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腹部副神经节瘤的临床特征及长期预后分析

Clinical features and long-term outcomes of abdominal paraganglioma

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【作者】 周亚琼严鹏张华敏杨怡冯凯歌陈秋宏王秋林王沛坚

【Author】 ZHOU Yaqiong;YAN Peng;ZHANG Huamin;YANG Yi;FENG Kaige;CHEN Qiuhong;WANG Qiulin;WANG Peijian;Department of Cardiovascular Medicine,the First Affiliated hospital of Chengdu Medical College;Key Laboratory of Ageing and Vascular Homeostasis of Sichuan Higher Education Institude;

【通讯作者】 王秋林;王沛坚;

【机构】 成都医学院第一附属医院心血管内科衰老与血管稳态四川省高等学校重点实验室

【摘要】 目的探讨腹部副神经节瘤(PGL)患者的临床病理特征及长期预后分析。方法对相关病例数据库进行回顾性检索,分析腹部PGL患者的临床病理特征和长期随访预后,应用Kaplan-Meier法进行生存分析,探讨腹部PGL术后复发及死亡的影响因素。结果共纳入24例患者,平均年龄58.9岁,肿瘤平均大小5 cm,主要位于肾下极以下。平均随访时间为54月。所有转移的患者肾上腺嗜铬细胞瘤评分(PASS)≥4分。1例患者出现同步转移,2例出现局部复发和远处转移。1例患者在诊断9年后死亡。经Kaplan-Meier曲线分析显示,PASS评分4~7分的患者复发或死亡的进展曲线明显高于PASS评分0~3分的患者(P=0.03)。结论腹部PGL是一种罕见且预后良好的肿瘤。然而,其复发风险高,因此长期随访十分重要,尤其是对于PASS≥4分的患者。

【Abstract】 Objective To investigate the clinicopathological features and long-term outcomes of patients with abdominal paraganglioma(PGL). Methods A retrospective study of relevant databases were performed to analyze theclinicopathological features and long-term follow-up outcomes of patients with abdominal PGL. KaplanMeier survival analysis was performed to explore the influencing factors of postoperative recurrence or death of abdominal PGL. Results A total of 24 patients were confirmed with abdominal PGL. The average age of diagnosis was 58.9 years. The average tumor size was 5 cm and they were predominantly located in the infrarenal position.The mean follow-up period was 54 months. All patients with metastases had pheochromocytoma of the Adrenal Gland Scaled Score(PASS)of ≥ 4. One patient presented with synchronous metastases while 2 developed local recurrence and distant metastases. One patient died 9 years after diagnosis. Kaplan-Meier curve analysis showed recurrence or death in patients with PASS score of 4 ~ 7 was significantly higher than patients with PASS score of 0~ 3(P = 0.03). Conclusions Abdominal PGL is a rare tumor with excellent long-term prognosis. Recurrence,although common,can occur decades after initial diagnosis. Long-term follow-up is therefore recommended for all patients with PGL,especially in patients with PASS of ≥ 4.

【关键词】 副神经节瘤复发手术治疗
【Key words】 paragangliomarecurrencesurgerytherapy
【基金】 国家自然科学基金项目(编号:81970262)
  • 【文献出处】 实用医学杂志 ,The Journal of Practical Medicine , 编辑部邮箱 ,2021年21期
  • 【分类号】R730.5
  • 【下载频次】96
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