Abstract：
BACKGROUND: Primary pulmonary salivary gland-type tumours (PPSGT) are rare lung neoplasms arising from submucosal seromucinous glands in the central airway.
RESULTS: We retrospectively analysed the clinicopathological features of 111 PPSGTs diagnosed at our institute between 2003 and 2021. The mean age at diagnosis was 43.8 years(range 6-78 years) and a male-to-female ratio of 2:1. On imaging, 92 % of cases had centrally located tumours and 37.3 % were early stage. The histopathological types included 70 cases (63 %) of mucoepidermoid carcinoma (MEC), 31 cases (27.7 %) of adenoid cystic carcinoma (ADCC), two cases of myoepithelial carcinoma, one case each of acinic cell carcinoma (ACC), clear cell carcinoma (CCC), epithelial myoepithelial carcinoma (EMC) and 5 others [including adenocarcinoma of minor salivary gland origin(n = 3), carcinoma with sebaceous differentiation(n = 1) and poorly differentiated carcinoma of salivary gland type(n = 1)]. The size of the tumours found in the resection specimens ranged from 1 cm to 13 cm, with an average size of 4.9 cm. High-risk attributes such as lymphovascular invasion (LVI), perineural invasion (PNI), pleural involvement, positive resection margins, and nodal metastasis were identified in 15.3 %, 15.3 %, 13.6 %,15.2 % and 6.7 % of cases, respectively. These attributes were found to be more frequent in ADCC than in MEC. Surgery was the main treatment modality [68/84 (80 %) cases]. ADCC cases had more recurrence and distant metastasis than MEC cases. The 3- year overall-survival (OS) and recurrence-free survival(RFS) were better in patients with age lesser than 60 years(p-value <0.0001), low pT stage (p-value 0.00038) and lower grade of MEC(p-value-0.0067).
CONCLUSIONS: It is crucial to have an acquaintance with the morphologic spectrum and immunophenotypic characteristics of PPSGT to recognize them in this unusual location. In tandem, it is crucial to differentiate them from conventional primary non-small cell lung carcinoma, as the management protocols and prognostic implications differ significantly.
RESULTS: We retrospectively analysed the clinicopathological features of 111 PPSGTs diagnosed at our institute between 2003 and 2021. The mean age at diagnosis was 43.8 years(range 6-78 years) and a male-to-female ratio of 2:1. On imaging, 92 % of cases had centrally located tumours and 37.3 % were early stage. The histopathological types included 70 cases (63 %) of mucoepidermoid carcinoma (MEC), 31 cases (27.7 %) of adenoid cystic carcinoma (ADCC), two cases of myoepithelial carcinoma, one case each of acinic cell carcinoma (ACC), clear cell carcinoma (CCC), epithelial myoepithelial carcinoma (EMC) and 5 others [including adenocarcinoma of minor salivary gland origin(n = 3), carcinoma with sebaceous differentiation(n = 1) and poorly differentiated carcinoma of salivary gland type(n = 1)]. The size of the tumours found in the resection specimens ranged from 1 cm to 13 cm, with an average size of 4.9 cm. High-risk attributes such as lymphovascular invasion (LVI), perineural invasion (PNI), pleural involvement, positive resection margins, and nodal metastasis were identified in 15.3 %, 15.3 %, 13.6 %,15.2 % and 6.7 % of cases, respectively. These attributes were found to be more frequent in ADCC than in MEC. Surgery was the main treatment modality [68/84 (80 %) cases]. ADCC cases had more recurrence and distant metastasis than MEC cases. The 3- year overall-survival (OS) and recurrence-free survival(RFS) were better in patients with age lesser than 60 years(p-value <0.0001), low pT stage (p-value 0.00038) and lower grade of MEC(p-value-0.0067).
CONCLUSIONS: It is crucial to have an acquaintance with the morphologic spectrum and immunophenotypic characteristics of PPSGT to recognize them in this unusual location. In tandem, it is crucial to differentiate them from conventional primary non-small cell lung carcinoma, as the management protocols and prognostic implications differ significantly.
摘要：
背景：原发性肺涎腺型肿瘤（PPSGT）是由中央气道粘膜下浆膜粘液腺引起的罕见肺部肿瘤。
结果：我们回顾性分析了2003年至2021年间在我们研究所诊断的111例PPSGT的临床病理特征。诊断时的平均年龄为43.8岁（范围6-78岁），男女比例为2：1。在成像方面，92%的病例有位于中央的肿瘤，37.3%为早期。组织病理学类型包括70例（63%）粘液表皮样癌（MEC），腺样囊性癌(ADCC)31例(27.7%),2例肌上皮癌，腺泡细胞癌(ACC)各1例,透明细胞癌（CCC），上皮肌上皮癌（EMC）和其他5个[包括小唾液腺起源的腺癌（n=3），皮脂腺分化癌（n=1）和涎腺低分化癌（n=1）]。切除标本中发现的肿瘤大小从1厘米到13厘米不等，平均尺寸为4.9厘米。高风险属性，如淋巴管浸润（LVI），神经周浸润（PNI），胸膜受累,切缘阳性,淋巴结转移占15.3%，15.3%,13.6%,15.2%和6.7%的病例,分别。发现这些属性在ADCC中比在MEC中更频繁。手术是主要的治疗方式[68/84（80％）例]。ADCC病例较MEC病例有更多的复发和远处转移。年龄小于60岁的患者的3年总生存期（OS）和无复发生存期（RFS）更好（p值<0.0001），低PT阶段（P值0.00038）和较低等级的MEC（P值0.0067）。
结论：了解PPSGT的形态谱和免疫表型特征以识别其异常位置是至关重要的。串联，将它们与常规原发性非小细胞肺癌区分开来至关重要，因为管理方案和预后影响显著不同。
结果：我们回顾性分析了2003年至2021年间在我们研究所诊断的111例PPSGT的临床病理特征。诊断时的平均年龄为43.8岁（范围6-78岁），男女比例为2：1。在成像方面，92%的病例有位于中央的肿瘤，37.3%为早期。组织病理学类型包括70例（63%）粘液表皮样癌（MEC），腺样囊性癌(ADCC)31例(27.7%),2例肌上皮癌，腺泡细胞癌(ACC)各1例,透明细胞癌（CCC），上皮肌上皮癌（EMC）和其他5个[包括小唾液腺起源的腺癌（n=3），皮脂腺分化癌（n=1）和涎腺低分化癌（n=1）]。切除标本中发现的肿瘤大小从1厘米到13厘米不等，平均尺寸为4.9厘米。高风险属性，如淋巴管浸润（LVI），神经周浸润（PNI），胸膜受累,切缘阳性,淋巴结转移占15.3%，15.3%,13.6%,15.2%和6.7%的病例,分别。发现这些属性在ADCC中比在MEC中更频繁。手术是主要的治疗方式[68/84（80％）例]。ADCC病例较MEC病例有更多的复发和远处转移。年龄小于60岁的患者的3年总生存期（OS）和无复发生存期（RFS）更好（p值<0.0001），低PT阶段（P值0.00038）和较低等级的MEC（P值0.0067）。
结论：了解PPSGT的形态谱和免疫表型特征以识别其异常位置是至关重要的。串联，将它们与常规原发性非小细胞肺癌区分开来至关重要，因为管理方案和预后影响显著不同。
