Primary tracheo-bronchial adenoid cystic carcinoma: A surgical series with literature review.

IF 0.8 Q4 RESPIRATORY SYSTEM Respirology Case Reports Pub Date : 2025-01-22 eCollection Date: 2025-01-01 DOI:10.1002/rcr2.70062
Kaouther Ben Amara, Imen Bouassida, Rahma Ayadi, Mariem Affes, Amina Abdelkbir, Mahdi Abdennadher, Salma Ben Othmen, Ayda Ayadi-Kaddour, Hazem Zribi, Adel Marghli
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Abstract

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.

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原发性气管支气管腺样囊性癌:一例外科病例并文献回顾。
气管支气管腺样囊性癌(TBACC)是一种罕见的疾病。其治疗主要是手术。我们在此描述临床和准临床类型的TBACC,以及他们的手术治疗和预后。我们对1994年至2022年间接受手术的8例患者进行了回顾性研究,这些患者的最终病理检查结果为原发性TBACC。共有3名男性和5名女性,平均年龄为44岁。支气管镜检查发现气管管腔在颈部(2例)、气管中部(2例)和气管远端及隆突(4例)处有一芽状形成。支气管吻合切除术和套筒肺切除术是最常见的手术方法。5例患者手术切除R0。2例R1手术切除不全患者行手术后辅助放疗。所有原发性ACC患者的5年总生存率为72%。TBACC是一种罕见的低中度恶性肿瘤。选择合适的手术方式是获得良好预后的挑战。
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来源期刊
Respirology Case Reports
Respirology Case Reports RESPIRATORY SYSTEM-
CiteScore
1.40
自引率
0.00%
发文量
178
审稿时长
8 weeks
期刊介绍: Respirology Case Reports is an open-access online journal dedicated to the publication of original clinical case reports, case series, clinical images and clinical videos in all fields of respiratory medicine. The Journal encourages the international exchange between clinicians and researchers of experiences in diagnosing and treating uncommon diseases or diseases with unusual presentations. All manuscripts are peer-reviewed through a streamlined process that aims at providing a rapid turnaround time from submission to publication.
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