Objective: To summarize the clinical characteristics of paraganglioma. Methods: A total of 215 pheochromocytoma/ paraganglioma cases in Peking University First Hospital between January 1996 and December 2014 were retrospectively analyzed. The patients were divided into two groups according to the tumor position: pheochromacytoma group (173 patients) and paraganglioma group (42 patients). Then the clinical characteristics between the groups were compared. Results: There were 42 paraganglioma cases in the study, accounting for 19.5%. The most common position was retroperitoneum (66.7%, 28/42), followed by bladder (19.0%, 8/42), para-aorta (11.9%, 5/42) and pelvic cavity (2.4%, 1/42). Sixty-nine percent (29/42) of patients had different degree of hypertension, while up to 31.0% (13/42) was silent type. There was no statistical difference between the two groups in severity of hypertension, age, gender, duration, abnormal glucose metabolism, and the incidence of hypokalemia( all P>0.05). Adrenaline level was higher in pheochromocytoma group (13.4% vs 9.5%, P=0.004), while noradrenaline level was higher in paraganglioma group (80.8% vs 59.2%, P=0.001). Malignancy (21.4% vs 4.6%, P<0.001) and bigger tumor size [(6.67±3.24)cm vs (5.55±2.76)cm, P=0.024] was more common in paraganglioma group. The maximum tumor volume was 18.0 cm×18.0 cm×10.5 cm vs 14.0 cm×13.0 cm×7.0 cm, while the minimum was 1.8 cm×1.3 cm×1.3 cm vs 1.2 cm×1.0 cm×1.0 cm. There was no significant difference in the pathological manifestations between the two groups. In the study, 11.9% (5/42) of patients in paraganglioma group was misdiagnosed or indefinite at the early diagnosis. Conclusion: Compared with pheochromocytoma, paraganglioma has the characteristics of widely distribution, more various clinical performance, more silent type and higher malignant rate. It is critical to put emphasis on its early diagnosis and treatment.
目的:总结副神经节瘤患者的临床特点。 方法:回顾性分析。1996年1月至2014年12月于北京大学第一医院诊治的嗜铬细胞瘤/副神经节瘤患者215例。按照病变部位分为嗜铬细胞瘤组(173例)及副神经节瘤组(42例),比较其临床特点。 结果: 215例患者中副神经节瘤42例,占19.5%。病变部位腹膜后最多见,占66.7%(28/42),膀胱19.0%(8/42),腹主动脉旁11.9%(5/42),盆腔2.4%(1/42)。69.0%(29/42)的患者存在不同程度的高血压,多达31.0%(13/42)为寂静性。经比较,两组间高血压比例、年龄、性别、病程、糖代谢异常、低钾血症发生率差异均无统计学意义(均P>0.05);嗜铬细胞瘤组肾上腺素升高更为多见(13.4%比9.5%,P=0.004),副神经节瘤组去甲肾上腺素升高更多见(80.8%比59.2%,P=0.001);副神经节瘤组恶性比例更高(21.4%比4.6%,P<0.001),肿瘤直径更大[(6.67±3.24)cm比(5.55±2.76) cm,P=0.024),肿瘤体积最大者分别为18.0cm×18.0 cm×10.5 cm和14.0 cm×13.0 cm×7.0 cm,最小者分别为1.8 cm×1.3 cm×1.3 cm比1.2 cm×1.0 cm×1.0 cm。两组病理表现无明显差异。11.9%(5/42)的副神经节瘤首诊误诊或诊断不明确。 结论:与嗜铬细胞瘤相比,副神经节瘤侵犯部位广泛,临床表现更多样,寂静性多见,瘤体更大,恶性比例更高。提高认识、早期识别、早期诊治至关重要。.
Keywords: Clinical characteristics; Paraganglioma; Pheochromocytoma.