Division of Endocrinology and Metabolism, Department of Internal Medicine, Cerrahpasa Faculty of Medicine, University of Istanbul, Istanbul, Turkey.
Neurosurgery. 2009 Dec;65(6 Suppl):E110-1; discussion E111. doi: 10.1227/01.NEU.0000344004.56155.B6.
We aim to report a case of Kaposi sarcoma (KS) with Cushing's syndrome caused by endogenic glucocorticoid-induced immunosuppression.
A 43-year-old woman presented with delirium, hirsutism, fatigue, and hypertension. At the time of presentation, physical findings showed a Cushingoid appearance, with moon-like facies, hirsutism, and hyperpigmentation. Laboratory findings showed the following: adrenocorticotropic hormone, 86.7 pg/mL (normal range, 0-46 pg/mL); baseline cortisol level, 50 microg/dL (normal range, 6.2-19 microg/dL); potassium, 2.2 mEq/L (normal range, 3.5-5 mEq/L); and midnight cortisol level, 33 microg/dL. Serum cortisol levels failed to suppress after low and high doses of dexamethasone; these findings confirmed the diagnosis of ectopic adrenocorticotropic hormone production. Magnetic resonance imaging revealed a 12 x 15-mm, round, hypothalamic mass lesion in the center of the median eminence.
Endoscopic biopsy from the floor of the third ventricle was performed, and pathological examination of the lesion showed a diffuse adrenocorticotropic hormone-secreting adenoma. The patient developed diffuse skin lesions that were proven to be a KS by skin biopsy while she was prepared for transcranial surgery. After surgical removal of the adenoma, she became hypocortisolemic and required cortisol replacement. Within 1 month after surgery, all KS lesions disappeared spontaneously.
Excessive cortisol may induce immunosuppression. KS is one of the most common malignant tumors of patients with immunosuppression. To the best of our knowledge, this is the first case of Cushing's syndrome with KS caused by endogenous glucocorticoid-induced immunosuppression.
我们旨在报告一例由内源性糖皮质激素诱导的免疫抑制引起的库欣综合征伴卡波西肉瘤（KS）病例。
一名 43 岁女性出现谵妄、多毛症、疲劳和高血压。就诊时，体格检查表现为库欣样外观，出现满月脸、多毛症和色素沉着过度。实验室检查发现：促肾上腺皮质激素，86.7 pg/mL（正常值范围，0-46 pg/mL）；基础皮质醇水平，50 mcg/dL（正常值范围，6.2-19 mcg/dL）；钾，2.2 mEq/L（正常值范围，3.5-5 mEq/L）；午夜皮质醇水平，33 mcg/dL。地塞米松低剂量和高剂量后血清皮质醇水平未能抑制；这些发现证实了异位促肾上腺皮质激素分泌的诊断。磁共振成像显示在正中隆起中心有一个 12 x 15 毫米的圆形下丘脑肿块病变。
对第三脑室底部进行了内镜活检，病变的病理检查显示为弥漫性促肾上腺皮质激素分泌腺瘤。在准备颅穿手术的过程中，患者出现弥漫性皮肤病变，经皮肤活检证实为 KS。在腺瘤切除后，她出现了皮质醇不足，并需要皮质醇替代治疗。手术后 1 个月内，所有 KS 病变均自发消失。
过多的皮质醇可能会导致免疫抑制。KS 是免疫抑制患者中最常见的恶性肿瘤之一。据我们所知，这是首例由内源性糖皮质激素诱导的免疫抑制引起的库欣综合征伴 KS 病例。
