肺原发实体型血管瘤样纤维组织细胞瘤合并肺腺癌1例并文献复习

Primary pulmonary solid-type angiomatoid fibrous histiocytoma with lung adenocarcinoma:A case report and literature review

肺原发血管瘤样纤维组织细胞瘤(angiomatoid fibrous histiocytoma,AFH)是一种少见的软组织肿瘤,同时合并肺腺癌的病例更为罕见。2023年4月十堰市太和医院诊断1例肺原发实体型AFH同时合并肺腺癌的病例。该患者为48岁男性,因复查胸部CT显示右肺多发结节状高密度影入院,临床考虑“肺恶性肿瘤”,行右肺部分切除。肉眼观右肺上叶见一实性结节,结节切面灰白实性质中,与周围界清;右肺下叶见一肿块,肿块切面灰白灰黑、实性、质中,边界欠清。镜下右肺上叶肿瘤细胞界线清楚,可见纤维性假包膜,间质见淋巴细胞、浆细胞和组织细胞浸润,并形成淋巴套样结构;右肺下叶肿瘤细胞呈浸润性生长,排列呈实性、腺管样和片状,以腺泡型和实性型为主,局灶区见不规则形腺体和复杂筛状结构。右肺上叶肿瘤细胞波形蛋白(Vimentin)、CD99、CD68和CD163阳性表达,平滑肌肌动蛋白(smooth muscle actin,SMA)、上皮膜抗原(epithelial membrane antigen,EMA)、CD31和S100部分表达,细胞角蛋白(cytokeratin,CK)-Pan(CK-P)、间变性淋巴瘤激酶(anaplastic lymphoma kinase,ALK)、CD34、CD21、CD23、CD35和结蛋白(Desmin)阴性表达,分子检测出尤文肉瘤断裂区域1基因(EWS RNA binding protein 1,EWSR1)-环腺苷酸反应元件结合蛋白1(cAMP responsive element binding protein 1,CREB1)融合基因;右肺下叶肿瘤细胞CK7、甲状腺转录因子-1(thyroid transcription factor-1,TTF-1)和napsin A阳性表达,CK5/6、P40和P63阴性表达,增殖指数Ki-67为70%。肺原发实体型AFH合并肺腺癌的确诊需结合临床资料并依赖于免疫组织化学和分子检测结果。

Primary pulmonary angiomatoid fibrous histiocytoma(AFH)is a rare soft tissue tumor,and its co-occurrence with lung adenocarcinoma is even more uncommon.In April 2023,Taihe Hospital in Shiyan diagnosed a case of primary pulmonary solid-type AFH with concurrent lung adenocarcinoma.The patient,a 48-year-old male,was admitted after a follow-up chest CT revealed multiple nodular high-density shadows in the right lung.Suspected of having a malignant lung tumor,he underwent a partial right lung resection.Gross examination revealed a solid nodule in the upper right lung lobe with a well-defined border,showing a grayish-white solid cur surface.A mass with a grayish-white to grayish-black solid appearance and indistinct boundary was found in the lower right lung lobe.Microscopically,the upper lobe tumor had a clear boundary with a fibrous pseudocapsule and was infiltrated by lymphocytes,plasma cells,and histiocytes forming a lymphoid cuff-like structure.The lower lobe tumor exhibited infiltrative growth with a predominantly solid and alveolar pattern,irregular glands,and complex cribriform structures in focal areas.Immunohistochemically,the upper lobe tumor cells were positive for vimentin,CD99,CD68,and CD163,partially positive for smooth muscle actin(SMA),epithelial membrane antigen(EMA),CD31,and S100,and negative for cytokeratin(CK)-Pan(CKP),anaplastic lymphoma kinase(ALK),CD34,CD21,CD23,CD35,and Desmin.Molecular testing identified an EWS RNA binding protein 1(EWSR1)-cAMP responsive element binding protein 1(CREB1)fusion gene.The lower lobe tumor cells were positive for CK7,thyroid transcription factor-1(TTF-1),and napsin A,but negative for CK5/6,P40,and P63,with a Ki-67 proliferation index of 70%.A definitive diagnosis of primary pulmonary solid-type AFH with lung adenocarcinoma requires clinical correlation along with immunohistochemical and molecular test results.