妊娠合并嗜铬细胞瘤和副神经节瘤临床特征及预后分析

Clinical characteristics and prognostic analysis of pregnancy complicated with pheochromocytoma and paraganglioma

目的:探讨妊娠合并嗜铬细胞瘤和副神经节瘤(PPGL)临床特征及其对预后的影响。方法:回顾性分析2014年1月至2023年1月我院收治的16例妊娠合并PPGL患者及按年龄相匹配的32例非妊娠合并PPGL对照组患者的临床资料,比较两组患者的临床特征,无进展生存期(PFS)的差异。结果:妊娠合并PPGL组与非妊娠合并PPGL组在高血压发生率、24小时尿肾上腺素(E)、24小时尿去甲肾上腺素(NE)的表达、基因突变、复发转移数量上比较,差异具有统计学意义(P<0.05)。妊娠合并PPGL的PFS较非妊娠组短(χ^(2)=28.107,P<0.01)。多因素分析显示PPGL合并妊娠、基因突变、突变基因SDHB是PPGL患者PFS的独立预后因素(P<0.05)。结论:妊娠合并PPGL出现复发转移数量较多且时间较早,妊娠是影响PPGL复发转移的重要因素,影响患者的预后。

Objective:To investigate the clinical characteristics of pregnancy complicated with pheochromocytoma and paraganglioma(PPGL)and their impact on prognosis.Methods:The clinical data of 16 pregnant patients with PPGL and 32 age-matched non-pregnant patients with PPGL treated in our hospital from January 2014 to January 2023 were retrospectively analyzed,and the clinical characteristics and progression-free survival(PFS)of the two groups were compared.Results:There were statistically significant differences between pregnancy with PPGL group and non-pregnancy with PPGL group in the incidence of hypertension,the expression of 24-hour urine adrenaline(E),24-hour urine norepinephrine(NE),gene mutation,and the number of recurrence and metastasis(P<0.05).Pregnancy with PPGL had shorter PFS than non-pregnancy with PPGL(χ^(2)=28.107,P<0.01).Multivariate analysis showed that PPGL combined with pregnancy,gene mutation and mutated gene SDHB were independent prognostic factors for PFS in PPGL patients(P<0.05).Conclusion:The recurrence and metastasis of pregnancy with PPGL occurred not only in large number but also in early time.Pregnancy is an important factor affecting the recurrence and metastasis of PPGL,which affects the prognosis of patients.