摘要
目的探讨肺原发性黏液样软骨肉瘤(PCL)的临床病理特点、诊断和鉴别诊断要点以及治疗和预后。方法报道1例发生于左肺上叶的骨外黏液样软骨肉瘤,观察其组织形态特点及免疫组化结果,并结合文献复习,总结该类肿瘤的临床病理特点及预后。结果患者男性,66岁。左肺上叶占位两年,切除标本呈结节状,大小8.5cm×6cm×4cm。镜下见大部分区域为卵圆形及短梭形的细胞,呈条索状及网状排列,瘤细胞异型性明显,胞质嗜酸性,部分细胞胞质空泡状,核仁明显,核分裂9个/10HPF,可见坏死。局部有横纹肌样及上皮样分化。富于黏液软骨样基质,未见明显钙化。免疫组化:肿瘤细胞Vimentin(弥漫强+)、Bcl-2(+)、FLI-1(+)、Syn(+);S-100(软骨部分+)、MyoD1(部分+)、CD117(局灶+)、NSE(局灶+)、Desmin(局灶+)、CK(个别细胞+)、TTF-1(个别细胞+)、EMA(个别细胞+)、CD34(血管+);ERG(-)P40(-)、CK7(-)、CK5/6(-)、P63(-)、Napsin-A(-)、S-100(-)、CK18(-);Ki67(+80%)。最终诊断:肺原发的骨外黏液样软骨肉瘤。结论肺原发的骨外黏液样软骨肉瘤十分罕见,此类肿瘤具有独特的组织学形态特点,免疫组化有助于与肺原发的多种软组织肿瘤和肉瘤样癌鉴别。该肿瘤生长缓慢,常无特征性的临床表现,术后常复发和晚期可转移,必要时可辅以放疗及化疗。
Objective To investigate the clinicopathological features,diagnosis,differential diagnosis,treatment and prognosis of primary extraskeletal myxoid chondrosarcoma of the lung.Methods A case of myxoid chondrosarcoma of the left upper lobe of the lung was reported.The histological features and immunohistochemical results were observed.The clinicopathological features and prognosis of this tumor were summarized.Results The patient was 66 years old.A mass in the upper lobe of left lung was found two years ago,and the specimen was nodular,sized 8.5 cm × 6 cm × 4 cm. Microscopically,most of the cells were oval and short spindle-shaped,arranged in cords and networks.The tumor cells were characterized by obvious atypia,eosinophilic cytoplasm,vacuole-like cytoplasm in some cells,obvious nucleolus,less intercellular mucoid matrix,mitosis ( 9 /10 HPF) and necrosis.There were Thabdoid and epithelioid differentiation.The central part of the tumor was rich in mucoid cartilage like matrix with less mucinous components,sparse tumor cells and no obvious calcification.Immunohistochemically,tumor cells were positive for vimentin,Bcl-2,FLI-1,Syn,S-100,MyoD1, CD117,NSE,Desmin,CK,TTF-1,EMA,CD34 ( blood vessel),but negative for ERG,P40,CK7,CK5 /6,P63, Napsin-A,S-100,and CK18;Ki67 index was 80%.Final diagnosis was primary extraskeletal myxoid chondrosarcoma of lung.Conclusion Primary extraskeletal myxoid chondrosarcoma of the lung is very rare.This tumor has unique histological characteristics.Immunohistochemistry is helpful in differentiating from various soft tissue tumors and sarcomatoid carcinoma of the lung.The tumor grows slowly and often has no characteristic clinical manifestations.It often recurs and metastases late after surgery.Radiotherapy and chemotherapy can be used as a supplement if necessary.
作者
刘有
张晓欢
宋志刚
王湛博
LIU You;ZHANG Xiao-huan;WANG Zhan-bo(Department of Pathology,Hainan Branch of PLA General Hospital,Sanya 572013,China;Department of Pathology,PLA General Hospital,Beijing 100853,China;Department of Radiology,Hainan Branch of PLA General Hospital,Sanya 572013,China)
出处
《诊断病理学杂志》
2019年第8期523-527,共5页
Chinese Journal of Diagnostic Pathology
关键词
骨外黏液样软骨肉瘤
肺
临床病理
免疫组化
Myxoid chondrosarcoma
Lung
Clinicopathology
Immunohistochemistry
