摘要
目的:探讨恶性孤立性纤维性肿瘤(malignant solitary fibrous tumor,MSFT)的临床病理特征、诊断、鉴别诊断、分子遗传学、治疗及预后。方法:对2例MSFT进行光镜、免疫组织化学研究,并复习相关文献。结果:男性2例,年龄分别为66岁及48岁。发生部位:骶前1例,临床表现为进行性二便障碍3年;后上纵隔1例,临床表现为活动后心慌半个月。肿瘤大小分别为10 cm×9 cm×6 cm及12 cm×10 cm×9 cm。镜检:细胞稀少区和细胞丰富区交替分布,两者之间有粗的玻璃样变胶原纤维分隔。密集区肿瘤细胞丰富,呈束状、漩涡状排列,可见血管外皮瘤样结构;瘤细胞呈梭形、短梭形,核染色质粗,异型性明显,有肿瘤性坏死、较多量核分裂(>4/10 HPF),局部出血。免疫表型:肿瘤细胞CD34,Bcl-2,CD99及Vimentin弥漫阳性;P53部分阳性,上皮细胞膜抗原局灶阳性;ki-67增殖指数20%～30%;S100,Desmin,C K及胶质纤维酸性蛋白阴性。结论:M SFT是一种少见的间叶肿瘤,确诊主要依靠形态学及免疫组织化学,需与血管外皮细胞瘤、恶性纤维组织细胞瘤、纤维肉瘤、滑膜肉瘤、恶性间皮瘤、恶性外周神经鞘膜瘤和脑膜瘤等相鉴别。MSFT的预后不良,易复发和转移,根治性切除是主要治疗手段,术后应长期随访。
Objective To investigate clinicopathologic characteristics,diagnosis and differential diagnosis,molecular genetics,treatment,and prognosis of malignant solitary fibrous tumor（MSFT）. Methods Two cases of MSFT of sacro-anterior and posterosuperior of mediastinum were histologically and immunohistochemically studied and the literatures were reviewed. Results A case is a 66-year-old man with 3-year history of slowly progressive obstructions of stool and pee.Another case is a 48-year-old man with a half of month history of palpitation after exercises.The tumors trom 2 cases were 10 cm×9 cm×6 cm and 12 cm×10 cm×9 cm in size.Under microscopy： the tumor was composed of areas of alternating hypercellularity and hypocellularity which were separated by dense collagenous fibrous stroma.In hypercellular areas,the tumor cells were spindle to short-spindle shaped and arranged in fascicular or storiform pattern.In focal areas,hemangiopericytoma-like structure was presented.The tumor cells showed marked nuclear atypia,increased mitotic activity（4/10 HPF）,coagulative necrosis and focal hemorrhage.Immunohistochemically,the tumor cells were diffusely positive staining for CD34,Bcl-2,CD99,Vimentin,focally positive staining for P53 and epithelial membrane antigen,while negative staining for S100,Desmin,CK,and glial fibrillary acidic protein.Ki-67 labelling index was 20%-30%. Conclusion MSFT is a rare mesenchymal tumor.Its diagnosis mainly relies on morphologic features and immunohistochemical profiles.Differential diagnosis includes spindle-shaped cellular mesenchymal tumors,such as malignant fibrous histocytoma,fibrosarcoma,synovial sarcoma,malignant mesothelioma,malignant peripheral nerve sheath tumor,meningioma and so on.The prognosis of MSFT is poor and tends to recur and even metastasize.The major treatment is to completely resect it by operation and long-term clinical follow-up is necessary.
出处
《国际病理科学与临床杂志》
CAS
2011年第6期491-495,共5页
Journal of International Pathology and Clinical Medicine
关键词
恶性孤立性纤维性肿瘤
临床病理学
诊断
鉴别诊断
malignant solitary fibrous tumor
clinicopathology
diagnosis
differential diagnosis
