Ben Amara Kaouther, Bouassida Imen, Ayadi Rahma, Affes Mariem, Abdelkbir Amina, Abdennadher Mahdi, Ben Othmen Salma, Ayadi-Kaddour Ayda, Zribi Hazem, Marghli Adel
Thoracic Surgery Department Abderrahmen Mami University Hospital-Ariana, Faculty of Medicine of Tunis, University of Tunis El Manar Ariana Tunisia.
Department of Pathology Abderrahmen Mami University Hospital-Ariana, Faculty of Medicine of Tunis, University of Tunis El Manar Ariana Tunisia.
Respirol Case Rep. 2025 Jan 22;13(1):e70062. doi: 10.1002/rcr2.70062. eCollection 2025 Jan.
Tracheo-bronchial adenoid cystic carcinoma (TBACC) is a rare disease. Its treatment is mainly surgical. We herein describe the clinical and para-clinical varieties of TBACC as well as their surgical treatment and prognosis. We conducted a retrospective study of eight patients operated on between 1994 and 2022 and whose definitive pathological examination concluded with primary TBACC. There were three men and five women with an average age of 44 years. The bronchoscopy found a budding formation reducing the tracheal lumen at the cervical (two cases), middle (two case) and distal trachea and carina (four cases). Bronchial anastomosis resection and sleeve pneumonectomy were the most common procedures. Surgical resections R0 were achieved in five patients. Surgery followed by adjuvant radiotherapy was performed in two patients with incomplete surgical resection R1. The overall survival for all patients with primary ACC was 72% at 5 years. TBACC is a rare and low-to-moderate grade malignant tumour. The choice of the surgical procedure and the appropriate approach is challenging to obtain a promising prognosis.
气管支气管腺样囊性癌（TBACC）是一种罕见疾病。其治疗主要以手术为主。我们在此描述TBACC的临床和临床旁特征，以及其手术治疗和预后情况。我们对1994年至2022年间接受手术且最终病理检查确诊为原发性TBACC的8例患者进行了回顾性研究。其中男性3例，女性5例，平均年龄44岁。支气管镜检查发现，在颈部气管（2例）、中部气管（2例）以及气管远端和隆突处（4例）有肿物形成，导致气管腔狭窄。支气管吻合切除术和袖状肺叶切除术是最常见的手术方式。5例患者实现了R0手术切除。2例手术切除不完全（R1）的患者在术后接受了辅助放疗。所有原发性腺样囊性癌患者的5年总生存率为72%。TBACC是一种罕见的低至中度恶性肿瘤。选择合适的手术方式和恰当的治疗方法对于获得良好预后具有挑战性。
