恶性孤立性纤维性肿瘤4例临床病理分析并文献复习
| 作者: |
1邵云,
1王海艳,
1王晶晶,
1许春伟,
1吴永芳,
1王怀涛,
1张博,
1李晓兵
1 军事医学科学院附属医院理科，北京 100071 |
| 通讯: |
邵云
Email: shysep@163.com |
| DOI: | 10.3978/j.issn.2095-6959.2015.06.019 |
摘要
目的：探讨恶性孤立性纤维性肿瘤(malignant solitary fibrous tumor，MSFT)临床病理特点、诊断和鉴别诊断及治疗预后。方法：对4例MSFT回顾并分析其临床病理特点，并复习相关文献。
结果：男性2例，女性2例，发病年龄43~64岁。肿瘤部位分别为胸腔内、乙状结肠系膜、右肩部、左足。镜下肿瘤呈细胞丰富区与稀疏区相间的特点，局灶形态类似血管外皮瘤。瘤细胞梭形或短梭形，异型性明显，核分裂像易见，局灶可见坏死。免疫组化：瘤细胞表达Vim、CD34、CD99、Bcl-2，不表达CK、S-100。结论：MFST少见，确诊依靠形态学和免疫组织化学，需与血管外皮瘤、纤维肉瘤、滑膜肉瘤、恶性间皮瘤及恶性外周神经鞘瘤相鉴别。MFST易复发和转移，手术完整切除是其主要治疗手段，需长期随访。
关键词：
恶性孤立性纤维性肿瘤
临床病理特征
诊断
鉴别诊断
结果：男性2例，女性2例，发病年龄43~64岁。肿瘤部位分别为胸腔内、乙状结肠系膜、右肩部、左足。镜下肿瘤呈细胞丰富区与稀疏区相间的特点，局灶形态类似血管外皮瘤。瘤细胞梭形或短梭形，异型性明显，核分裂像易见，局灶可见坏死。免疫组化：瘤细胞表达Vim、CD34、CD99、Bcl-2，不表达CK、S-100。结论：MFST少见，确诊依靠形态学和免疫组织化学，需与血管外皮瘤、纤维肉瘤、滑膜肉瘤、恶性间皮瘤及恶性外周神经鞘瘤相鉴别。MFST易复发和转移，手术完整切除是其主要治疗手段，需长期随访。
Clinicopathologic analyses of malignant solitary fibrous tumor and review of the literature
CorrespondingAuthor: SHAO Yun Email: shysep@163.com
DOI: 10.3978/j.issn.2095-6959.2015.06.019
Abstract
Objective: To investigate the clinicopathologic characteristics, diagnosis and differential diagnosis, treatment and prognosis of malignant solitary fibrous tumor (MSFT). Methods: The clinical features, histological features and immunohistochemical features of the five cases diagnosed with MSFT were analyzed, the related literatures were reviewed. Results: Of the 4 patients with MSFT, two were males and two was female, aged 43~64 years. The neoplasms were located in intrathoracic cavity, mesentery of sigmoid colon, right shoulder and left foot. Histologically the tumor was composed of areas of altemating hypercellularity and hypocellularity. In some areas, hemangiopericytoma-like structure was present. The tumor cells were spindle to short-spindle shaped with marked nuclear atypia and increased mitotic activity. Focal hemorrhage and necrosis was present. Immunohistochemically, Vim, Bcl-2, CD99 and CD34 were positive, while CK and S-100 were negative in all the cases.
Conclusion: MSFT is a rare mesenchymal tumor, and the diagnosis mainly relies on the pathological morphology and immunohistochemistry. To avoid misdiagnosis, it is important for us to be fully aware of this tumor.
Conclusion: MSFT is a rare mesenchymal tumor, and the diagnosis mainly relies on the pathological morphology and immunohistochemistry. To avoid misdiagnosis, it is important for us to be fully aware of this tumor.