Langton Katharina, Gruber Matthias, Masjkur Jimmy, Steenblock Charlotte, Peitzsch Mirko, Meinel Jörn, Lenders Jacques, Bornstein Stefan, Eisenhofer Graeme
a Institute of Clinical Chemistry and Laboratory Medicine , University Hospital and Medical Faculty Carl Gustav Carus, Technische Universität Dresden , Dresden , Germany.
b Department of Medicine III , University Hospital and Medical Faculty Carl Gustav Carus, Technische Universität Dresden , Dresden , Germany.
Gynecol Endocrinol. 2018 Jan;34(1):20-24. doi: 10.1080/09513590.2017.1379497. Epub 2017 Sep 22.
Pheochromocytomas in pregnancy are rare but potentially lethal. Even rarer is the combination of pheochromocytoma in pregnancy with subsequent development of ectopic Cushing's syndrome. We report a 36-year-old woman, previously diagnosed with essential hypertension, who developed severe hypertension in pregnancy complicated by insulin-dependent gestational diabetes. A cesarean section was performed at 32 weeks following a hypertensive crisis after routine administration of betamethasone. Postnatal persistence of signs and symptoms of catecholamine excess led to the diagnosis of a left adrenal pheochromocytoma. Between diagnosis and planned tumor removal, the patient developed signs and symptoms of Cushing's syndrome (facial edema and hirsutism, myopathy and fatigue). Biochemical testing confirmed hypercortisolism with extremely elevated levels of plasma adrenocorticotropin, urinary cortisol and multiple steroids of a plasma panel that were all normal at previous testing. The previously noradrenergic tumor also started producing epinephrine. Histopathological examination confirmed the pheochromocytoma, which was also immunohistochemically positive for adrenocorticotropin. Full post-surgical recovery was sustained with normal blood pressure and biochemical findings after one year. This report not only underlines the chameleon behavior of pheochromocytoma but also illustrates its potential for a metamorphosing presentation. Corticosteroid administration in pregnancy requires a cautious approach in patients with hypertension.
妊娠期嗜铬细胞瘤罕见但可能致命。妊娠期嗜铬细胞瘤合并异位库欣综合征的情况更为罕见。我们报告一名36岁女性,既往诊断为原发性高血压,孕期出现严重高血压,并伴有胰岛素依赖型妊娠期糖尿病。在常规给予倍他米松后发生高血压危象,于32周时行剖宫产。产后儿茶酚胺过量的体征和症状持续存在,导致诊断为左肾上腺嗜铬细胞瘤。在诊断至计划切除肿瘤期间,患者出现了库欣综合征的体征和症状(面部水肿、多毛、肌病和疲劳)。生化检测证实存在皮质醇增多症,血浆促肾上腺皮质激素、尿皮质醇及血浆检测的多种类固醇水平极度升高,而此前检测均正常。先前的去甲肾上腺素能肿瘤也开始分泌肾上腺素。组织病理学检查证实为嗜铬细胞瘤,免疫组化检测促肾上腺皮质激素也呈阳性。术后一年血压和生化指标恢复正常,实现了完全康复。本报告不仅强调了嗜铬细胞瘤的多变性,还说明了其呈现转变表现的可能性。对于妊娠期高血压患者,使用皮质类固醇需要谨慎。