Klepinowska Marta, Sowińska-Przepiera Elżbieta, Andrysiak-Mamos Elżbieta, Kiedrowicz Bartosz, Sagan Karol, Syrenicz Anhelli
Department of Endocrinology, Metabolic and Internal Diseases, Pomeranian Medical University Hospital No. 1, Szczecin, Poland, Szczecin, Poland.
Endokrynol Pol. 2025;76(3):229-235. doi: 10.5603/ep.104445.
Adrenocortical carcinoma (ACC) is a rare malignant neoplasm. Hypercortisolism and inhibition of gonadotropin secretion usually result in menstrual disorders and secondary amenorrhea. The coincidence of ACC and pregnancy is therefore extremely rare. The signs of hypercortisolism are commonly seen in otherwise healthy pregnancies, which decreases the doctor's vigilance. We present the diagnostic challenges and current treatment recommendations according to European guidelines from the European Society of Endocrinology (ESE) and European Network for the Study of Adrenal Tumors (ENS@T) 2018 and Polish guidelines 2024.
We conducted an extensive search via MEDLINE using the phrases "Adrenocortical carcinoma", "ACC", and "Pregnancy" without temporal or language restrictions. Only cases with ACC diagnosed during pregnancy were taken into consideration. Ten papers were found, with 12 described cases. We analyzed the management and outcome both for the mother and the child. We also included a case of a woman treated in our department. A 29-year-old woman in the 20th/21st gestation week (GW) presented to us with Cushing's syndrome symptoms and androgenization. Laboratory tests showed low plasma adrenocorticotropic hormone (ACTH), high cortisol, testosterone, dehydroepiandrosterone sulfate (DHEA-SO4), androstenedione, 24-hour urinary free cortisol (UFC), and hypokalemia. In the abdominal magnetic resonance imaging (MRI) there was a mass in the left adrenal gland. An open surgery was performed in the 21st GW with no perioperative complications. The pathology report established the diagnosis of ACC. The tumor board along with the patient decided to defer the adjuvant therapy until the 32nd GW to increase the odds for the fetus to survive. In the 31st GW an urgent caesarian section was performed due to risk of fetal hypoxia. Computed tomography (CT) scan after the delivery showed local recurrence in the tumor bed. The patient was qualified to mitotane therapy and underwent tumor bed radiotherapy followed by chemotherapy, but the treatment did not stop the progression of the disease. She passed away 14 months after the diagnosis.
It is critical to remember about the possibility of ACC occurrence during pregnancy, as well as to know about the differences in hormonal tests in pregnant women such as higher free plasma cortisol, ACTH, UFC, and high rate of false-positive results of low-dose dexamethasone suppression test (LDDST) in comparison to non-pregnant women. Therapeutical options are scarce and pose an ethical dilemma.
肾上腺皮质癌（ACC）是一种罕见的恶性肿瘤。皮质醇增多症和促性腺激素分泌受抑制通常会导致月经紊乱和继发性闭经。因此，ACC与妊娠同时发生极为罕见。皮质醇增多症的体征在其他方面健康的妊娠中很常见，这降低了医生的警惕性。我们根据欧洲内分泌学会（ESE）和欧洲肾上腺肿瘤研究网络（ENS@T）2018年的欧洲指南以及2024年的波兰指南，介绍诊断挑战和当前的治疗建议。
我们通过MEDLINE进行了广泛检索，使用了“肾上腺皮质癌”“ACC”和“妊娠”等短语，没有时间或语言限制。仅考虑在妊娠期间诊断为ACC的病例。共找到10篇论文，其中描述了12例病例。我们分析了母亲和孩子的治疗及结局。我们还纳入了在我们科室接受治疗的一名女性病例。一名29岁女性，妊娠第20/21周（GW），出现库欣综合征症状和雄激素化表现。实验室检查显示血浆促肾上腺皮质激素（ACTH）水平低，皮质醇、睾酮、硫酸脱氢表雄酮（DHEA-SO4）、雄烯二酮、24小时尿游离皮质醇（UFC）水平高，且存在低钾血症。腹部磁共振成像（MRI）显示左肾上腺有一个肿块。在妊娠第21周进行了开放手术，无围手术期并发症。病理报告确诊为ACC。肿瘤委员会与患者决定将辅助治疗推迟至妊娠第32周，以增加胎儿存活几率。在妊娠第31周，由于胎儿缺氧风险进行了紧急剖宫产。产后计算机断层扫描（CT）显示肿瘤床局部复发。患者符合米托坦治疗条件，接受了肿瘤床放疗，随后进行化疗，但治疗未能阻止疾病进展。她在确诊后14个月去世。
必须牢记妊娠期间发生ACC的可能性，以及了解孕妇激素检测的差异，例如与非孕妇相比，孕妇血浆游离皮质醇、ACTH、UFC水平较高，且低剂量地塞米松抑制试验（LDDST）假阳性率较高。治疗选择有限，且存在伦理困境。
