肺透明细胞瘤的临床病理学特征分析

Analysis of clinicopathological features of clear cell tumor of the lung

目的分析肺透明细胞瘤(CCTL)的临床病理学特征。方法收集解放军总医院第一医学中心和解放军总医院海南医院病理科2008年8月至2019年8月病例共9例CCTL患者的临床资料、组织形态特点、免疫组织化学染色及特殊染色结果,分析其临床病理学特征。结果本组病例男3例,女6例,年龄28~70岁(平均52.2岁)。肿瘤均位于肺的周边部,8例为单发,1例为多发(24个结节)。肿瘤最大径0.5~5.5 cm不等,呈圆形或椭圆形,与周围肺组织边界清楚,无完整的纤维性包膜。镜下见肿瘤细胞卵圆形、短梭形或多角形,核仁明显,无核分裂像;瘤细胞多围绕薄壁血管呈片状分布,血管周围间质可有透明变性,未见坏死。免疫组织化学染色:肿瘤细胞波形蛋白弥漫阳性,血管内皮细胞标记CD34、黑色素A、抗黑色素瘤特异性单抗(HMB45)及酸性钙结合蛋白(S-100)不同程度阳性,广谱细胞角蛋白(CK)、上皮膜抗原(EMA)、平滑肌肌动蛋白(SMA)、结蛋白、共同型急性淋巴细胞白血病抗原(CD10)、配对盒基因8(PAX-8)及肌调节蛋白(Myo-D1)均阴性,增殖细胞核抗原(Ki-67)阳性指数低。过碘酸雪夫染色(PAS染色)阳性,淀粉酶消化糖原后PAS染色(d-PAS染色)阴性。预后方面,9例均经手术切除,术后随访5~137个月,除1例失访,其余8例均存活,未出现本病的复发及转移。结论CCTL是一种罕见的良性肿瘤,大多数为单发,极少数可以多发;病理组织学形态及免疫组织化学染色有助于诊断及鉴别诊断。手术完整切除后,预后良好。但当肿瘤出现恶性的组织学特点时,需密切随访。

Objective To analyze the clinicopathological features of clear cell tumor of the lung(CCTL).Methods A total of 9 cases were collected from August 2008 to August 2019 in the Department of Pathology of the First Medical Center of PLA General Hospital and Hainan Hospital of PLA General Hospital.Their clinical data,pathological characteristics,immunohistochemical staining and special staining results were summarized and analyzed.Results There were 3 males and 6 females,aged from 28 to 70 years(average 52.2 years).All tumors were located in the peripheral part of the lung,and were solitary in 8 cases,and multiple(24 nodules)in 1 case.The lesion was round or oval,with clear boundary.The diameter of the nodule was 0.5-5.5 cm.Histologically,the tumor cells were oval,short fusiform or polygonal,with obvious nucleoli.The tumor cells were mostly distributed in sheet around thin-walled vessels,and there was hyaline degeneration around the blood vessels.Neither necrosis nor mitosis could be seen.Immunohistochemical staining showed tumor cells were diffusely positive for Vimentin,and CD34,Melan-A,specific monoclonal antibody against melanoma(HMB45)and S-100 were positive with different degrees.Broad spectrum cytokeratin(CK),epithelial membrane antigen(EMA),smooth muscle actin(SMA),desmin,CD10,paired box gene 8(PAX-8)or myomodulatory protein(Myo-D1)were all negative.The positive index of the proliferating cell nuclear antigen(Ki-67)was low.Schiff dyeing with periodate(PAS)staining was positive,PAS staining of glycogen digested by amylase(d-PAS)staining was negative.All the tumors in the nine cases were resected and patients were followed up for 5-137 months.Except 1 case was lost for follow-up,the other 8 cases survived without recurrence or metastasis of the disease.Conclusions CCTL is a rare benign tumor,most of which are single,few of which can be multiple;histopathological characteristics and immunohistochemical staining are helpful for diagnosis and differentiated diagnosis.After complete resection,the prognosis was good.However,when histological features indicating malignancy,intense follow-up should be considered.