胃肠道钙化性纤维性肿瘤的临床病理分析并文献复习

目的探讨胃肠道钙化性纤维性肿瘤(CFT)的临床病理特点及诊断和鉴别诊断要点。方法回顾性分析2016年1月至2019年6月首都医科大学附属北京友谊医院收治的6例胃肠道CFT患者的病例资料,对其进行病理形态学观察和免疫组织化学染色分析,并进行文献复习。结果6例胃肠道CFT患者中,男性2例,女性4例;年龄20~43岁,平均年龄35岁;5例发生于胃,1例发生于回盲部;胃肠镜表现为黏膜下隆起性病变,黏膜面光滑;组织学表现为大量玻璃样变性的胶原纤维,其内可见稀疏分布的梭形细胞,散在及灶状淋巴细胞、浆细胞浸润,伴营养不良性钙化和砂粒体形成;免疫组化:肿瘤细胞表达Vimentin,不表达CD117、DOG-1、CD34、Actin、Desmin、S-100,ALK、Ig G、Ig G4,Ki-67指数为0~5%;6例患者均采用手术或内镜下肿物切除治疗,随访6~31个月,均未出现复发及转移。结论胃肠道CFT是一种罕见的良性纤维性肿瘤,容易误诊为消化道其他间叶来源性肿瘤,应结合临床资料、组织形态及免疫表型做出综合诊断。手术切除可治愈,预后良好。

Clinicopathological study of gastrointestinal calcifying fibrous tumor and review of the literature

Objective To investigate the clinicopathological features,diagnosis and differential diagnosis of gastrointestinal calcifying fibrous tumor(CFT).Methods 6 cases of gastrointestinal CFT patients admitted to Beijing Friendship Hospital,Capital Medical University from January 2016 to June 2019 were retrospective analyzed with histopathological observation and immunohistochemical staining,and related literatures were further reviewed.Results Of the 6 patients of gastrointestinal CFT,2 were male,and 4 were female.The average age was 35 years old(from 20 to 43 years old).Five cases were originated in stomach and 1 case in ileocecum.Gastrointestinal endoscope findings are sub-mucosal bulging lesions with smooth mucosal surface.Histologically,the tumors were composed of abundant hyalinized collagen with spindle shaped cells,accompanied with scattered lymphocytes and plasma cell infiltration,and dystrophic calcification or psammomatous body.Immunohistochemically,tumor cells expressed Vimentin,but negative for CD117,DOG-1,CD34,Actin,Desmin,S-100,ALK,Ig G and Ig G4,Ki-67 index was 0 to 5%.All the patients were treated with surgical excision or endoscopic excision,and no recurrence or metastasis was observed during follow-up(from 6-31 months).Conclusion Gastrointestinal CFT is a rare,benign fibrous lesion,which is easily misdiagnosed as other gastrointestinal mesenchymal tumors.The final diagnosis should be made based on clinical data,histopathological changes and immunohistochemical findings.It can be cured by surgical excision and the prognosis is excellent.