Zhang L, Wei J G, Fang S G, Luo R K, Xu Z G, Li D J, Kong L F
Department of Pathology, Henan Provincial People's Hospital; People's Hospital of Zhengzhou University; People's Hospital of Henan University, Zhengzhou 450003, China.
Department of Pathology, Shaoxing People's Hospital, Zhejiang Province, Shaoxing 312000, China.
Zhonghua Bing Li Xue Za Zhi. 2020 Feb 8;49(2):129-133. doi: 10.3760/cma.j.issn.0529-5807.2020.02.005.
To investigate the clinicopathological characteristics, histogenesis, immunophenotypes, molecular genetic characteristics, diagnosis and differential diagnosis of calcifying fibrous tumors (CFT). A total of 32 cases of CFT (22 cases from Henan Provincial People's Hospital and 10 cases from PLA Army Medical Center) diagnosed between June 2009 and February 2019 were reviewed. The clinical and pathologic data were analyzed. There were 12 male and 20 female patients, aged from 15 to 63 years (mean 40.8 years). Eleven cases occurred in stomach, four cases in retroperitoneum, four cases in ovary, two cases in scrotum, two cases in mediastinum, two cases in head and neck, one case each in thoracic cavity, lung, adrenal gland, kidney, sigmoid colon, epididymis and mesosalpinx. All the tumors were solid masses with clear boundaries. The maximal dimension of the tumors ranged from 0.6 to 10.0 cm. Microscopically, there was hypocellular stromal sclerosis and wavy storiform coarse collagen with superimposed scattered or patchy lymphocytes and plasma cells; calcification or gravel formation were also detected. Immunohistochemistry showed that spindle cells were positive for vimentin and some were positive for CD34; and they were negative for calponin, SMA, desmin, S-100 protein, SOX10, STAT6, β-catenin, ALK, CD117, DOG1, CKpan, and EMA. No ALK rearrangement was detected by FISH in all cases. No C-KIT and PDGFRA mutation was detected in all the tested 11 cases of stomach, four cases of retroperitoneal and one case of sigmoid colon CFT. MDM2 was not amplified by FISH in all four tested cases of retroperitoneal CFT. CFT is a rare benign tumor of fibroblastic cell origin. The diagnosis mainly depends on histomorphologic analysis and immunophenotyping. CFT should be differentiated from other benign and malignant spindle cell mesenchymal tumors.
探讨钙化性纤维性肿瘤（CFT）的临床病理特征、组织发生、免疫表型、分子遗传学特征、诊断及鉴别诊断。回顾性分析2009年6月至2019年2月期间诊断的32例CFT（河南省人民医院22例，解放军陆军军医大学第一附属医院10例）。分析其临床及病理资料。患者12例男性，20例女性，年龄15～63岁（平均40.8岁）。11例发生于胃，4例发生于腹膜后，4例发生于卵巢，2例发生于阴囊，2例发生于纵隔，2例发生于头颈部，胸腔、肺、肾上腺、肾、乙状结肠、附睾及输卵管系膜各1例。所有肿瘤均为边界清晰的实性肿块。肿瘤最大径0.6～10.0 cm。镜下见细胞稀少的间质硬化，有波浪状束状粗胶原纤维，散在或灶状淋巴细胞及浆细胞浸润；可见钙化或砂粒体形成。免疫组化显示梭形细胞波形蛋白阳性，部分CD34阳性；钙调蛋白、平滑肌肌动蛋白、结蛋白、S-100蛋白、SOX10、信号转导和转录激活因子6、β-连环蛋白、间变性淋巴瘤激酶、CD117、犬瘟热病毒1、细胞角蛋白广谱、上皮膜抗原均阴性。所有病例荧光原位杂交均未检测到间变性淋巴瘤激酶重排。11例胃、4例腹膜后及1例乙状结肠CFT检测均未发现C-KIT和血小板衍生生长因子受体A突变。4例腹膜后CFT荧光原位杂交检测均未发现MDM2扩增。CFT是一种罕见的成纤维细胞源性良性肿瘤。诊断主要依靠组织形态学分析及免疫表型分析。CFT应与其他良恶性梭形细胞间叶组织肿瘤相鉴别。
