原发性脾血管肉瘤的诊治分析

Diagnosis and treatment of primary splenic angiosarcoma

目的总结分析原发性脾血管肉瘤的临床和影像学特征、治疗方法及疗效。方法回顾性分析2012年1月至2019年12月郑州大学第一附属医院收治的6例病理诊断明确的原发性脾血管肉瘤患者资料,总结分析患者临床特征、CT及超声影像学表现、治疗方法和生存情况。结果6例原发性脾血管肉瘤患者中男性4例,女性2例,平均年龄52.6岁。6例患者均缺乏特异性临床表现,经术后病理确诊。CT检查发现6例患者脾脏体积均增大,脾内占位性病变,1例为脾内单发,5例为脾内多发,部分病灶相互融合,其中1例伴肝内多发转移。超声检查显示3例患者脾脏体积增大,脾内实性低回声,边缘血流丰富。5例患者行脾切除术,瘤体切除完全;1例脾内多发病灶伴肝内多发占位患者,无法根治切除,仅行脾切除术。伴肝转移患者术后2个月复查肝脏病灶明显较前增多,术后生存3个月;2例术后行辅助化疗患者分别于术后9、13个月复发,术后生存期分别为16、19个月;1例术后辅助化疗患者未出现明显复发征象,术后27个月死于其他疾病;1例术后行靶向治疗患者术后10个月复发,生存14个月;1例行单纯手术治疗患者术后4个月复发,6个月死亡。结论原发性脾血管肉瘤患者缺乏特异性临床表现及影像学特征,确诊需要病理学检查,手术切除为该疾病的主要治疗方法,手术联合术后辅助化疗可能是未来治疗脾血管肉瘤的有效方案。

Objective To analyze the clinical and imaging features,and treatment and curative effects of primary splenic angiosarcoma.Methods A retrospective study was conducted on 6 patients with primary splenic angiosarcoma diagnosed histopathologically at the First Affiliated Hospital of Zhengzhou University from January 2012 to December 2019.The clinical characteristics,CT and ultrasound imaging findings,treatment methods and survival outcomes of these patients were analyzed.Results There were 4 males and 2 females,with an average age of 52.6 years.These 6 patients with primary splenic angiosarcoma had no specific clinical manifestations,and the diagnoses were all confirmed histopathologically after surgery.CT examination of these 6 patients showed that the spleen volume was increased,with splenic space-occupying lesions.The lesions were single in 1 patient and multiple in 5 patients.Some lesions fused with each other.There were multiple intrahepatic metastases in 1 patient.Ultrasound in 3 patients showed enlarged solid hypoechoic spleens,and rich blood flow at the edges.Five patients underwent splenectomy with complete removal of tumors.For the patient with multiple lesions in the spleen and multiple space-occupying lesions in the liver who could not be treated radically,only splenectomy was done.This patient developed significantly more liver lesions 2 months after surgery,and survived for 3 months.Two patients who underwent postoperative adjuvant chemotherapy relapsed at 9 months and 13 months after surgery.They survived for 16 months and 19 months,respectively.A patient who received postoperative adjuvant chemotherapy survived for 27 months without any obvious signs of recurrence.A patient who underwent targeted therapy relapsed after 10 months,and survived for 14 months after surgery.The remaining patient who underwent simple surgery relapsed 4 months after surgery and died 6 months later.Conclusions Primary splenic angiosarcoma is a highly malignant tumor.In this study,it lacked specific clinical manifestations and imaging features and its diagnosis required histopathological examination.Surgical resection is the main treatment for this disease.Whether surgery combined with postoperative adjuvant therapy is a more effective treatment than surgery alone for splenic angiosarcoma requires more studies in the future to determine.