Wu Qiang, Xu Feng
Lung Cancer Center and Institute, West China Hospital, Sichuan University, Chengdu 610041, Sichuan Province, China.
World J Clin Cases. 2021 Nov 6;9(31):9535-9541. doi: 10.12998/wjcc.v9.i31.9535.
Adenoid cystic carcinoma (ACC) occurs mainly in the head and neck. Tracheal ACC (TACC) is uncommon. Primary resection is recommended as the main treatment of choice, and radiotherapy is considered for residual disease in the postoperative setting. Definitive radiotherapy is an alternative approach to cure unresectable TACC. As the status of radiosensitivity in TACC is uncertain, the evidence for radiotherapy in unresectable TACC is not well established, especially in terms of the optimal dosage and its response evaluation. Herein, we report a case of locally advanced TACC.
A 49-year-old woman was diagnosed with TACC, which included a range of lesions arising in the upper trachea extending caudally 2 cm to 7 cm of the glottis. She was treated with definitive radiotherapy, given the low likelihood of complete resection of the disease. Due to the indolent growth and the propensity for infiltration along the airways, the scheduled radiation dose of 76 Gy in 38 fractions with 6-MV X-ray delivered by intensity-modulated radiotherapy was conducted to the primary tumor volume. After irradiation of 40 Gy, the patient's dyspnea on exertion was dramatically relieved and bronchoscopy revealed that the previous large polypoid intra-luminal mass was significantly eliminated, with near-complete response. The patient completed two phases of scheduled radiotherapy, and acute reactions to treatment included subjective chest tightness and grade 2 esophagitis, managed medically. After 5 years of treatment, the patient is alive without recurrent disease, and there were no serious late radiation esophagus and lung damage, with only slight dysphagia without perforation and fistula.
Taken together, TACC is uncommon and the treatment of unresectable TACC is challenging. This case indicated that patients with unresectable TACC who rapidly respond to radiation may benefit from primary radical radiotherapy. Radiotherapy may be considered an effective alternative treatment modality.
腺样囊性癌（ACC）主要发生于头颈部。气管腺样囊性癌（TACC）较为罕见。推荐以手术切除作为主要治疗方式，术后残留病灶可考虑放疗。根治性放疗是无法切除的TACC的一种替代治疗方法。由于TACC的放射敏感性状况尚不确定，因此关于无法切除的TACC放疗的证据并不充分，尤其是在最佳剂量及其疗效评估方面。在此，我们报告一例局部晚期TACC病例。
一名49岁女性被诊断为TACC，病变范围包括上气管，向下延伸至声门以下2 cm至7 cm处。鉴于疾病完全切除的可能性较低，她接受了根治性放疗。由于肿瘤生长缓慢且有沿气道浸润的倾向，采用调强放疗以6兆伏X射线分38次给予原发肿瘤体积76 Gy的预定放射剂量。照射40 Gy后，患者运动时的呼吸困难明显缓解，支气管镜检查显示先前腔内巨大息肉样肿物显著消退，近乎完全缓解。患者完成了两个阶段的预定放疗，治疗的急性反应包括主观胸闷和2级食管炎，通过药物治疗。治疗5年后，患者存活且无疾病复发，无严重的晚期放射性食管和肺部损伤，仅有轻微吞咽困难，无穿孔和瘘管形成。
综上所述，TACC较为罕见，无法切除的TACC的治疗具有挑战性。该病例表明，对放疗迅速反应的无法切除的TACC患者可能从根治性放疗中获益。放疗可被视为一种有效的替代治疗方式。
