青少年嗜铬细胞瘤24例临床分析

Clinical Analysis of 24 Patients With Pheochromocytomas in Adolescents

目的:总结分析青少年嗜铬细胞瘤(PHEO)和副神经节瘤的临床特点。方法:回顾性分析北京协和医院1952年6月至2005年6月期间收治的24例来诊年龄18岁以下PHEO和副神经节瘤患者的临床表现、实验室、影像学、核医学检查等定性定位诊断以及手术和病理情况,患者平均年龄(13．4±3．2)岁, 男:女=7:1。结果:临床表现以持续性高血压为主(75％),按肿瘤部位分,29％为双肾上腺,29％为副神经节瘤;按肿瘤性质和遗传性分,29％为恶性,21％为家族性。结论:青少年嗜铬细胞瘤男性多见,临床表现常较典型,与成人相比,双肾上腺PHEO、恶性、副神经节瘤发生率较高,与遗传关系更密切。术后需长期随访。

Objective：Pheochromocytomas（PHEO） in adolescents are very rare. The purpose of this retrospective analysis was to investigate the clinical characteristics of patients with PHEO in adolescents. Methods：We analyzed the clinical data from 24 patients with PHEO admitted to Peking Union Medical College Hospital between 1952 and 2005. Results：The average age at diagnosis was 13.4 ±3. 2 years. Twenty-one were male and three female. Most of the patients （75%） presented with persistent hypertension. Seven patients（29% ） had bilateral PHEO, and seven（29% ）had paraganglioma. Of the 24 patients, three had multiple endocrine neoplasia Ⅱ （ MEN Ⅱ ）, two had yon Hippel-Lindau （VHL） disease. Pathologic examinations and long-term follow-up revealed evidence of malignant features in 7 patients （29%）. Conclusion：The data suggests that the clinical features of PHEO in adolescents are more typical than those in adults. During the diagnosis for PHEO in adolescents, hereditary syndromes, bilateral PHEO, malignancy and paraganglioma should be considered.