Höfle G, Gasser R W, Lhotta K, Janetschek G, Kreczy A, Finkenstedt G
Department of Internal Medicine, University of Innsbruck, Austria.
Horm Res. 1998;50(4):237-42. doi: 10.1159/000023282.
A 43-year-old female patient underwent abdominal ultrasonography and CT scan because of uncharacteristic abdominal pain. A 3-cm homogeneous adrenal tumor was diagnosed. The endocrine tests revealed an adrenal preclinical Cushing's syndrome (PCS). Due to the latent hormone excess we decided to operate on the adrenal tumor. Since the tumor was small, laparoscopic adrenalectomy was performed. Histological evaluation showed an adrenocortical tumor of undetermined nature. Four months later the patient presented with a metastasizing cortisol- and androgen-producing adrenocortical carcinoma (ACC). After pretreatment with ketoconazole to suppress the biosynthesis of adrenal steroids under substitution with hydrocortisone, we reduced the tumor load by surgery. Postoperatively we continued ketoconazole and started o, p'-dichlorodiphenyldichloroethane as well as chemotherapy with doxorubicin and suramin. However, the patient died from ACC 7 months after adrenalectomy. It is known from several reports that PCS may persist clinically silently or may progress to full-blown Cushing's syndrome. This is the first time a malignant course of PCS is described. Independent of the initial therapeutic strategy of PCS, i. e. surgery or regular follow-up visits, we must be aware that also relatively small adrenal tumors can harbor malignancy.
一名43岁女性患者因不明原因的腹痛接受了腹部超声检查和CT扫描。诊断出一个3厘米大小的肾上腺均匀性肿瘤。内分泌检查显示为肾上腺临床前库欣综合征（PCS）。由于存在潜在的激素过量，我们决定对肾上腺肿瘤进行手术。鉴于肿瘤较小，遂行腹腔镜肾上腺切除术。组织学评估显示为性质未明的肾上腺皮质肿瘤。四个月后，患者出现转移性分泌皮质醇和雄激素的肾上腺皮质癌（ACC）。在用酮康唑预处理以抑制肾上腺类固醇生物合成并同时给予氢化可的松替代治疗后，我们通过手术降低了肿瘤负荷。术后我们继续使用酮康唑，并开始使用邻对二氯二苯二氯乙烷以及阿霉素和苏拉明进行化疗。然而，患者在肾上腺切除术后7个月死于肾上腺皮质癌。从多篇报道中可知，PCS可能在临床上无症状地持续存在，也可能进展为典型的库欣综合征。这是首次描述PCS的恶性病程。无论PCS最初的治疗策略是手术还是定期随访，我们都必须意识到，相对较小的肾上腺肿瘤也可能隐匿恶性病变。
