色素沉着息肉综合征伴肺黏液腺癌一例并文献复习  

Peutz-Jeghers syndrome complicated with pulmonary mucinous adenocarcinoma： a case report and review of the literature

作　　者：李明晖[1] 沈巨信[1] 钟波[1] 何薇[1] 马建勇[1] 

机构地区：[1]浙江绍兴市人民医院浙江大学绍兴医院呼吸科,312000

出　　处：《中华结核和呼吸杂志》2011年第12期919-922,共4页Chinese Journal of Tuberculosis and Respiratory Diseases

摘　　要：目的探讨色素沉着息肉综合征（又称Peutz—Jeghers综合征）患者的临床表现、影像学和病理特点及其并发症。方法分析绍兴市人民医院呼吸科收治的1例色素沉着息肉综合征患者的临床特点、影像学及病理资料，以“Peutz-eghers综合征，肺癌”为检索词检索PubMed检索系统和中国生物医学文献光盘数据库（CBMdisc），并结合国外文献报道的3例色素沉着息肉综合征伴肺癌病例进行分析。结果患者为女性，30岁，以咳嗽、咳痰、咯血就诊。因口唇周围及指趾特征性的色素沉着及全消化道息肉诊断为色素沉着息肉综合征。胸部CT示右下肺结节影。手术后病理检查确诊为右下肺黏液腺癌，瘤体直径为2．5cm。检索到的3例患者均为男性，年龄为22～43岁。2例表现为咳嗽、咳痰及咯血，1例体检时X线胸片发现异常，均在短期内死亡。病理诊断均确诊为肺腺癌。结论色素沉着息肉综合征合并肺癌者罕见，患者预后较差。Objective To explore the clinical characteristics, complications and prognosis of Peutz- Jeghers syndrome. Methods The clinical manifestations, imaging characteristics and pathological data of one patient with Peutz-Jeghers syndrome treated in Shaoxing People＇ s Hospital were presented, and 3 cases of Peutz-Jeghers syndrome complicated with lung cancer reported in the literature were reviewed. The literature review was carried out by searching the Pubmed and CBMdisc database with Peutz-Jeghers syndrome and lung cancer as the key words. Results The patient treated in our hospital was a 30-year-old female. The primary manifestations were cough, sputum production and hemoptysis. Multiple gastrointestinal hamartomatous polyps were found, and associated with melanin pigmentation of the buccal mucosa, the lips, the hands and the feet. Therefore the diagnosis of Peutz-Jeghers syndrome was made. Chest CT scan showed a peripheral nodule at the right lower lobe, which was confirmed to be lung mueinous adeuocarcinoma by surgery and pathology. The 3 cases reported in the literature were males aging from 22 to 43 years old. Cough, expectoration and hemoptysis were defined as the manifestations for 2 patients, and another case was found by physical check-up. They all died within a short period. Pathological study revealed adenocareinoma of the lung in all the cases. Conclusion Peutz-Jeghers syndrome complicated with lung cancer is rare and has a poor prognosis.

关 键 词：Peutz—Jeghers综合征 肺肿瘤 腺癌 黏液 

分 类 号：R735.3[医药卫生—肿瘤]