Witek Przemysław, Witek Joanna, Zieliński Grzegorz, Podgajny Zbigniew, Kamiński Grzegorz
Department of Endocrinology and Isotope Therapy, Military Institute of Medicine, Warsaw, Poland.
Outpatient Clinic, Institute of Mother and Child, Warsaw, Poland.
Neuro Endocrinol Lett. 2015;36(3):201-8.
Ectopic adrenocorticotropic hormone secretion (EAS) is responsible for approximately 10-15% cases of Cushing's syndrome. EAS is associated with various tumors such as small cell lung cancer and well-differentiated bronchial or gastrointestinal neuroendocrine tumors. Hormonal diagnostics include assessments in basic conditions as well as dynamic tests, such as the high-dose dexamethasone suppression test and corticotrophin releasing hormone (CRH) stimulation test. Treatment selection depends on the type of tumor and its extent. In the case of neuroendocrine tumors, the main treatments are surgery and administration of somatostatin analogs that may be additionally radiolabeled for targeted radiotherapy. The tumor histology and the presence and control of hypercortisolemia and metastases are of major importance in prognosis. In this article we presented the principles of modern hormonal and imaging diagnostics techniques as well as the key issues associated with treatment of ACTH-dependent Cushing's syndrome due to EAS.
异位促肾上腺皮质激素分泌（EAS）导致了约10%-15%的库欣综合征病例。EAS与多种肿瘤相关，如小细胞肺癌以及高分化支气管或胃肠道神经内分泌肿瘤。激素诊断包括基础状态评估以及动态试验，如大剂量地塞米松抑制试验和促肾上腺皮质激素释放激素（CRH）刺激试验。治疗方案的选择取决于肿瘤的类型及其范围。对于神经内分泌肿瘤，主要治疗方法是手术和使用生长抑素类似物，这些类似物还可进行放射性标记用于靶向放疗。肿瘤组织学以及高皮质醇血症和转移灶的存在与控制对预后至关重要。在本文中，我们阐述了现代激素和影像学诊断技术的原理以及与因EAS导致的促肾上腺皮质激素（ACTH）依赖性库欣综合征治疗相关的关键问题。
