原发性脾血管肉瘤术后肝转移1例并文献复习

Primary splenic angiosarcoma with hepatic metastasis after splenectomy:a case report with literature review

目的讨论原发性脾血管肉瘤的临床及病理特征。方法回顾性分析2016-12-29收住、被确诊为原发性脾血管肉瘤患者术后肝转移1例患者的临床资料、切除肿瘤标本的组织形态学及免疫组化染色结果;以“血管肉瘤”“脾脏”“原发”为检索词,检索Medline/PubMed数据库2015年1月至2019年7月的文献进行复习。结果患者为79岁男性,因上腹部疼痛入院。CT扫描发现脾大,脾实质内弥漫分布不均匀强化的低密度病灶。术后病理组织学发现血管源性恶性肿瘤细胞。免疫组化提示肿瘤细胞CD31、CD34、ERG、CD8呈阳性,CD68、CD163、SMA呈阴性,故确诊为原发性脾血管肉瘤。术后9个月CT发现多发肝转移病灶。最终患者因肺炎伴呼吸衰竭死亡,总生存时间为10个月。共检索出20篇外文文献报道20例患者,男6例,女14例;年龄13~83岁,行脾脏切除术后生存时间最短者2周,最长者随访13年。结论原发性脾血管肉瘤罕见,转移率高,临床预后差,早期诊断及脾破裂前手术对患者生存有重要意义。

Objective To discuss the clinical and pathological characteristics of primary splenic angiosarcoma(PSA).Methods A retrospective analysis was performed on the clinical data,histomorphology and immunohistochemical staining results of one patient admitted to 2016-12-29 confirmed as PSA with postoperative liver metastasis.Using the terms"hematosarcoma","spleen"and"primary"as the search terms,search the Medline/PubMed database from January 2015 to July 2019.Results A 79-year-old male patient presented with left upper quadrant abdominal pain.CT scans revealed an enlarged spleen with heterogeneous enhancing hypodense lesions diffusely distributed in spleen parenchyma.Histopathology examination after splenectomy revealed an angiogenic malignant neoplasm,and immunohistochemistry showed that the tumor cells were positive for CD31,CD34,ERG,CD8 and negative for CD68,CD163,SMA,so the patient was diagnosed as primary splenic angiosarcoma.At 9 months after operation,multiple liver metastases foci were found by CT scanning.Finally,the patient succumbed to the pneumonia and respiratory failure with a survival of 10 months.A total of 20 foreign literature reports were retrieved in 20 patients,6 males and 14 females,aged 13 to 83 years.The shortest survival time after splenectomy was 2 weeks,and the longest was followed up for 13 years.Conclusions PSA is an extremely rare and mesenchymal malignant tumor of vascular origin often with a poor prognosis and high metastatic rate.The best chance for survival follows an early diagnosis and a prompt splenectomy prior to splenic rupture.