肺透明细胞糖瘤的临床病理特征分析

Analysis of clinicopathological features of lung clear cell sugar tumor

目的分析肺透明细胞糖瘤(CCST)的临床病理特征。方法回顾性分析6例CCST患者的临床表现、病理特点、治疗及预后。结果6例CCST患者,男、女各3例,年龄28~78岁,均为体检时偶然发现。CT显示肿瘤均位于肺周边部,5例病灶为单发,1例因肺多发结节考虑肺癌转移。肿瘤标本与周围肺组织界限清楚,切面灰红或灰白。镜下显示肿瘤由巢状、腺泡状、器官样或片状排列的透明细胞组成,肿瘤细胞巢之间为丰富、纤细的血管网。多发结节者病理证实为并存肺癌肉瘤,组织学表现为高级别胎儿型腺癌与未分化肉瘤。免疫组化结果显示,肿瘤细胞表达抗黑素瘤特异性单抗、抗平滑肌抗体、波形蛋白、黑色素A和CD34。肺癌肉瘤中腺癌成分局灶表达甲状腺转录因子1、突触素、嗜铬素A、甲胎蛋白和婆罗双树样基因4。6例患者均行楔形肺切除术。术后随访2~63个月,均未出现复发和转移。结论CCST是一种罕见的具有血管周上皮样细胞分化的肿瘤,需要结合常规病理及免疫组化才能确诊。

Objective To analyze the clinicopathological features of the lung clear cell sugar tumor(CCST).Methods The clinical data of 6 patients with CCST were analyzed retrospectively,which included clinical manifestations,pathological features,treatment and prognosis.Results Six patients were aged from 28 to 78 years old,of whom 3 cases were male and 3 cases were female.The clinical manifestations were not specific and all the tumors were found by physical examination.CT image showed that all the tumors located in the peripheral parts of the lungs.Five tumors were single solitary lesion.One case had multiple pulmonary nodules and was considered to be malignant neoplasm of metastasis.Grossly,the well-demarcated nodules showed grey red to white in section.Microscopically,the tumor was composed of clear cells arranged in nests,glandular,organoid or sheets.There were abundant vascular networks between the nests of tumor cells.The patient with multiple nodules was pathologically confirmed to be concurrent lung carcinosarcoma with high-grade fetal adenocarcinoma and undifferentiated sarcoma.Immunohistochemically,CCST was positive for HMB45,SMA,vimentin,Melan A and CD34.The adenocarcinoma showed focally positive for TTF-1,Syn,CgA,AFP and SALL4.All the patients underwent wedge-shaped pneumonectomy and were followed up from 2 to 63 months without recurrence or metastasis.Conclusion CCST is a rare tumor with perivascular epithelioid cell differentiation and the diagnosis needs to be confirmed by routine pathology and immunohistochemistry.