline carcinoma were reported and the related literatures were reviewed. The literature review was carried out respectively in Wanfang Data, CNKI and PubMed until April 2017 with ＂nuclear protein in testis＂,＂ midline carcinoma＂,＂ pulmonary＂ being the search terms, Results A 28 year old male patient was admitted to the hospital on March 2017 because of lower limbs weakness for 17 days and chest pain for five days. The imaging results revealed a large mass in right lower lung as well as lesions in the lymph node and bone metastasis. Lung biopsy was performed and the pathological study confirmed the diagnosis of primary pulmonary NUT midline carcinoma for the tumor was immunoreactive for NUT. The tumor size was reduced and the symptoms were improved after the administration of chemotherapy, the patient was being followed up. A total of 24 literatures were retrieved from Wanfang Data, CNKI and PubMed. There were 16 male and 15 female patients, aging from five to 71 years old. The symptoms had no specificity. Computed tomography scans showed a large, centrally located primary mass with confluent involvement of mediastinal lymph nodes. The prognosis was extremely poor with a median survival of 4.1 months. Conclusions Primary pulmonary NUT midline carcinoma is a rare, aggressive, poor prognosis disease without effective treatment. It usually presents in the nonsmoking young patients with rapid progression and the histological diagnosis is undifferentiated or poorly differentiated lung carcinoma.