Cushing's syndrome

库欣综合征
  • 文章类型: Journal Article
    目的:介绍一系列由肾上腺皮质腺瘤引起的妊娠库欣综合征(CS),突出临床特征,荷尔蒙评估和结果。
    方法:我们描述了五名患有CS的孕妇,详细介绍临床表现和实验室检查结果。
    结果:常见的临床特征包括满月脸,水牛背部和严重的高血压。观察到血液皮质醇水平升高,昼夜节律中断,促肾上腺皮质激素(ACTH)水平被抑制。影像学显示单侧肾上腺肿瘤。2例妊娠中期行腹腔镜肾上腺切除术,三个人做了产后手术。所有需要的激素替代疗法,术后病理证实为肾上腺皮质腺瘤。
    结论:由于与正常妊娠重叠的特征,妊娠期间CS的诊断具有挑战性:血液皮质醇水平升高和血液皮质醇昼夜节律异常,抑制援助诊断。由于缺乏明确的最佳治疗方法,治疗应个体化。腹腔镜肾上腺切除术可能是最佳选择,包括激素替代疗法在内的多学科管理。
    OBJECTIVE: To present a case series of Cushing\'s syndrome (CS) during pregnancy caused by adrenocortical adenomas, highlighting clinical features, hormonal assessments and outcomes.
    METHODS: We describe five pregnant women with CS, detailing clinical presentations and laboratory findings.
    RESULTS: Common clinical features included a full moon face, buffalo back and severe hypertension. Elevated blood cortisol levels with circadian rhythm disruption and suppressed adrenocorticotrophic hormone (ACTH) levels were observed. Imaging revealed unilateral adrenal tumours. Two cases underwent laparoscopic adrenalectomies during the second trimester, while three had postpartum surgery. All required hormone replacement therapy, with postoperative pathological confirmation of adrenocortical adenomas.
    CONCLUSIONS: Diagnosis of CS during pregnancy is challenging due to overlapping features with normal pregnancy: elevated blood cortisol levels and abnormal diurnal rhythm of blood cortisol, suppressed aid diagnosis. Treatment should be individualised due to a lack of explicit optimum therapeutic approaches. Laparoscopic adrenalectomy may be an optimal choice, along with multidisciplinary management including hormone replacement therapy.
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  • 文章类型: Case Reports
    肾上腺皮质嗜酸细胞腺瘤通常表现为无功能性肾上腺肿瘤,但可能导致患者出现库欣综合征。本文报道1例由右肾上腺皮质嗜酸细胞瘤引起的库欣综合征。患者在武川市人民医院泌尿外科(遵义,中国),2022年11月,由于多毛症,体重增加和高血压。使用腹部入路进行腹腔镜右肾上腺肿瘤切除术。手术后,患者的血压和心率在健康范围内波动,月经恢复正常。腹腔镜肾上腺切除术较开腹肾上腺切除术有明显优势,比如更少的创伤,恢复时间短,并发症少。因此,这种罕见疾病的治疗是安全可行的。
    Adrenal cortical eosinophilic adenoma usually presents as non-functional adrenal tumor but may lead to Cushing\'s syndrome in patients. The present article reports a patient with Cushing\'s syndrome caused by right adrenocortical oncocytoma. The patient was treated in Urology Department of Wuchuan People\'s Hospital (Zunyi, China) in November 2022 because of hirsutism, weight gain and hypertension. A laparoscopic right adrenal tumor resection was performed using an abdominal approach. Following surgery, blood pressure and heart rate of the patient fluctuated within a healthy range and menstruation returned to normal. Laparoscopic adrenalectomy has obvious advantages over open adrenalectomy, such as less trauma, shorter recovery time and fewer complications. Thus, this treatment for this rare disease is safe and feasible.
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  • 文章类型: Case Reports
    肾上腺皮质癌(ACC)是一种非常罕见的恶性肿瘤,预后不良。它主要出现在生命的第四至第五个十年中,在白人女性中更为常见。ACC最常被检测为偶然发现,但可能有其他表现,如急发性库欣综合征或肺栓塞。在目前的情况下,在成像过程中偶然发现了一名24岁女性的ACC,病人后来出现了肺栓塞.实验室调查提示皮质醇增多症以及高雄激素血症。术前使用β受体阻滞剂治疗后,metyrapone,和治疗性抗凝,她接受了左根治性肾切除术,左开放肾上腺切除术和下腔静脉(IVC)切除和重建。手术并不复杂,她出院了,并计划进行门诊辅助化疗。此病例突显了一个事实,即看似无缘无故的肺栓塞可能表明潜在的隐匿性恶性肿瘤的可能性,并且未发现的ACC应包括在此类病例的鉴别诊断中。
    Adrenocortical carcinoma (ACC) is a very rare malignancy with a poor prognosis. It is predominantly noted in the fourth to fifth decades of life and is more common in White females. ACC is most commonly detected as an incidental finding but may have other presentations, such as rapid-onset Cushing\'s syndrome or pulmonary embolism. In the current case, ACC was incidentally observed in a 24-year-old female during imaging, and the patient later developed a pulmonary embolism. Lab investigations were suggestive of hypercortisolism along with hyperandrogenism. Following preoperative treatment with beta-blockers, metyrapone, and therapeutic anticoagulation, she underwent left radical nephrectomy with left open adrenalectomy and inferior vena cava (IVC) resection and reconstruction. Surgery was uncomplicated, and she was discharged with plans for outpatient adjuvant chemotherapy. This case highlights the fact that a seemingly unprovoked pulmonary embolism may point to the possibility of an underlying occult malignancy and undetected ACC should be included in the differential diagnosis of such cases.
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  • 文章类型: Case Reports
    背景:由促肾上腺皮质激素(ACTH)分泌嗜铬细胞瘤引起的皮质醇增多症的发生极为罕见,到目前为止,记录的实例有限。
    方法:我们介绍一例患有严重代谢紊乱的异位ACTH分泌型嗜铬细胞瘤患者。我们的临床病例概述了诊断史,术前纠正患者的代谢紊乱和手术策略治疗罕见的异位ACTH产生嗜铬细胞瘤。
    结论:异位分泌促肾上腺皮质激素的嗜铬细胞瘤表现出多方面的临床特征,需要及时诊断和多学科管理,以克服相关的严重的临床错乱。
    结论:对于异位ACTH分泌的诊断,生化和激素检测与成像程序的结合是强制性的,在肾上腺肿块存在的情况下,应考虑分泌ACTH的嗜铬细胞瘤的可能性.
    BACKGROUND: The occurrence of hypercortisolism resulting from adrenocorticotropic hormone (ACTH)-secreting pheochromocytoma is exceedingly uncommon, with limited documented instances thus far.
    METHODS: We present a case of ectopic ACTH-secreting pheochromocytoma in a patient who suffered from severe metabolic disorders. Our clinical case outlines the diagnostic history, preoperative correction of the patient\'s metabolic disturbances and surgical strategy for management of a rare ectopic ACTH producing pheochromocytoma.
    CONCLUSIONS: Ectopic adrenocorticotropic hormone-secreting pheochromocytoma displays multifaceted clinical features and requires prompt diagnosis and multidisciplinary management in order to overcome the related severe clinical derangements.
    CONCLUSIONS: The combination of biochemical and hormonal testing and imaging procedures is mandatory for the diagnosis of ectopic ACTH secretion, and in the presence of an adrenal mass, the possibility of an ACTH-secreting pheochromocytoma should be taken into account.
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  • 文章类型: Case Reports
    胃肠道穿孔是外源性皮质醇增多症的一个很好解决的并发症;然而,内源性库欣综合征(CS)患者在临床实践中通常不会出现这种情况。有关该主题的文献有限,仅包括临床病例报告/系列,仅有23例内源性库欣综合征患者发生胃肠道穿孔。这主要归因于库欣综合征本身的罕见性以及此类并发症发生的机率低。我们报告了一例最近诊断为促肾上腺皮质激素(ACTH)依赖性库欣综合征的30岁女性,最初有三个月的进行性体重增加史,广义弱点,痤疮,月经不调,和严重的低钾血症,然后在ACTH依赖性库欣综合征诊断后仅一个月出现胃溃疡穿孔,并通过紧急手术治疗。
    Gastrointestinal perforation is a well-addressed complication of exogenous hypercortisolism; however, patients with endogenous Cushing\'s syndrome (CS) do not usually experience this condition in clinical practice. The literature on this subject is limited and consists solely of clinical case reports/series with only 23 instances of gastrointestinal perforation occurring in individuals with endogenous Cushing\'s syndrome. This is mainly attributed to the rarity of Cushing\'s syndrome itself and the low chance of occurrence of such complications. We report a case of a recently diagnosed adrenocorticotropic hormone (ACTH)-dependent Cushing\'s syndrome in a 30-years-old female who presented initially with a three-month history of progressive weight gain, generalized weakness, acne, menstrual irregularity, and severe hypokalemia, and then developed a gastric ulcer perforation only one month after her ACTH-dependent Cushing\'s syndrome diagnosis and was managed through emergent surgery.
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  • 文章类型: Case Reports
    Sertoli-Leydig细胞瘤(SLCT)是一种罕见的卵巢肿瘤。库欣综合征(CS),另一方面,是由于任何原因血液中长期高水平的糖皮质激素以及由此产生的症状而形成的临床表现。在某些情况下,远离肾上腺区域的肿瘤合成肾上腺皮质激素。在这些异位肿瘤中,CS分泌皮质醇作为疾病原因的卵巢肿瘤是例外情况。换句话说,在这种情况下,我们认为卵巢肿瘤通过分泌皮质醇和ACTH样肽引起库欣综合征。文献报道5例。在此病例报告中,我们介绍了一例病例,其中由于库欣综合征和双侧附件肿块而接受腹腔镜手术的患者通过组织病理学检查发现了卵巢的SCLT。
    Sertoli-Leydig cell tumor (SLCT) is a rare tumor of the ovary.Cushing\'s syndrome (CS), on the other hand, is a clinical picture formed by the long-term high levels of glucocorticoids in the blood for any reason and the resulting symptoms. Exceptionally in some of cases, a tumor far from the adrenal region synthesizes adrenocortical hormones. Among such ectopic neoplasms, CS Tumors of the ovary that secrete cortisol as a cause of the disease is an exceptional case. In other words, in this case, we argue that the tumor in the ovary causes Cushing\'s syndrome by secreting cortisol and ACTH-like peptides. There are 5 cases reported in the literature. In this case report we present a case in which SCLT of the ovary was detected by histopathological examination in a patient who underwent laparoscopic surgery due to Cushing\'s syndrome and bilateral adnexal mass.
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  • 文章类型: Journal Article
    胰腺神经内分泌肿瘤患者很少发生异位库欣综合征(CS)。考虑到与CS相关的高发病率和死亡率,早期识别症状至关重要。回顾了2008年1月至2022年4月在Moffitt癌症中心看到的胰腺神经内分泌肿瘤(NENs)数据库,并确定了异位CS病例。提取了肿瘤特征的信息,临床症状和体征,疗法,和结果。确定了13例,初始诊断时年龄为16至65岁(中位数42)。13例患者中有12例出现转移性肿瘤。尽管在重新活检时描述了两种转化为低分化,但在诊断时全部分化。共有4例患者也经历了Zollinger-Ellison综合征。三名患者接受了双侧肾上腺切除术以管理不受控制的CS。从最初诊断癌症开始,中位总生存期为56个月,但从诊断CS开始仅18个月。我们的研究表明,在胰腺NEN中发生异位CS是一种高度病态的疾病,并且与侵袭性疾病相关。可以考虑双侧肾上腺切除术来控制综合征。据我们所知,这是最大的机构病例系列分泌ACTH的转移性胰腺NEN.
    Ectopic Cushing\'s syndrome (CS) occurs rarely in patients with pancreatic neuroendocrine neoplasms. Early recognition of symptoms is critical given the high morbidity and mortality associated with CS. A database of pancreatic neuroendocrine neoplasms (NENs) seen at the Moffitt Cancer Center between 1/2008 and 4/2022 was reviewed and cases of ectopic CS were identified. Information was extracted on tumor characteristics, clinical signs and symptoms, therapies, and outcomes. Thirteen cases were identified, ranging in age from 16 to 65 years at the initial time of diagnosis (median 42). Twelve of 13 patients had metastatic tumors at presentation. All were well-differentiated at diagnosis although two were described as transformed to poorly differentiated on rebiopsy. A total of 4 patients also experienced Zollinger-Ellison syndrome. Three patients underwent bilateral adrenalectomy to manage uncontrolled CS. Median overall survival of was 56 months from the time of initial cancer diagnosis but only 18 months from diagnosis of CS. Our study showed that ectopic CS is a highly morbid condition when occurring in pancreatic NENs and is associated with aggressive disease. Bilateral adrenalectomy can be considered for syndrome control. To our knowledge, this is the largest institutional case-series of ACTH-secreting metastatic pancreatic NEN.
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  • 文章类型: Case Reports
    库欣综合征被认为是肾细胞癌的副肿瘤综合征。然而,似乎缺乏令人信服的证据来证实这一观点。在英语文献中,由于肾癌异位分泌促肾上腺皮质激素而导致的库欣综合征的唯一合格文献可以追溯到1988年,它与尸检期间发现的已故患者有关。这里,我们介绍了第一例库欣综合征作为肾癌的副肿瘤特征,在手术切除肿瘤后表现出完全消退。此外,我们对这一特定主题的文献进行回顾。
    Cushing\'s syndrome has been believed to be a paraneoplastic syndrome of renal cell carcinomas. However, there appears to be a dearth of compelling evidence to substantiate this notion. The only eligible documentation of Cushing\'s syndrome due to ectopic adrenocorticotropic hormone secretion by renal cancer in English literature dates back to 1988, and it pertains to a deceased patient discovered during an autopsy. Here, we present the first case of Cushing\'s syndrome as a paraneoplastic feature of renal cancer which showed complete resolution following surgical removal of the tumor. Additionally, we conduct review of the literature on this particular subject.
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  • 文章类型: Case Reports
    垂体或肾上腺病变可引起库欣综合征,每百万人中有10-15人的发病率。越来越多的肿瘤亚型构成了称为肾细胞癌(RCC)的异质性疾病。在这里,我们描述了一例肾透明细胞癌和肾上腺腺瘤。如前所述,建议对这些患者进行常规的垂体-肾上腺轴评估.这两种疾病同时发生的主要病因极为罕见。
    Pituitary or adrenal lesions can cause Cushing syndrome, which has an incidence of 10-15 per million people. A growing variety of tumor subtypes make up the heterogeneous illness known as renal cell carcinoma (RCC). Herein, we described a case with renal clear cell carcinoma and an adrenal adenoma. As was mentioned, it is recommended that these patients routinely have their pituitary-adrenal axis evaluated. The primary etiology of these two illnesses occurring simultaneously is extremely rare.
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  • 文章类型: Case Reports
    背景:异位分泌促肾上腺皮质激素(ACTH)的神经内分泌肿瘤是罕见的疾病。分泌ACTH的胰腺神经内分泌癌患者预后不良。据报道,感染和凝血病是死亡原因。然而,分泌ACTH的神经内分泌癌的病态并发症的详细临床描述尚未报道.
    方法:一名78岁的日本妇女因全身水肿和上腹部不适而咨询医疗中心。实验室分析显示,皮质醇血症伴有ACTH分泌增加,血清皮质醇水平无昼夜变化。增强的计算机断层扫描(CT)扫描显示胰头有3厘米的肿瘤。内窥镜超声引导下细针抽吸的细胞学材料与分泌ACTH的胰腺神经内分泌癌兼容。Ki-67指数为40%。她被转移到Mie大学医院接受手术治疗。病人被诊断为尿路感染,巨细胞病毒肝炎,食管念珠菌病,肺浸润怀疑卡氏肺孢子虫肺炎,外周深静脉血栓形成,肺栓塞,和弥散性血管内凝血。多器官感染和血栓形成对抗菌和抗凝治疗反应良好。放射性同位素研究揭示了胰腺肿瘤和肝脏转移灶,而生长抑素受体闪烁显像显示阴性结果,提示原发性和转移性肿瘤分化差。入院前的CT扫描显示无转移性肝脏病变,提示胰腺肿瘤进展迅速。而不是手术,抗肿瘤化疗。患者被转移到另一家医院开始化疗。然而,4个月后她死于肿瘤进展迅速.
    结论:分泌ACTH的胰腺神经内分泌肿瘤是一种罕见的疾病,预后极差。肿瘤的临床过程和急性并发症仍未报道。在这里,我们报告了一个快速进展的ACTH分泌胰腺神经内分泌肿瘤的临床过程,该肿瘤由于多器官中的许多类型的病原体而出现感染性并发症。广泛的血栓形成,肺栓塞,和弥散性血管内凝血。
    BACKGROUND: Ectopic adrenocorticotropic hormone (ACTH)-secreting neuroendocrine tumors are rare diseases. Patients with ACTH-secreting pancreatic neuroendocrine carcinomas have a poor prognosis. Infections and coagulopathies have been reported as the cause of death. However, detailed clinical descriptions of the morbid complications of ACTH-secreting neuroendocrine carcinomas have not been reported.
    METHODS: A 78-year-old Japanese woman consulted a medical center due to systemic edema and epigastric discomfort. Laboratory analysis revealed hypercortisolemia with increased ACTH secretion without diurnal variation in serum cortisol level. An enhanced computed tomography (CT) scan revealed a 3-cm tumor in the pancreatic head. The cytological material from endoscopic ultrasound-guided fine-needle aspiration was compatible with ACTH-secreting pancreatic neuroendocrine carcinoma. The Ki-67 index was 40%. She was transferred to Mie University Hospital for surgical treatment. The patient was diagnosed with urinary tract infection, cytomegalovirus hepatitis, esophageal candidiasis, pulmonary infiltrates suspicious for Pneumocystis carinii pneumonia, peripheral deep vein thrombosis, pulmonary embolism, and disseminated intravascular coagulation. The multiple organ infections and thromboses responded well to antimicrobial and anticoagulant therapy. Radioisotope studies disclosed a pancreatic tumor and a metastatic lesion in the liver, whereas somatostatin receptor scintigraphy showed negative findings, suggesting the primary and metastatic tumors were poorly differentiated. A CT scan before admission showed no metastatic liver lesion, suggesting that the pancreatic tumor was rapidly progressing. Instead of surgery, antitumor chemotherapy was indicated. The patient was transferred to another hospital to initiate chemotherapy. However, she died four months later due to the rapidly progressive tumor.
    CONCLUSIONS: ACTH-secreting pancreatic neuroendocrine neoplasm is a rare disease with a very poor prognosis. The clinical course and acute complications of the tumor remain unreported. Here we report the clinical course of a rapidly progressive case of ACTH-secreting pancreatic neuroendocrine tumor that developed infectious complications due to many types of pathogens in multiple organs, widespread thromboses, pulmonary embolism, and disseminated intravascular coagulation.
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