Division of Endocrinology, Diabetes, Metabolism, and Nutrition, Mayo Clinic, Rochester, MN, USA.
Department of Biomedical Statistics and Informatics, Mayo Clinic, Rochester, MN, USA.
Lancet Diabetes Endocrinol. 2021 Jan;9(1):13-21. doi: 10.1016/S2213-8587(20)30363-6. Epub 2020 Nov 26.
Phaeochromocytoma or paraganglioma (collectively known as PPGL) in pregnant women can lead to severe complications and death due to associated catecholamine excess. We aimed to identify factors associated with maternal and fetal outcomes in women with PPGL during pregnancy.
We did a multicentre, retrospective study of patients with PPGL and pregnancy between Jan 1, 1980, and Dec 31, 2019, in the International Pheochromocytoma and Pregnancy Registry and a systematic review of studies published between Jan 1, 2005, and Dec 27, 2019 reporting on at least five cases. The inclusion criteria were pregnancy after 1980 and PPGL before or during pregnancy or within 12 months post partum. Eligible patients from the retrospective study and systematic review were included in the analysis. Outcomes of interest were maternal or fetal death and maternal severe cardiovascular complications of catecholamine excess. Potential variables associated with these outcomes were evaluated by logistic regression.
The systematic review identified seven studies (reporting on 63 pregnancies in 55 patients) that met the eligibility criteria and were of adequate quality. A further 197 pregnancies in 186 patients were identified in the International Pheochromocytoma and Pregnancy Registry. After excluding 11 pregnancies due to potential overlap, the final cohort included 249 pregnancies in 232 patients with PPGL. The diagnosis of PPGL was made before pregnancy in 37 (15%) pregnancies, during pregnancy in 134 (54%), and after delivery in 78 (31%). Of 144 patients evaluated for genetic predisposition for phaeochromocytoma, 95 (66%) were positive. Unrecognised PPGL during pregnancy (odds ratio 27·0; 95% CI 3·5-3473·1), abdominal or pelvic tumour location (11·3; 1·5-1440·5), and catecholamine excess at least ten-times the upper limit of the normal range (4·7; 1·8-13·8) were associated with adverse outcomes. For patients diagnosed during pregnancy, α-adrenergic blockade therapy was associated with fewer adverse outcomes (3·6; 1·1-13·2 for no α-adrenergic blockade vs α-adrenergic blockade), whereas surgery during pregnancy was not associated with better outcomes (0·9; 0·3-3·9 for no surgery vs surgery).
Unrecognised and untreated PPGL was associated with a substantially higher risk of either maternal or fetal complications. Appropriate case detection and counselling for premenopausal women at risk for PPGL could prevent adverse pregnancy-related outcomes.
US National Institutes of Health.
孕妇的嗜铬细胞瘤或副神经节瘤（统称为 PPGL）会因相关儿茶酚胺过量而导致严重并发症和死亡。我们旨在确定与 PPGL 孕妇的母婴结局相关的因素。
我们对 1980 年 1 月 1 日至 2019 年 12 月 31 日期间国际嗜铬细胞瘤和妊娠登记处中嗜铬细胞瘤和妊娠的患者进行了多中心回顾性研究，并对 2005 年 1 月 1 日至 2019 年 12 月 27 日期间至少报告了 5 例病例的研究进行了系统回顾。纳入标准为 1980 年后妊娠，妊娠前或妊娠期间或产后 12 个月内发生 PPGL。回顾性研究和系统评价中符合条件的患者均纳入分析。感兴趣的结局为母婴死亡和儿茶酚胺过量引起的母亲严重心血管并发症。通过逻辑回归评估与这些结局相关的潜在变量。
系统评价确定了符合纳入标准且质量足够的 7 项研究（报道了 55 例患者的 63 例妊娠）。在国际嗜铬细胞瘤和妊娠登记处中又发现了 197 例妊娠，涉及 186 例患者。排除 11 例可能重叠的妊娠后，最终队列纳入了 232 例 PPGL 患者的 249 例妊娠。37 例（15%）妊娠前诊断为 PPGL，134 例（54%）妊娠期间诊断，78 例（31%）产后诊断。在评估嗜铬细胞瘤遗传易感性的 144 例患者中，95 例（66%）为阳性。妊娠期间未识别的 PPGL（比值比 27.0；95%CI 3.5-3473.1）、腹部或盆腔肿瘤位置（11.3；1.5-1440.5）和儿茶酚胺水平超过正常上限 10 倍（4.7；1.8-13.8）与不良结局相关。对于在妊娠期间诊断的患者，α-肾上腺素能阻滞剂治疗与较少的不良结局相关（无 α-肾上腺素能阻滞剂治疗 vs α-肾上腺素能阻滞剂治疗的 3.6；1.1-13.2），而妊娠期间手术与更好的结局无关（无手术 vs 手术的 0.9；0.3-3.9）。
未被识别和未经治疗的 PPGL 与母婴并发症的风险显著增加有关。对有发生 PPGL 风险的绝经前妇女进行适当的病例检测和咨询，可以预防与妊娠相关的不良结局。
美国国立卫生研究院。
