小儿巨大腹膜后副神经节瘤伴转移:1例报告并文献复习

目的结合病例分析儿童巨大腹膜后副神经节瘤伴转移诊疗过程，总结儿童副神经节瘤的诊疗经验。方法回顾性分析重庆医科大学附属儿童医院泌尿外科收治1例副神经节瘤伴远处转移患者的临床特点、诊疗经过以及预后情况。结果副神经节瘤病例较少，在儿童更为罕见。本例患儿主要表现为血压高，偶尔头晕头痛，偶有气促。通过手术治疗处理巨大原发灶，通过化学治疗处理肺部转移灶及椎体浸润灶。结论对伴有转移的恶性副神经节瘤，尤其是临床症状不典型并且诊断不明确的患者，如果手术治疗应明确肿瘤性质、明确肿瘤与周围组织血管的关系。术中减少触碰、尽可能保证瘤体包膜完整，是保证手术成功的关键。术后用化学治疗处理转移灶，短期效果理想，长期效果需要继续随访。 

Diagnosis and treatment of giant retroperitoneal paraganglioma with metastasis in children: a case report

Objective To analyze the diagnosis and treatment of a child of giant retroperitoneal paraganglioma with metastase,and summarize diagnosis and treatment experience of paraganglioma in children. Methods We retrospectivly analyzed the case history, diagnosis, treatment and prognosis of a case of paraganglioma with multiple metastases in urology department of children’s hospital affiliated to chongqing medical university. Results The main manifestations of this case were high blood pressure, occasional dizziness and headache, and occasional shortness of breath.Large primary lesions were treated with surgery, and pulmonary metastases and vertebral infiltration were treated with chemotherapy. Conclusion For malignant paraganglioma with metastasis, especially for patients with atypical clinical symptoms and unclear diagnosis, the nature of the tumor and the relationship between the tumor and surrounding tissues and blood vessels should be clarified if surgical treatment is performed.The key to ensure the success of the operation is to reduce the touch and to ensure the integrity of the tumor capsule.Chemotherapy has a good short-term effect, but the long-term effect needed follow-up.