Department of Radiation Oncology, The First Hospital of Lanzhou University, Lanzhou, 730000, People's Republic of China.
Radiat Oncol. 2021 Feb 19;16(1):38. doi: 10.1186/s13014-021-01770-0.
Primary tracheal adenoid cystic carcinoma (TACC) is rare and originates from the minor salivary gland. Biologically, TACC results in delayed presentation, and the therapeutic effects of multimodal treatment differ across individuals. This study aimed to review cases of TACC to identify clinical features, imaging modalities, treatment, and patient outcomes across follow-ups.
The PubMed, Web of Science and MEDLINE databases were searched to identify articles reporting cases of TACC. The study variables included in the analysis were patient demographics, biological characteristics, presenting symptoms, imaging modalities, treatments, follow-up times and survival outcomes.
A total of 76 articles and 1252 cases were included in this review. The most common presenting symptom was dyspnoea (86.0%), followed by cough (58.0%). Surgery alone (40.9%), surgery with postoperative radiotherapy (36.4%) and radiotherapy alone (19.2%) were used most frequently treatments modalities. Of the 1129 cases with disease control and survival data, there was no evidence of disease in 78.7%, local recurrence was reported in 3.8%. Distant metastasis rate was 24.9% of 418 reported cases, lung (44.2%) was the most commonly involved organ. The 5, 10 years survival rate of patients treated with surgery alone and surgery with postoperative radiotherapy were 86.4%, 55.6% and 97.3%, 44.4%, respectively.
TACC most common presenting symptoms were dyspnoea, cough and shortness of breath. Surgery alone and surgery with postoperative radiotherapy are predominant treatment modalities. Both seems to provide a good result in term of disease control and long-term survival rate in patients with TACC.
原发性气管腺样囊性癌（TACC）较为罕见，来源于小唾液腺。从生物学角度来看，TACC 表现出较晚的发病特征，且不同个体的多模态治疗效果存在差异。本研究旨在回顾 TACC 病例，以确定临床特征、影像学表现、治疗方法和随访期间的患者结局。
通过检索 PubMed、Web of Science 和 MEDLINE 数据库，查找报道 TACC 病例的文章。分析中纳入的研究变量包括患者人口统计学特征、生物学特征、首发症状、影像学表现、治疗方法、随访时间和生存结局。
本研究共纳入 76 篇文章和 1252 例病例。最常见的首发症状是呼吸困难（86.0%），其次是咳嗽（58.0%）。最常采用的治疗方式是单纯手术（40.9%）、手术联合术后放疗（36.4%）和单纯放疗（19.2%）。在 1129 例有疾病控制和生存数据的病例中，无疾病证据的比例为 78.7%，局部复发率为 3.8%。418 例报告的远处转移率为 24.9%，肺部（44.2%）是最常累及的器官。单纯手术和手术联合术后放疗患者的 5 年、10 年生存率分别为 86.4%、55.6%和 97.3%、44.4%。
TACC 最常见的首发症状是呼吸困难、咳嗽和呼吸急促。单纯手术和手术联合术后放疗是主要的治疗方式。这两种治疗方式似乎都能为 TACC 患者的疾病控制和长期生存率带来良好的结果。
