肾上腺皮质癌（ACC）是一种起源于肾上腺皮质的罕见恶性肿瘤，其特点是 5 年生存率较差。其发生频率为每 200 万人口中有 2-4 例。女性比男性更容易受到影响，并且大多在四五十岁时被发现。在大多数情况下，由于体细胞肾上腺皮质细胞中的基因驱动突变，癌发生是偶发的，在其他情况下，它可能是基因决定的综合征的一部分，例如李法美尼综合征或沃默综合征（I 型多发性内分泌腺瘤病） 。 ACC 最常见的情况是在初始阶段没有任何症状，导致诊断不佳。由于缺乏早期检测，肿瘤不被认为是恶性的，从而降低了进一步治疗的益处。有时，只有在复发或出现转移后，切除的肿瘤确实是肾上腺皮质癌这一事实才变得清楚。我们介绍了一名 46 岁女性的肾上腺皮质癌病例，她在出现症状 1.5 年后就去看医生。这个临床病例说明了恶性肿瘤晚期诊断的后果。我们想强调利用实验室仪器和基因组分析的所有潜力及时检测肿瘤的重要性。由于肿瘤意识较低，患者寻求医疗帮助的速度很慢，这不仅导致了转移，还导致了心血管系统的并发症。

Adrenocortical carcinoma (ACC) is a rare malignant tumor originating in the adrenal cortex and characterized by poor 5-year survival. It occurs with a frequency of 2-4 cases per 2 million in the population. Women are more frequently affected than men and it is mostly detected in the fourth and fifth decades. In the most of cases, the cancerogenesis occurs sporadically because of gene driver mutations in somatic adrenocortical cells, in other cases it can be found as part of a genetically determined syndrome such as Li-Fraumeni syndrome or Wermer's syndrome (multiple endocrine adenomatosis type I). ACC most frequently happens occurs without symptoms in the initial stages leading to poor diagnoses. Because of this lack of early detection, the tumor is not considered malignant reducing the benefits of further treatment. Sometimes the fact that the resected tumor is indeed adrenocortical carcinoma becomes clear only after recurrence, or after the appearance of metastases. We present a case of adrenocortical carcinoma in a 46-year-old woman who went to the doctor in 1.5 year after symptoms were manfested. This clinical case illustrates the consequences of late diagnosis of a malignant tumor. We would like to emphasize the importance of timely detection of a neoplasm, using all of the potential of laboratory-instrumental and genomic analysis. Due to low oncological awareness, our patient was slow to seek medical help, which in turn led not only to metastases, but also to complications in the cardiovascular system.