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肾横纹肌样瘤伴自发性破裂:病例报告及文献复习

Rhabdoid tumor of the kidney with spontaneous rupture: case report and review of literature.

作者信息

Chang Chi-Jen, Yeh Ming-Lun, Chen Cha-Chun

机构信息

Division of Pediatric Surgery, Department of Surgery, Shin-Kong Wu Ho-Su Memorial Hospital, No. 95, Wen-chang Road, Shih-Lin District, Taipei, Taiwan.

出版信息

Pediatr Surg Int. 2008 Apr;24(4):451-3. doi: 10.1007/s00383-007-1934-6. Epub 2007 May 10.

Abstract

Spontaneous rupture of the kidney is uncommon; here we report a case of spontaneous rupture of the kidney due to a rhabdoid tumor. An 11-year-old boy presented with left flank pain and hematuria, was admitted to a hospital where he was found to have an abnormality of the left kidney on computed tomography (CT) scan. He was referred to our department for further evaluation and treatment on the next day. Spontaneous rupture of left renal tumor was suspected by a drop in hemoglobin level, hemoglobin decreased from 9.2 to 7.6 mg/dl within 72 h. The hemoglobin level continued to drop despite blood transfusion. Urgent trans-abdominal exploration of the left kidney was performed. During the operation, rupture of left renal tumor with massive bleeding was noted. Para-aortic lymph node metastasis is evident. The surgical specimen contained a large peri-renal hemorrhage and tumor rupture into peri-pelvic soft tissue. Histopathological diagnosis was rhabdoid tumor consisting of round nuclei, prominent nucleoli and eosinophic cytoplasm. Two courses of adjuvant chemotherapy with actinomycin D, vincristine and epirubicin and radiotherapy (1,200 cGY) were performed post-operatively. The patient died 5 months after operation due to metastasis of the tumor to the lung and intra-abdominal organs.

摘要

肾自发性破裂并不常见;在此我们报告一例因横纹肌样瘤导致的肾自发性破裂病例。一名11岁男孩因左侧胁腹疼痛和血尿就诊,入院后经计算机断层扫描(CT)发现左肾异常。次日他被转诊至我科作进一步评估和治疗。因血红蛋白水平下降怀疑左肾肿瘤自发性破裂,血红蛋白在72小时内从9.2降至7.6mg/dl。尽管输血,血红蛋白水平仍持续下降。遂对左肾进行紧急经腹探查。手术中,发现左肾肿瘤破裂并伴有大量出血。腹主动脉旁淋巴结转移明显。手术标本可见大量肾周出血以及肿瘤破入肾盂周围软组织。组织病理学诊断为横纹肌样瘤,由圆形细胞核、明显核仁及嗜酸性细胞质组成。术后进行了两个疗程的辅助化疗,使用放线菌素D、长春新碱和表柔比星,并进行了放疗(1200cGY)。患者术后5个月因肿瘤转移至肺和腹腔器官而死亡。

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