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Clinical features and management of ectopic ACTH syndrome at a single institute in Japan

机译:日本一家机构的异位ACTH综合征的临床特征和治疗

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摘要

Ectopic ACTH syndrome (EAS) is a diagnostic challenge because it is often indistinguishable from Cushing's disease. We describe our series of EAS patients referred to us during 1992-2009. Among 16 cases (9 females / 7 males), with mean age of 58.4 ± 19.0yr, the ectopic source was identified in ten (proven EAS), whereas unidentified in six (occult/ unknown EAS). Their salient clinical manifestations included Cushingoid feature (88%), skin pigmentation (88%), profound hypokalemia (88%), hypertension (75%), diabetes/impaired glucose tolerance (75%), hyperlipidemia (69%), and severe infection (44%). Dynamic endocrine tests revealed markedly elevated plasma ACTH levels (211 ± 116pg/mL) and cortisol levels (60.9 ± 30.1μg/dL) which showed resistance to overnight high-dose (8mg) dexamethasone suppression test in 15 (94%) and unresponsiveness to CRH stimulation in 12 (75%). No ACTH gradient during inferior petrosal sampling was noted in 13 of 15 (87%). Imaging tests by CT/MRI identified the tumors in 8 of 16 (50%), in 4 of 11 (36%) and 4 of 6 (66.7%) octreotide-responders by somatostatin receptor scintigraphy, but in only one of 9 (11.1%) by FDG-PET scan. Six cases deceased, including small cell carcinoma (2) and adenocarcinoma (1) of lung, neuroendocrine carcinoma of pancreas (1) and stomach (1), and olfactory neuroblastoma (1), whereas 4 cases survived after removal of the rumors, including bronchial carcinoid tumor (3) and thymic hyperplasia (1). Six occult/unknown EAS patients survived for 67.5 months after medical treatment with metyrapone to control hypercortisolism. Thus, various endocrine tests combined with imaging studies are required to correctly localize the tumors. Control of hypercortisolemia by metyrapone, even if tumor is unrecognized, is critical for better prognosis, and the long-term follow-up by repeated endocrine and imaging tests is mandatory.
机译:异位促肾上腺皮质激素综合症(EAS)是一项诊断难题,因为它通常与库欣氏病无法区分。我们描述了1992-2009年间转诊给我们的一系列EAS患者。在平均年龄为58.4±19.0岁的16例患者(9例女性/ 7例男性)中,有十例(经证实的EAS)发现了异位来源,而六例(隐匿性/未知EAS)中发现了异位来源。他们的主要临床表现包括库欣类特征(88%),皮肤色素沉着(88%),严重低钾血症(88%),高血压(75%),糖尿病/糖耐量异常(75%),高脂血症(69%)和严重感染(44%)。动态内分泌测试显示血浆ACTH水平(211±116pg / mL)和皮质醇水平(60.9±30.1μg/ dL)显着升高,显示对15天(94%)的过夜高剂量(8mg)地塞米松抑制试验有抵抗力,对有12位(75%)的CRH刺激。 15名患者中有13名(87%)在下岩质采样期间未发现ACTH梯度。 CT / MRI成像检查通过生长抑素受体闪烁显像法在奥曲肽应答者中发现了16例中的8例(50%),11例中的4例(36%)和6例中的4例(66.7%),但9例中只有1例(11.1) %)通过FDG-PET扫描。死亡6例,包括小细胞癌(2)和肺腺癌(1),胰腺神经内分泌癌(1)和胃(1)和嗅觉神经母细胞瘤(1),而4例在去除谣言后幸存,包括支气管类癌(3)和胸腺增生(1)。接受甲吡酮治疗以控制皮质醇过多的药物治疗后,有6位隐匿性/未知的EAS患者存活了67.5个月。因此,需要各种内分泌检查和影像学研究来正确定位肿瘤。甲吡酮控制高皮质醇血症,即使无法识别肿瘤,对于更好的预后也至关重要,并且必须通过反复的内分泌和影像学检查进行长期随访。

著录项

  • 来源
    《Endocrine journal》 |2010年第12期|p.1061-1069|共9页
  • 作者单位

    Department of Clinical and Molecular Endocrinology, Tokyo Medical and Dental University Graduate School, Tokyo 113-8519, Japan;

    Department of Clinical and Molecular Endocrinology, Tokyo Medical and Dental University Graduate School, Tokyo 113-8519, Japan;

    Department of Clinical and Molecular Endocrinology, Tokyo Medical and Dental University Graduate School, Tokyo 113-8519, Japan;

    Department of Clinical and Molecular Endocrinology, Tokyo Medical and Dental University Graduate School, Tokyo 113-8519, Japan;

    Department of Clinical and Molecular Endocrinology, Tokyo Medical and Dental University Graduate School, 1-5-45, Yushima, Bunkyo-ku, Tokyo 113-8519, Japan;

  • 收录信息 美国《科学引文索引》(SCI);美国《化学文摘》(CA);
  • 原文格式 PDF
  • 正文语种 eng
  • 中图分类
  • 关键词

    ectopic ACTH syndrome (EAS); octreotide suppression test; somatostatin receptor scintigraphy (SRS); metyrapone;

    机译:异位ACTH综合征(EAS);奥曲肽抑制试验;生长抑素受体闪烁显像(SRS);甲吡酮;
  • 入库时间 2022-08-18 01:33:33

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