Abstract：
Non-small cell lung carcinoma with predominantly clear cell features is a rare histologic presentation of lung carcinoma. We have examined 31 cases of lung carcinomas showing extensive clear cell features. The patients were 10 women and 21 men aged 47-92 years (mean: 70 years). The tumors showed a predilection for the right upper and lower lobes and measured from 0.8 to 9.5 cm (mean: 4.2 cm). By immunohistochemistry, 9 cases were typed as adenocarcinoma, 19 cases as squamous cell carcinoma, and 3 showed a \"null\" phenotype with complete loss of markers for adenocarcinoma or squamous cell carcinoma. Most cases that typed as adenocarcinoma showed a solid growth pattern. A subset of the solid adenocarcinoma cases showed a distinctive \"pseudosquamous\" morphology. Next-generation sequencing was performed in 20 cases and showed a variety of molecular alterations. The most common abnormalities were found in the TP53 gene (9 cases), FGFR gene family (8 cases), KRAS (5 cases), AKT1 (5 cases), and BRAF (3 cases). Clinical follow-up was available in 21 patients; 16/21 patients died of their tumors from 6 months to 12 years after initial diagnosis (mean: 4.2 years, median: 1.5 years). Four patients were alive and well from 4 to 27 years (mean: 11.5 years, median: 7.5 years); all were pathologic stage 1 or 2. NSCLC with clear cell features can display aggressive behavior and needs to be distinguished from various other tumors of the lung that can show clear cell morphology. The identification of targetable molecular alterations in some of these tumors may be of value for therapeutic management.
摘要：
具有主要透明细胞特征的非小细胞肺癌是肺癌的罕见组织学表现。我们检查了31例表现出广泛透明细胞特征的肺癌。患者为10名女性和21名男性，年龄为47-92岁（平均：70岁）。肿瘤显示右上叶和下叶好发，测量范围为0.8至9.5cm（平均：4.2cm）。通过免疫组织化学，9例分型为腺癌,鳞状细胞癌19例，和3显示“空”表型，完全丧失了腺癌或鳞状细胞癌的标志物。大多数类型为腺癌的病例显示出稳固的生长模式。实体腺癌病例的一部分显示出独特的“假鳞状”形态。在20例病例中进行了下一代测序，并显示出各种分子改变。最常见的异常是TP53基因（9例），FGFR基因家族（8例），KRAS（5例）AKT1（5例），和BRAF（3例）。21例患者获得了临床随访；16/21例患者在初次诊断后6个月至12年死于肿瘤（平均：4.2年，中位数：1.5年）。4例患者在4至27年之间存活良好（平均：11.5年，中位数：7.5年）；全部为病理阶段1或2。具有透明细胞特征的NSCLC可以表现出攻击行为,并且需要与可以表现出透明细胞形态的各种其他肺肿瘤区分开。在这些肿瘤中的一些中鉴定可靶向的分子改变对于治疗管理可能是有价值的。
