摘要
目的探讨恶性孤立性纤维性肿瘤（MSFT）的临床病理学特征、诊断和鉴别诊断、分子遗传学、治疗及预后。方法报道4例罕见的MSFT,并结合文献进行复习。结果女性1例,男性3例,年龄29-65岁,中位年龄46岁。其中中枢神经系统1例,临床表现为头痛、头晕、恶心等;腹腔2例,无明显症状,体检发现;骶前1例,临床表现为进行性二便障碍1年;肿块直径3.5-13 cm,平均9 cm。镜检示细胞稀疏区与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割。密集区肿瘤细胞丰富,呈束状、旋涡状排列,可见血管外皮瘤样结构;瘤细胞呈梭形、短梭形,核染色质较粗,异型性显著,有肿瘤性坏死、较多核分裂（〉4/10HPF）,局部出血。免疫表型示肿瘤细胞bcl-2、CD99、CD34和vimentin弥漫（＋）,SMA、h-caldesmon局灶（＋）,Ki-67增值指数20%-40%;EMA、CD117和S-100（-）。结论 MSFT非常罕见,确诊主要依赖病理形态学和免疫表型,需要与滑膜肉瘤、恶性外周神经鞘膜瘤、纤维肉瘤和恶性纤维组织瘤等鉴别。MSFT预后不良,易复发和转移,手术切除后,应注意随访。
Purpose To explore the clinicopathologic characteristics,histogenesis,diagnosis and differential diagnosis,molecular genetics,treatment and prognosis of malignant solitary fibrous tumor（ MSFT）. Methods Four cases of MSFT were reported and the literatures reviewed. Results The four cases occurred in 1 woman and 3 men,aged from 29 to 63years（ median 46）,and measured 3. 5 - 13 cm（ median 9 cm） in size. 1 was located in central nervous system,presented with headache, dizziness, nausea etc; the other two case were situated in the intrathoracic cavity without obvious symptoms,discovered by physical examination; while the other case was a man with 1-year history of slowly progressive obstructions of stool and pee. Microscopically,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 /10HPF）,coagulative necrosis and focal hemorrhage. Immunohistochemically,the tumor cells were diffusely positive for bcl-2,CD99,CD34,and vimentin; focally positive for MSA,SMA and h-caldesmon,but negative for EMA,CD117 and S-100.Ki-67 labelling index was 20% - 40%. Conclusions The MSFT is extremely rare,and its diagnosis is mainly rely on its pathological morphology and immunohistochemical profiles. Differential diagnosis includes those spindle-shaped cell tumors,such as synovial sarcoma,malignant peripheral nerve sheath tumors,fibrosarcoma malignant fibrous histocytoma and so on. MSFT tends to recur and even metastasize,complete resection remains the major treatment modality and close follow-up is recommended.
出处
《诊断病理学杂志》
CSCD
2015年第8期464-466,470,共4页
Chinese Journal of Diagnostic Pathology
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
