Department of Internal Medicine, Seoul National University Bundang Hospital, 300 Gumi-dong, Bundang-gu, Seongnam-si, Gyeongi-do 463-707, Republic of Korea.
Jpn J Clin Oncol. 2011 Nov;41(11):1287-91. doi: 10.1093/jjco/hyr144. Epub 2011 Oct 6.
We report the first case of adrenocortical carcinoma secreting cortisol (Cushing's syndrome) and aldosterone (Conn's syndrome) with extensive distant metastasis at the time of diagnosis. A 72-year-old male with exertional dyspnea sought evaluation at our institution. The pattern of tumor spread (lung, pleura, bone and adrenal gland) and respiratory symptoms secondary to the tumor led clinicians to diagnose the primary tumor site as lung cancer and the adrenal mass as a metastatic site. However, endocrinologic studies and a biopsy revealed the primary site to be adrenocortical carcinoma. After histopathologic confirmation, the patient was treated with palliative chemotherapy, including mitotane, cisplatin, etoposide and doxorubicin. The patient died on the 14th day after chemotherapy of rapidly progressive and unexpected pneumonia, which was thought to be an opportunistic infection secondary to Cushing's syndrome. Our case suggests that a thorough endocrinologic investigation is important in patients with an adrenal mass and clinicians should be aware that patients with adrenocortical carcinoma and Cushing's syndrome are susceptible to infections and need to be observed carefully for the possible development of unrecognized opportunistic infections.
我们报告了首例在诊断时即分泌皮质醇(库欣综合征)和醛固酮(Conn 综合征)且广泛远处转移的肾上腺皮质癌病例。一名 72 岁男性因劳累性呼吸困难到我院就诊。肿瘤的扩散模式(肺部、胸膜、骨骼和肾上腺)以及肿瘤引起的呼吸症状导致临床医生将原发肿瘤部位诊断为肺癌,将肾上腺肿块诊断为转移部位。然而,内分泌研究和活检显示原发性部位为肾上腺皮质癌。在组织病理学确认后,患者接受了姑息化疗,包括米托坦、顺铂、依托泊苷和多柔比星。患者在化疗后第 14 天死于进展迅速且意外的肺炎,这被认为是库欣综合征继发的机会性感染。我们的病例表明,对于肾上腺肿块患者,全面的内分泌学检查很重要,临床医生应该意识到患有肾上腺皮质癌和库欣综合征的患者容易发生感染,需要密切观察可能出现的未被识别的机会性感染。