肺内癌性淋巴管炎的临床特征与诊断

背景与目的:肺内癌性淋巴管炎(pulmonarylymphangiticcarcinomatosis,PLC)是肿瘤肺内转移的一种特殊类型,常被误诊为其它肺间质病变。本研究旨在探讨PLC的临床特征,为其鉴别、评价其预后提供经验性资料。方法:将近10年来中山大学附属第一医院病理诊断明确的43例PLC与其它病因明确的肺间质病变46例患者的临床资料作回顾性对比分析。结果:PLC组:原发肺癌20例,原发肺外肿瘤23例(乳腺癌9例,大肠癌8例,胃癌6例)。其影像学表现:从肺门向肺野呈放射状、条索状不均一阴影,部分条索可达胸膜并见小结节;或肺野呈毛玻璃状改变;纵隔、肺门淋巴结肿大51.2%(22/43),胸腔积液53.5%(23/43)。肺外远处转移多见,包括淋巴结转移(锁骨上、腋窝、腹膜后等)19例(44.2%),胸膜转移15例(34.9%),骨转移9例(20.9%),肝转移6例(14.0%),心包转移3例(7.0%),脑转移3例(7.0%)。血清CEA升高53.5%(23/43)。咳嗽、气促、呼吸困难等呼吸道症状经气道解痉药治疗无效。病情急进性发展,31例(72.1%)2～7个月死亡。其它肺间质病变组:影像学表现为肺野内不规则的纤维条索影,未见纵隔、肺门淋巴结肿大、胸腔积液等上述肺外表现。咳嗽、气促、呼吸困难等呼吸道症状经气道解痉药治疗有效。病情发展缓慢。结论:PLC常发生于肺癌、乳腺癌、大肠癌、胃癌等肿瘤的肺内转移。当患者出现上述肺间质病变,呼吸道症状经气道解痉药治疗无效,病情急进性发展时,应高度怀疑PLC。PLC预后差。

Clinical features and diagnosis of pulmonary lymphangitic carcinomatosis

BACKGROUND & OBJECTIVE: Pulmonary lymphangitic carcinomatosis (PLC) is a special type of pulmonary metastasis of carcinoma. It is easy to be misdiagnosed as other pulmonary interstitial diseases. This study was to discuss the clinical features of PLC, and provide experience information for diagnosis, differentiated diagnosis, and evaluation of prognosis of PLC. METHODS: A retrospective comparison analysis was performed on 43 PLC patients with pathologic diagnosis and 46 patients with other pulmonary interstitial diseases with clear etiology in the first affiliated hospital of Sun Yat-sen University within the past decade. RESULTS: In PLC group, 20 patients were found with primary lung cancer; 23 patients were found with primary non-pulmonary carcinoma: 9 cases of breast cancer, 8 cases of large intestinal carcinoma, and 6 cases of gastric carcinoma. The changes of imaging included linear and radiating appearances from the hilum to the outer part even extending to the pleura with nodules, ground-glass opacity of the lung. Enlargement of lymph nodes in mediastinum was present in 51.2% (22/43) and that in pleural effusion was present in 53.5% (23/43) of patients. Extrapulmonary manifestations (metastasis) included 19 cases (44.2%) of lymph nodes to the supraclavicular region, axillary fossa, and post-peritoneal region, 15 cases (34.9%) to the pleura, 9 cases (20.9%) to the bones, 6 cases (14.0%) to the liver, 3 cases (7.0%) to the pericardium, and 3 cases (7.0%) to the brain. The elevated serum level of CEA was commonly observed (23/43, 53.5%). Respiratory manifestations of PLC, such as coughing, panting, dyspnea, and so on, could not be cured by anti-spasm treatment. The development of PLC was so progressive that 31 patients (72.1%) were followed for only 2-7 months before death. The changes of imaging in other pulmonary interstitial disease group included irregular linear or reticular appearances, enlargement of lymph nodes in the mediastinum and hilum, and extrapulmonary manifestations like pleural effusion were not observed. Respiratory manifestations, such as coughing, panting, dyspnea, and so on, could be cured by anti-spasm treatment. Moreover, the development of PLC was relatively slow. CONCLUSIONS: PLC often occurs in patients with primary carcinoma in lung, breast, large intestine, stomach, and so on. More attention should be paid to the diagnosis of PLC in patients who have pulmonary interstitial lesions as described above, and whose respiratory symptoms could not be relieved by anti-spasm treatment and developed progressively. The prognosis of PLC is poor.