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[经病理分析诊断的原发性气管恶性血管球瘤伴肺转移:1例病例报告及文献复习]

[Primary tracheal malignant glomus tumor with lung metastasis diagnosed by pathological analysis: a case report and literature review].

作者信息

Huang B, Chen F G, Zhuang J, Zheng W C, Zhu W Y, Zhang Q C, Wang S H, Guo C M, Xie C M

机构信息

Department of Respiratory Medicine, Affiliated Shantou Hospital of Sun Yat-sen University, Shantou 515000, China.

出版信息

Zhonghua Jie He He Hu Xi Za Zhi. 2017 Sep 12;49(9):697-702. doi: 10.3760/cma.j.issn.1001-0939.2017.09.016.

Abstract

To study the clinical manifestations, pathological features, diagnosis, differential diagnosis, treatment and prognosis of primary tracheobronchial or pulmonary malignant glomus tumor (MGT). A case of primary tracheal MGT with lung metastasis diagnosed by pathological analysis admitted to Affiliated Shantou Hospital of Sun Yat-sen University in May. 2015 was analyzed, and the related literatures were reviewed. We searched databases including PubMed, Embase, Ovid, Cochrane, Wanfang and Chinese National Knowledge infrastructure (CNKI), using the keyword "tracheal or bronchial or pulmonary malignant glomus tumor" from Jan. 1975 to Dec. 2016. A 47 year-old male patient was admitted to the hospital because of cough, chest tightness and shortness of breath for 3 days. The chest CT showed a soft tissue mass with a diameter of 2.5 cm in the lower tracheal segment, and the lumen was narrowed. Meanwhile, multiple nodular opacities were shown in both lungs. The admission diagnosis was thyroid cancer with multiple metastases of lung. Electronic bronchoscopic airway tumor ablation and cryotherapy were performed, and then the biopsy of the tumor was conducted and the pathological study confirmed the diagnosis of primary tracheal MGT. After 1 month, the tracheal tumor recurred. Then, electronic bronchoscopic airway tumor ablation and cryotherapy were performed again. The patient declined further therapy such as radiotherapy or chemotherapy and died one month later. A total of 14 literatures including 15 cases were retrieved from databases. In addition of this case, a total of 16 cases were analyzed, including 9 males, 7 females. Age of onset ranged from 9 to 74 years, and the average age was 49 years. These patients' chest CT showed airway mass or lung space occupying lesions, and the clinical manifestations were nonspecific. Primary MGT in trachea, bronchus or lung is a rare disease, which is easy to be misdiagnosed or to miss diagnosis. The final diagnosis depends on pathological morphology, and the main treatment is lobectomy or tracheal segment resection surgery. Due to its high invasiveness, local recurrence and metastasis may occur easily. The primary MGT in trachea, bronchus or lung is of poor prognosis.

摘要

探讨原发性气管支气管或肺恶性血管球瘤(MGT)的临床表现、病理特征、诊断、鉴别诊断、治疗及预后。分析2015年5月中山大学附属汕头医院收治的1例经病理分析确诊为原发性气管MGT伴肺转移的病例,并复习相关文献。我们检索了包括PubMed、Embase、Ovid、Cochrane、万方和中国知网(CNKI)在内的数据库,使用关键词“气管或支气管或肺恶性血管球瘤”检索1975年1月至2016年12月的文献。1例47岁男性患者因咳嗽、胸闷、气短3天入院。胸部CT显示气管下段有一直径2.5 cm的软组织肿块,管腔狭窄。同时,双肺可见多发结节状阴影。入院诊断为甲状腺癌伴肺多发转移。行电子支气管镜气道肿瘤消融及冷冻治疗,随后进行肿瘤活检,病理研究确诊为原发性气管MGT。1个月后,气管肿瘤复发。再次行电子支气管镜气道肿瘤消融及冷冻治疗。患者拒绝放疗或化疗等进一步治疗,1个月后死亡。从数据库中检索到14篇文献,共15例。除本病例外,共分析16例,其中男性9例,女性7例。发病年龄9~74岁,平均年龄49岁。这些患者的胸部CT显示气道肿块或肺部占位性病变,临床表现无特异性。气管、支气管或肺原发性MGT是一种罕见疾病,易误诊或漏诊。最终诊断依赖于病理形态学,主要治疗方法是肺叶切除术或气管节段切除术。因其侵袭性强,易发生局部复发和转移。气管、支气管或肺原发性MGT预后较差。

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