文章摘要

恶性孤立性纤维瘤9例临床病理观察

作者: 1陈广业, 2马强, 1朱鸿静, 1张宏利, 1刘家君, 1杨清, 3徐开梅
1 重庆市荣昌区人民医院病理科,重庆 402460
2 第三军医大学大坪医院野战外科研究所病理科,重庆 400042
3 重庆市璧山区人民医院病理科,重庆 402760
通讯: 陈广业 Email: xkmei2015@sina.com
徐开梅 Email: xkmei2015@sina.com
DOI: 10.3978/j.issn.2095-6959.2015.12.022

摘要

目的:探讨恶性孤立性纤维瘤(malignant solitary fibrous tumor,MSFT)的临床病理特点、诊断及 鉴别诊断、治疗预后。方法:回顾性分析9例恶性孤立性纤维瘤临床特点,形态学及免疫表型特 征,以及治疗预后情况。结果:9例恶性孤立性纤维瘤中,男性6例,女性3例,平均54岁。肿瘤 分别位于颅内3例,肺1例,腹膜后1例,左上腹1例,盆腔1例,眼眶1例,左大腿1例。镜下,4例 肿瘤细胞由上皮样细胞和梭形细胞组成,呈疏密交替分布,可见血管外皮细胞瘤样结构及不同程 度的胶原纤维穿插;2例肿瘤细胞为较一致的圆形或多角型上皮样细胞,密集排列无疏密交替; 2例中梭形细胞弥漫成片,缺乏疏密交替,细胞排列呈编织状,束带状及鱼骨状;1例有明显的疏 密分区,两区细胞均为短梭形细胞,有栅栏状排列结构,背景呈粘液样改变。9例肿瘤细胞均有 明显异型性,核分裂象易见(≥4/10 HPF),部分病例可见出血坏死。免疫表型上,9例CD34(+)及 Vimentin(+)、4例CD99(+)、4例Bcl-2(+)。有随访资料的7例中5例术后复发。结论:恶性孤立性纤 维瘤是比较罕见的恶性软组织肿瘤,该肿瘤的诊断需结合形态学、免疫表型等综合判断。手术切 除是首选治疗手段,但术后复发率高。
关键词: 恶性孤立性纤维瘤 软组织肿瘤 梭形细胞 上皮样细胞 CD34

A clinical pathological analysis of 9 cases of malignant solitary fibrous tumor

Authors: 1CHEN Guangye, 2MA Qiang, 1ZHU Hongjing, 1ZHANG Hongli, 1LIU Jiajun, 1YANG Qing, 3XU Kaimei
1 Department of Pathology, Rongchang People’s Hospital of Chongqing, Chongqing 402460
2 Department of Pathology, Daping Hospital and Research Institute of Surgery, Third Military Medical University, Chongqing 400042
3 Department of Pathology, Bishan People’s Hospital of Chongqing, Chongqing 402760, China

CorrespondingAuthor: CHEN Guangye Email: xkmei2015@sina.com

DOI: 10.3978/j.issn.2095-6959.2015.12.022

Abstract

Objective: To explore the clinicopathologic features, diagnosis and differential diagnosis, treatment and prognosis of malignant solitary fibrous tumor (MSFT). Methods: The clinical data, histopathological features, immunophenotype, treatment and prognosis of nine cases of MSFT were reviewed. Results: The patients included 6 males and 3 females with average age of 54 years. Three cases had intracranial tumors, one case had tumor in lung, one in retroperitoneal, one in left upper abdomen, one in pelvic, one in orbital and one in left thigh. Under the microscope, tumors of four cases were composed of epithelioid cells and spindle cells with a distribution of alternate density, hemangiopericytoma-like structure and interspersing collagen fibers. Tumor cells in two cases showed uniformly round or polygonal epithelioid cells in high density. Tumor cells in two cases were the diffused spindle cells arranged in braided, ribbon and fishbone-like structures without alternating density areas. Tumor cells in one case were short spindle cells in both of hypocellular and hypercellular with palisade structure and myxoid background. Tumor cells of nine cases showed significant cell atypia and mitoses (more than 4/10 HPF). Hemorrhage and necrosis could be seen in some cases. Considering immunophenotype, the tumor cells showed positive staining for CD34 and Vimentin in nine cases, positive staining for CD99 in four cases and positive staining for Bcl-2 in four cases. The recurrence appeared in five cases after surgery. Conclusion: MSFT is a rare malignant soft tissue tumor and the diagnosis of the tumor requires a comprehensive judgment of morphology and immunophenotype. Surgical resection is the first choice of treatment, but the recurrence rate is high.

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