Salgado Luiz Roberto, Fragoso Maria Candida B Villares, Knoepfelmacher Mirta, Machado Marcio Carlos, Domenice Sorahia, Pereira Maria Adelaide Albergaria, de Mendonça Berenice Bilharinho
Division of Endocrinology and Metabolism, Hospital das Clínicas, University of Sao Paulo Medical School, Avenida Dr Eneas de Carvalho Aguiar, 155, 8th floor, Cerqueira Cesar, 05403-060 Sao Paulo, Brazil.
Eur J Endocrinol. 2006 Nov;155(5):725-33. doi: 10.1530/eje.1.02278.
Ectopic ACTH syndrome (EAS) occurs in about 5-10% of all patients with ACTH-dependent hypercortisolism with most of them caused by intrathoracic neoplasms. It may be associated with overt malignancies or with occult and indolent tumors. We assessed the accuracy of dynamic tests, inferior petrosal sinus sampling (IPSS) using desmopressin, and imaging in the work-up diagnosis of EAS.
Tumor markers, imaging, and outcome data from 25 patients (13F/12M) aged 18-72 years. High dexamethasone suppression test (HDDST), desmopressin test, GHRP-6 test, corticotropin-releasing hormone (CRH) test, IPSS, computed tomography (CT), magnetic resonance imaging (MRI), and (111)In-pentetreotide scintigraphy were revised.
In 5 out of 20 patients HDDST was positive. In 13 patients who underwent desmopressin test, ACTH- and cortisol-positive responses were seen in six and five patients respectively. GHRP-6 test was positive in two out of three cases. Two patients underwent CRH test with negative response. In the seven patients submitted to IPSS using desmopressin in six of them, none had ACTH gradients. CT was positive in 15 out of 21 patients and MRI in 8 out of 17 cases. (111)In-pentetreotide scintigraphy was positive in three out of five patients. Fourteen patients had intrathoracic tumors, five had pheochromocytomas, three had pancreatic tumors, one had a glomic tumor, and had three occult tumors. Six out of 11 patients with metastasis died and 3 others without metastasis died.
IPSS with desmopressin was helpful for differential diagnosis. Patients initially harboring occult carcinoids may also exhibit severe hypercortisolism and those harboring tymic carcinoids had poor prognoses when compared with bronchial carcinoids and pheocromocytomas.
异位促肾上腺皮质激素（ACTH）综合征（EAS）发生于约5% - 10%的所有依赖ACTH的皮质醇增多症患者中，其中大多数由胸内肿瘤引起。它可能与明显的恶性肿瘤或隐匿性惰性肿瘤有关。我们评估了动态试验、使用去氨加压素的岩下窦采血（IPSS）以及影像学检查在EAS诊断工作中的准确性。
来自25例年龄在18 - 72岁患者（13例女性/12例男性）的肿瘤标志物、影像学检查及预后数据。回顾了高剂量地塞米松抑制试验（HDDST）、去氨加压素试验、生长激素释放肽-6（GHRP - 6）试验、促肾上腺皮质激素释放激素（CRH）试验、IPSS、计算机断层扫描（CT）、磁共振成像（MRI）以及铟-111奥曲肽闪烁扫描。
20例患者中有5例HDDST呈阳性。13例接受去氨加压素试验的患者中，分别有6例和5例出现ACTH和皮质醇阳性反应。3例患者中有2例GHRP - 6试验呈阳性。2例患者接受CRH试验，反应均为阴性。7例接受使用去氨加压素的IPSS检查的患者中，6例未出现ACTH梯度。21例患者中有15例CT检查呈阳性，17例患者中有8例MRI检查呈阳性。5例患者中有3例铟-111奥曲肽闪烁扫描呈阳性。14例患者有胸内肿瘤，5例有嗜铬细胞瘤，3例有胰腺肿瘤，1例有球旁细胞瘤，3例有隐匿性肿瘤。11例有转移的患者中有6例死亡，另外3例无转移的患者也死亡。
使用去氨加压素的IPSS有助于鉴别诊断。最初患有隐匿性类癌的患者也可能表现出严重的皮质醇增多症，与支气管类癌和嗜铬细胞瘤相比，患有胸腺类癌的患者预后较差。
