Quan Cheng, Zhang Yingchao, Wang Yu
Department of Vascular Surgery, the Second Hospital of Jilin University, Changchun, China.
Department of Breast Surgery, the Second Hospital of Jilin University, Changchun, China.
Gland Surg. 2021 Oct;10(10):3053-3057. doi: 10.21037/gs-21-296.
Adrenocortical carcinoma (ACC) is a very rare and challenging malignancy with a dismal prognosis. The patients usually suffer from primary or metastatic tumors, and about half of the tumors have hormone secretion function to cause Cushing's syndrome (CS) and hypercortisolism. The optimal management and clinical outcomes of ACC remain ill-defined due to the rarity of the disease. Due to the failure of effective treatment, surgery remains the main treatment for ACC, which includes distant resectable metastases and ACC recurrent. We reported a 34-year-old woman who was diagnosed with left-sided ACC at another hospital and then underwent left adrenalectomy for ACC 2 years ago. Then she was admitted to our hospital with CS caused by adrenocorticotropic hormone (ACTH) secretion from an ACC metastatic to the liver. She underwent a complete resection of the tumors in the liver and was discharged without any severe complications after hormone replacement therapy. Unfortunately, six months after the hepatectomy, she eventually died due to progressive deterioration and the refusal of further treatment. ACC is a rare and challenging disease with few durable systemic options. Due to the difficulty of full cure, prompt serial follow-up after the operation is probably crucial for a better prognosis.
肾上腺皮质癌(ACC)是一种非常罕见且具有挑战性的恶性肿瘤,预后不佳。患者通常患有原发性或转移性肿瘤,约一半的肿瘤具有激素分泌功能,可导致库欣综合征(CS)和皮质醇增多症。由于该疾病罕见,ACC的最佳治疗方法和临床结果仍不明确。由于缺乏有效的治疗方法,手术仍然是ACC的主要治疗手段,包括可切除的远处转移灶和ACC复发。我们报告了一名34岁女性,她在另一家医院被诊断为左侧ACC,两年前接受了左侧肾上腺切除术。随后,她因ACC转移至肝脏分泌促肾上腺皮质激素(ACTH)导致CS入住我院。她接受了肝脏肿瘤的完整切除,在激素替代治疗后无任何严重并发症出院。不幸的是,肝切除术后六个月,她最终因病情逐渐恶化且拒绝进一步治疗而死亡。ACC是一种罕见且具有挑战性的疾病,几乎没有持久的全身治疗选择。由于难以完全治愈,术后及时进行连续随访可能对改善预后至关重要。