小肠原发性血管肉瘤一例并文献复习

Primary Angiosarcoma of the Small Intestine: A Case Report and Literature Review

摘要: 目的：探讨小肠原发性血管肉瘤的组织学特征、免疫组化特征、鉴别诊断和治疗方法。方法：回顾性分析１例小肠原发性血管肉瘤患者的临床资料、组织学诊断及免疫组化结果，并对相关文献进行复习。结果：患者，男，67岁，镜下观察肿瘤由实体性区域和出血坏死区域构成。肿瘤细胞异型性明显，细胞核大呈圆形、多形性，空泡样，具有多形性、间变性特点，核仁大而明显，核分裂象易见，约10个/10HPF；部分细胞胞质丰富，呈嗜双色性。免疫组化显示肿瘤细胞表达CD31、ERG及Fli-1，部分表达CD34。小肠血管肉瘤组织学特征有时可能与其他发生于小肠的肿瘤相混淆，需要与多种肿瘤相鉴别。小肠的血管肉瘤预后较其他部位的血管肉瘤差，本病例患者于术后10个月因全身多脏器转移而死亡。手术切除加术后化疗或姑息性化疗是小肠血管肉瘤的主要治疗方式。结论：小肠原发性血管肉瘤是一种罕见恶性肿瘤，预后差，临床症状无特异性，极易误诊或延迟诊断，因此经常被误诊为其他胃肠道常见病，病理医生需掌握其组织学、免疫组织化学特点及鉴别诊断要点，从而做出准确诊断。

Abstract: Objective: To investigate the histological and immunohistochemical features, differential diagnosis and treatment of primary angiosarcoma of small intestine. Methods: We retrospectively analyzed the clinical data, histological diagnosis and immunohistochemical results of one patient with primary hemangiosarcoma of the small intestine and reviewed relevant literatures. Results: The patient, male, 67 years old. Microscopically, the tumor consisted of solid areas and areas of hemorrhage and necrosis. The tumor cells showed obvious atypia, with large round, pleomorphic and vacuolated nuclei, pleomorphic and anaplastic. The nucleoli were large and obvious, and the mitotic figures were about 10/10HPF. Some cells are rich in cytoplasm, showing dichroism. Immunohistochemistry showed that the tumor cells expressed CD31, ERG and Fli-1, and partially expressed CD34. The histological features of small intestinal angiosarcoma may sometimes be confused with other tumors of the small intestine and need to be differentiated from multiple tumors. The prognosis of angiosarcoma in the small intestine is worse than that in other sites. The patient died 10 months after surgery due to systemic multiple organ metastases. Surgical resection combined with postoperative chemotherapy or palliative chemotherapy has been the main treatment for small intestinal angiosarcoma. Conclusion: Primary angiosarcoma of the small intestine is a rare malignant tumor with poor prognosis and no specific clinical symptoms. It is easy to be misdiagnosed or delayed diagnosis. Therefore, it is often misdiagnosed as other common diseases of the gastrointestinal tract. Pathologists should master the histological and immunohistochemical characteristics and key points of differential diagnosis so as to make a definite diagnosis.