巨大膀胱副神经节瘤致急性出血多学科协作诊疗一例

A Case of Multidisciplinary Treatment for Acute Hemorrhage Caused by Giant Bladder Paraganglioma

  • 摘要: 膀胱副神经节瘤是一种罕见的神经内分泌肿瘤,仅占膀胱肿瘤的0.05%。本文报道1例16岁男性患者因膀胱巨大副神经节瘤(最大径约10 cm)并发急性出血的诊疗过程。患者术前影像学检查提示膀胱肿瘤局部进展,遂接受3个疗程的替莫唑胺新辅助化疗。等待择期手术期间发生失血性休克,经急诊动脉栓塞术成功止血。随后,患者于急诊科在全身麻醉下行全膀胱根治性切除术联合回肠原位新膀胱术,术后恢复良好。病理检查确诊为膀胱副神经节瘤,琥珀酸脱氢酶B表达阳性,Ki-67增殖指数为3%。术后6周随访显示,患者血压及内分泌指标恢复正常,生活质量良好。本案例表明,多学科协作诊疗能有效整合各学科优势,制订全面、系统的治疗方案,对确保此类复杂病例的成功救治具有至关重要的意义。

     

    Abstract: Bladder paraganglioma is a rare neuroendocrine tumor,accounting for only 0.05% of all bladder tumors. This article reports the diagnosis and treatment of a 16-year-old male patient with acute hemorrhage secondary to a giant bladder paraganglioma (approximately 10 cm in maximum diameter). Preoperative imaging evaluation suggested locally advanced disease,and the patient subsequently received three cycles of neoadjuvant chemotherapy with temozolomide. He patient􀆳s blood tests revealed hemorrhagic shock. During the waiting period of surgery,hemostasis was successfully achieved through emergency transarterial embolization. Subsequently,the patient underwent emergency radical cystectomy combined with orthotopic ileal neobladder reconstruction under general anesthesia and recovered well after surgery. Postoperative pathological examination confirmed the diagnosis of bladder paraganglioma,with positive immunohistochemical staining for succinate dehydrogenase subunit B and a Ki-67 proliferation index of 3%. At the 6-week follow-up,the patient􀆳s blood pressure and endocrine parameters had returned to normal,with a satisfactory quality of life. This case demonstrates that a multidisciplinary team approach can effectively integrate expertise from various specialties to formulate comprehensive and systematic treatment plans,which is crucial for ensuring successful management of such complex cases.

     

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