Aiman Aiffa, Zahir Zafirah, Makhdoomi Rumana, Farooq Roudah Binti, Jeelani Tazeen
Sher-I-Kashmir Institute of Medical Sciences, Srinagar, Jammu and Kashmir, India.
Lung India. 2023 May-Jun;40(3):222-226. doi: 10.4103/lungindia.lungindia_204_22.
Primary pulmonary adenoid cystic carcinoma is a rare disease. Its clinico-pathological features, course of disease, therapeutic strategy, and survival data have not been fully elucidated. We aimed to study the clinicopathological characteristics of primary pulmonary adenoid cystic carcinomas in north India.
This study was a retrospective, single-centre, cohort study. The database of the hospital was searched for a period of seven years to identify all patients with primary pulmonary adenoid cystic carcinoma.
Out of 6050 lung tumors, 10 were primary adenoid cystic carcinomas. The mean age of diagnosis was 42 (±12) years. Six patients had the lesion in the trachea, main bronchus or truncus intermedius and four had parenchymal lesions. Seven patients had resectable tumours. Three patients achieved an R0 resection, two achieved R1 resection and two had R2 resection. On histopathological examination, almost all of the patients had cribriform pattern. Only four patients (57.1%) showed positivity for TTF-1 staining. The five-year survival of patients with resectable tumour and un-resectable tumour was 85.7% and 33.3%, respectively (P = 0.01). The predictors of poor outcome included non-operability of the tumour, presence of metastasis at the time of diagnosis, and macroscopically positive tumour margin during surgery.
Primary pulmonary adenoid cystic carcinoma is a unique and rare tumour that affects relatively younger individuals, males and females, as well as smokers and non-smokers equally. The features of bronchial obstruction are the most common. Surgery is the primary modality of treatment and completely resectable lesions have the best prognosis.
原发性肺腺样囊性癌是一种罕见疾病。其临床病理特征、疾病进程、治疗策略及生存数据尚未完全阐明。我们旨在研究印度北部原发性肺腺样囊性癌的临床病理特征。
本研究为一项回顾性、单中心队列研究。检索医院数据库七年期间的所有原发性肺腺样囊性癌患者。
在6050例肺部肿瘤中，10例为原发性腺样囊性癌。诊断时的平均年龄为42（±12）岁。6例患者病变位于气管、主支气管或中间段支气管，4例为实质病变。7例患者肿瘤可切除。3例患者实现R0切除，2例实现R1切除，2例为R2切除。组织病理学检查显示，几乎所有患者均为筛状结构。仅4例患者（57.1%）TTF-1染色呈阳性。可切除肿瘤患者和不可切除肿瘤患者的五年生存率分别为85.7%和33.3%（P = 0.01）。预后不良的预测因素包括肿瘤不可切除、诊断时存在转移以及手术时肉眼可见肿瘤切缘阳性。
原发性肺腺样囊性癌是一种独特的罕见肿瘤，对相对年轻的个体、男性和女性以及吸烟者和非吸烟者影响相同。支气管阻塞特征最为常见。手术是主要治疗方式，完全可切除的病变预后最佳。
