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  • Title: [Primary tracheal malignant glomus tumor with lung metastasis diagnosed by pathological analysis: a case report and literature review].
    Author: Huang B, Chen FG, Zhuang J, Zheng WC, Zhu WY, Zhang QC, Wang SH, Guo CM, Xie CM.
    Journal: Zhonghua Jie He He Hu Xi Za Zhi; 2017 Sep 12; 49(9):697-702. PubMed ID: 28910916.
    Abstract:
    Objective: To study the clinical manifestations, pathological features, diagnosis, differential diagnosis, treatment and prognosis of primary tracheobronchial or pulmonary malignant glomus tumor (MGT). Methods: 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. Results: 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. Conclusions: 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. 目的： 探讨原发性气管和肺恶性血管球瘤(MGT)的临床表现、病理特征、诊断、鉴别诊断、治疗及预后。 方法： 回顾性分析中山大学附属汕头医院呼吸内科2015年5月收治的1例经病理诊断的气管和肺MGT患者的临床资料并进行相关文献复习。分别以"气管恶性血管球瘤"和"肺恶性血管球瘤"为检索词，检索万方数据库、中国期刊全文数据库和维普数据库，分别以"tracheal maglinant glomus tumor"和"pulmonary maglinant glomus tumor"为检索词检索PubMed、Embase、Ovid及Cochrane数据库，检索时间为1975年1月至2016年12月。 结果： 患者男，47岁，因"咳嗽、胸闷、气促3 d"入院。胸部CT示气管下段内可见直径为2.5 cm的软组织肿块影，管腔变窄，双肺可见多发结节影，临床诊断考虑甲状腺癌并肺内多发转移。支气管镜下行气管肿物射频消融加冷冻治疗术，切除气管内肿物，病理诊断为气管MGT。1个月后气管内肿瘤复发，再次切除气管内肿瘤，术后患者拒绝行放疗和化疗，1个月后由于双肺肿瘤增多、增大致病情进展迅速而死亡。最终，该患者诊断为原发性气管和肺MGT。共检索到相关文献14篇，15例气管或肺MGT患者，均为个案报告。加上本例共16例，其中男9例，女7例，年龄9～74岁，平均为49岁。胸部CT表现为气道肿物或肺部占位病变，临床表现无特异性。 结论： 原发性气管和(或)肺的MGT是一种罕见病，具有高度侵袭性，易出现局部复发和转移，极易误诊、漏诊。确诊主要依据病理诊断。治疗以肺叶切除或气管节段切除为主。该病预后较差。.
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