adrenal mass

肾上腺肿块
  • 文章类型: Journal Article
    背景:关于巨大嗜铬细胞瘤(PHEO)的数据,根据尺寸≥10厘米定义,是稀缺的。
    目的:比较演示文稿,管理,巨大和非巨大PHEO患者的结局。
    方法:回顾性队列研究,2000-2023年。
    方法:转诊中心。
    方法:连续患有巨大PHEO的患者和随机选择的非巨大PHEO(参考)的患者,比例为1:6。
    结果:围手术期并发症,转移,死亡率。
    结果:在828例PHEO患者中,31(3.7%)具有巨大的PHEO(中位尺寸为12厘米,IQR10.0-13.5)。与参照物(n=186,中位数大小4厘米,IQR,2.9-5.0),巨大PHEO患者的儿茶酚胺过量症状较多(中位数为2vs1,P=.04),严重儿茶酚胺过量的患病率较高(76%vs30%,P<.0001)。94%的巨大PHEO患者和100%的参考患者进行了肾上腺切除术。除了术前α-肾上腺素能阻滞(89%),14例(7%)患者使用了甲酪氨酸,主要是巨大的PHEO患者(26%vs3%,P<.0001)。巨大PHEO患者围手术期并发症发生率较高(31%vs10%,P=.004)。在3年的中位随访期间,巨大PHEO患者的转移发生率较高(45%vs4%,P<.0001)。巨大PHEO患者的疾病特异性死亡率为7%,参考人群为0%(P=.02)。
    结论:与参照物相比,巨大PHEO患者更有症状,有较高程度的儿茶酚胺过量,围手术期并发症发生率较高。几乎一半的巨大PHEO患者发生了转移,保证密切跟进。
    BACKGROUND: Data on giant pheochromocytomas (PHEO), defined based on size ≥ 10 cm, are scarce.
    OBJECTIVE: to compare presentation, management, and outcomes of patients with giant vs non-giant PHEOs.
    METHODS: retrospective cohort study, 2000-2023.
    METHODS: referral center.
    METHODS: consecutive patients with giant PHEO and randomly chosen patients with non-giant PHEO (referents) at a 1:6 ratio.
    RESULTS: perioperative complications, metastases, mortality.
    RESULTS: Of 828 patients with PHEO, 31 (3.7%) had giant PHEO (median size 12 cm, IQR 10.0-13.5). In comparison to referents (n=186, median size 4 cm, IQR, 2.9-5.0), patients with giant PHEO had more symptoms of catecholamine excess (median of 2 vs 1, P=.04) and presented with a higher prevalence of severe catecholamine excess (76% vs 30%, P<.0001).Adrenalectomy was performed in 94% of patients with giant PHEOs and 100% referents. In addition to preoperative alpha-adrenergic blockade (89%), metyrosine was used in 14 (7%) patients, mostly in patients with giant PHEO (26% vs 3%, P<.0001). Patients with giant PHEO had a higher perioperative complication rate (31% vs 10%, P=.004).During a median follow-up of 3 years, metastases developed at a higher rate in patients with giant PHEOs (45% vs 4% in referents, P<.0001). Disease-specific mortality was 7% in patients with giant PHEOs and 0% in referents (P=.02).
    CONCLUSIONS: Patients with giant PHEO as compared to referents were more symptomatic, had a higher degree of catecholamine excess, and had a higher rate of perioperative complications. Almost half of patients with giant PHEO developed metastases, warranting a close follow-up.
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  • 文章类型: Journal Article
    肾上腺病变的临床表现各不相同,初级保健提供者应了解常见和严重的肾上腺疾病。所有肾上腺肿块都需要评估恶性肿瘤,无论是原发性还是转移性,和所有的群众需要评估不适当的荷尔蒙分泌。如果肾上腺功能不全,必须确定皮质醇不足的病因并进行适当治疗,以预防危及生命的并发症.
    Adrenal pathologies have variable clinical presentations and primary care providers should be aware of common and serious adrenal disorders. All adrenal masses require evaluation for malignancy, whether primary or metastatic, and all masses require evaluation for inappropriate hormonal secretion. In the event of adrenal insufficiency, the etiology of cortisol inadequacy must be identified and appropriately treated to prevent life-threatening complications.
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  • 文章类型: Journal Article
    这项研究的目的是评估最常用的放射学评估的诊断效能,以对比增强的腹部计算机断层扫描与肾上腺方案,原发性肾上腺肿块的基本功能评估和手术结果。
    我们回顾性分析了2017年8月至2023年9月收治的108名患者的机构记录,这些患者在经过彻底评估和稳定后接受了肾上腺肿块的手术干预。
    腰痛是44例(40.74%)患者中最常见的症状。36例(33.33%)患者发现无功能性肾上腺腺瘤。嗜铬细胞瘤是24例(22.22%)患者中最常见的功能性肾上腺肿块。16例(14.81%)患者的CECT腹部提示恶性肿瘤特征。在最终的组织病理学报告中,有26例(24.07%)患者患有嗜铬细胞瘤,有12例(11.11%)患者患有肾上腺皮质癌。CECT腹部的敏感度为75%,特异性为95.6%。血浆游离去甲肾上腺素和去甲肾上腺素分析显示灵敏度为90%,特异性92.86%,醛固酮与肾素比率的敏感性为92%,100%的特异性。在我们的研究中,低剂量地塞米松抑制试验和血浆脱氢表雄酮的敏感性和特异性为100%。80例(74.07%)患者行腹腔镜肾上腺切除术,20例(18.52%)患者行开放性肾上腺切除术。8例患者(7.41%)从腹腔镜转为开腹。腹腔镜入路的平均手术时间明显较少,失血少,住院次数少,术后并发症少。
    放射分析和功能分析显示出良好的灵敏度和高特异性。腹腔镜手术入路的优点是手术时间短,住院次数少,术后并发症少。
    UNASSIGNED: Objective of this study was to assess the diagnostic efficacy of the most commonly used radiological evaluation in form of contrast enhanced computed tomography of abdomen with adrenal protocol, basic functional evaluation and surgical outcomes of primary adrenal masses.
    UNASSIGNED: We have retrospectively analysed the institutional records of 108, patients admitted from August 2017 to September 2023, who had underwent surgical intervention for their adrenal mass after thorough evaluation and stabilization.
    UNASSIGNED: Flank pain was the most common symptoms in 44 (40.74%) patients. Non-functional adrenal adenoma was found in 36 (33.33%) patients. Pheochromocytoma was the most the common functional adrenal mass found in 24 (22.22%) patients. CECT abdomen had suggested features of malignancy in 16(14.81%) patients. In final histopathological report 26(24.07%) patients had pheochromocytoma and 12 (11.11%) patients had adrenocortical carcinoma. CECT abdomen had sensitivity of 75%, specificity of 95.6%. Plasma free metanephrines and normetanephrine analysis had shown sensitivity of 90%, specificity of 92.86%, aldosterone to renin ratio had sensitivity of 92%, specificity of 100%. Sensitivity and specificity of the low-dose dexamethasone suppression test and plasma dehydroepiandrosterone was 100% in our study. Eighty patients (74.07%) were operated with laparoscopic adrenalectomy, 20 (18.52%) patients with open adrenalectomy. Eight patients (7.41%) were converted from laparoscopic to open. Laparoscopic approach had significantly lesser mean operating time, lesser blood loss, lesser hospitalization and lesser post-operative complications.
    UNASSIGNED: Radiological analysis and functional analysis has shown good sensitivity and high specificity. Laparoscopic approach has advantage of lesser operative time, lesser hospitalization and lesser post-operative complications.
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  • 文章类型: Case Reports
    此病例报告描述了巨大的左肾上腺肿块(27x26×27cm,9.370公斤)在69岁的女性中。我们回顾了术前准备的多学科决策过程,栓塞,切除可行性,以及减轻术中并发症的策略。这是迄今为止报道的最大的肾上腺腺瘤切除。该案例强调了细致的手术计划和执行在管理罕见和具有挑战性的病例中的重要性。对患者预后有重大影响。
    This case report describes the surgical management of a giant left adrenal mass (27 x 26 × 27 cm, 9.370 kg) in a 69-year-old female. We review the multidisciplinary decision-making process for pre-operative preparation, embolization, resection feasibility, and strategies to mitigate intra-operative complications. This is the largest adrenal adenoma removal reported to date. The case highlights the importance of meticulous surgical planning and execution in managing rare and challenging cases, with significant implications for patient outcomes.
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  • 文章类型: Case Reports
    嗜铬细胞瘤很少出现原因不明的低钾血症,虽然文献中有一些病例报道。其背后的机制可能是β-2-肾上腺素受体过度激活和胰岛素抵抗促进的细胞钾摄取增加。我们介绍了一名68岁的高血压女性患者的情况,该患者在血管CT上发现了单侧肾上腺肿块,并伴有典型的肾上腺素能过度刺激(高血压危象,头痛,和出汗)与多次心律失常发作有关,但血浆和尿儿茶酚胺水平正常。在激素分泌过多和停止抗醛固酮药物治疗的过程中,患者出现耐药性低钾血症.由于无法纠正的低钾血症,我们无法对原发性醛固酮增多症进行激素检查,并将患者转诊为腹腔镜肾上腺切除术.组织学诊断为左嗜铬细胞瘤。术后,患者出现反弹型高钾血症.在单侧肾上腺肿块和低钾血症的患者中,除了原发性醛固酮增多症和促肾上腺皮质激素依赖性皮质醇增多症,手术前,临床医生也应排除可能的嗜铬细胞瘤。
    Pheochromocytoma rarely presents with unexplained hypokalaemia, although there are some case reports in the literature. The mechanism behind this could be the increased cellular potassium uptake promoted by beta-2-adrenoreceptor hyperactivation and insulin resistance. We present the case of a 68-year-old hypertensive female patient with a unilateral adrenal mass discovered on angio-CT and typical signs of adrenergic hyperstimulation (hypertensive crisis, headache, and sweating) associated with multiple arrhythmic episodes but with normal plasma and urinary catecholamine levels. During the work-up for hormonal hypersecretion and the cessation of anti-aldosterone medication, the patient presented resistant hypokalaemia. Due to uncorrectable hypokalaemia, we were unable to perform hormonal investigations for primary hyperaldosteronism and referred the patient for laparoscopic adrenalectomy. The histological diagnosis revealed left pheochromocytoma. Postoperatively, the patient experienced rebound hyperkalaemia. In a patient with a unilateral adrenal mass and hypokalaemia, besides primary hyperaldosteronism and adrenocorticotropic hormone-independent hypercortisolism, a possible pheochromocytoma should be ruled out as well by the clinician before surgery.
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  • 文章类型: Journal Article
    背景:根据地塞米松后皮质醇>1.8mcg/dL诊断轻度自主皮质醇分泌(MACS)。在MACS中,关于类固醇昼夜节律分泌和类固醇代谢组的证据很少。
    目的:研究MACS患者24小时(h)尿类固醇代谢组的特征,并确定MACS患者与参考受试者的尿类固醇谱和皮质醇浓度的昼夜节律差异。
    方法:横断面研究,2018-2023年。
    方法:转诊中心。
    方法:患有MACS和年龄的患者,sex-,BMI-,和更年期状态匹配的参考对象。
    方法:在24小时内收集尿液作为单独的白天和夜间收集。高分辨率质谱测定用于测量25种类固醇。每2小时对游离和总皮质醇进行血清测量,将患者和参考受试者分组。
    结果:类固醇,sums,和比率。
    结果:MACS患者(n=72)的mcg/24h中位数雄激素较低(2084vs3283,P<0.001),较高的糖皮质激素(15754vs12936,P<0.001),和更高的糖皮质激素/雄激素比率(8.7vs3.9,P<0.001),与引用主题相比。与参考受试者相比,患者的类固醇日/夜比率也较低,反映了MACS中相对夜间类固醇产量较高。在12名MACS患者和10名参考受试者的亚组中,总皮质醇和游离皮质醇的24小时曲线下面积相似.然而,晚上平均总数(5.3vs4.0mcg/dL,P=0.056)和游离(0.2vs0.1mcg/dL,P=0.035)患者的皮质醇高于参考受试者。
    结论:MACS患者表现出异常的尿类固醇代谢组,糖皮质激素与雄激素的比例很高,和更高的夜间类固醇产量。
    BACKGROUND: Mild autonomous cortisol secretion (MACS) is diagnosed based on post-dexamethasone cortisol>1.8 mcg/dL. Scarce evidence exists on steroid circadian secretion and steroid metabolome in MACS.
    OBJECTIVE: To characterize 24-hour (h) urine steroid metabolome in patients with MACS and determine circadian differences in urine steroid profiling and cortisol concentrations in patients with MACS versus referent subjects.
    METHODS: Cross-sectional study, 2018-2023.
    METHODS: Referral center.
    METHODS: Patients with MACS and age-, sex-, BMI-, and menopausal status-matched referent subjects.
    METHODS: Urine was collected over 24h period as separate day- and night-time collections. High-resolution mass spectrometry assay was used to measure 25 steroids. A subgroup of patients and referent subjects were admitted for every 2h serum measurements of free and total cortisol.
    RESULTS: Steroids, sums, and ratios.
    RESULTS: Patients with MACS (n=72) had lower mcg/24h median androgens (2084 vs 3283, P<0.001), higher glucocorticoids (15754 vs 12936, P<0.001), and higher glucocorticoid/androgen ratio (8.7 vs 3.9, P<0.001), compared to referent subjects. Patients also had lower steroid day/night ratios compared to referent subjects, reflecting a higher relative nocturnal steroid production in MACS. In a subgroup of 12 patients with MACS and 10 referent subjects, the 24-hour area under the curves for total and free cortisol were similar. However, evening mean total (5.3 vs 4.0 mcg/dL, P=0.056) and free (0.2 vs 0.1 mcg/dL, P=0.035) cortisol was higher in patients vs referent subjects.
    CONCLUSIONS: Patients with MACS demonstrate an abnormal urine steroid metabolome, with a high glucocorticoid to androgen ratio, and a higher nocturnal steroid production.
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  • 文章类型: Journal Article
    目的:评估临床和影像学特征是否可以预测激素亚型,增长,和肾上腺切除术治疗偶发性肾上腺皮质腺瘤(ACA)。
    方法:单中心队列研究。
    方法:2000年至2016年连续诊断为偶发ACA的成年患者。
    结果:在1516例附带ACA患者中(中位年龄59岁,62%的女性),699(46%)患有无功能腺瘤(NFA),482例(31%)有轻度自主皮质醇分泌(MACS),62(4%)患有原发性醛固酮增多症(PA),39人(3%)患有库欣综合征(CS),18(1%)患有PA和MACS(PA-MACS),和226(15%)有不完整的检查。年龄,性别,肿瘤大小,和肿瘤侧向性,但不是未增强的计算机断层扫描Hounsfield单位(HU),与荷尔蒙亚型有关。在多变量分析中,≥1cm的增长与年龄较小相关(每5年增长的比值比,OR=0.8,P=0.0047)和更长的影像学随访(OR=1.2/年,P<.0001)。355例(23%)患者进行了肾上腺切除术,包括38%的MACS和15%的NFA。NFA和MACS的肾上腺切除术在年轻患者中更为常见(OR=0.79/5年增加,P=0.002),较大的初始肿瘤大小(OR=2.3每增加1厘米,P<.0001),生长≥1cm(OR=15.3,P<0.0001),和较高的后地塞米松皮质醇(OR=6.6>5vs<1.8mcg/dL,P=0.002)。
    结论:年龄,性别,肿瘤大小,和侧向性与ACA激素亚型相关,可指导诊断和治疗.随着年龄的增长和随访时间的延长,肿瘤的生长更为常见。未增强的HU不能预测激素亚型或生长。MACS和NFA的肾上腺切除术主要在肿瘤较大的年轻患者中进行,增长,地塞米松后皮质醇升高。
    OBJECTIVE: The aim of this study is to assess whether clinical and imaging characteristics are associated with the hormonal subtype, growth, and adrenalectomy for incidental adrenal cortical adenomas (ACAs).
    METHODS: This is a single-center cohort study.
    METHODS: Consecutive adult patients with incidental ACA were diagnosed between 2000 and 2016.
    RESULTS: Of the 1516 patients with incidental ACA (median age 59 years, 62% women), 699 (46%) had nonfunctioning adenomas (NFAs), 482 (31%) had mild autonomous cortisol secretion (MACS), 62 (4%) had primary aldosteronism (PA), 39 (3%) had Cushing syndrome, 18 (1%) had PA and MACS, and 226 (15%) had incomplete work-up. Age, sex, tumor size, and tumor laterality, but not unenhanced computed tomography Hounsfield units (HU), were associated with hormonal subtypes. In a multivariable analysis, ≥1 cm growth was associated with younger age (odds ratio [OR] = 0.8 per 5-year increase, P = .0047) and longer imaging follow-up (OR = 1.2 per year, P < .0001). Adrenalectomy was performed in 355 (23%) patients, including 38% of MACS and 15% of NFA. Adrenalectomy for NFA and MACS was more common in younger patients (OR = 0.79 per 5-year increase, P = .002), larger initial tumor size (OR = 2.3 per 1 cm increase, P < .0001), ≥1 cm growth (OR = 15.3, P < .0001), and higher postdexamethasone cortisol (OR = 6.6 for >5 vs <1.8 μg/dL, P = .002).
    CONCLUSIONS: Age, sex, tumor size, and laterality were associated with ACA hormonal subtype and can guide diagnosis and management. Tumor growth was more common with younger age and longer follow-up. Unenhanced HU did not predict hormonal subtype or growth. Adrenalectomy for MACS and NFA was mainly performed in younger patients with larger tumor size, growth, and elevated postdexamethasone cortisol.
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  • 文章类型: Case Reports
    我们报道一例22岁女性脚踏板水肿,低钾血症,和高血压。怀疑醛固酮增多症,进行了进一步的工作,仅在1毫克地塞米松抑制试验后显示血清促肾上腺皮质激素(ACTH)低和皮质醇水平不适当正常,提示原发性皮质醇增多症。胸部CT,腹部,骨盆显示左侧肾上腺肿块.根据临床表现和生化异常,我们预计这个肿瘤会分泌醛固酮,但我们患者的血清醛固酮和肾素水平均正常。最终手术切除证实了最初的恶性肿瘤怀疑,因为它被发现是肾上腺皮质癌。此病例突显了这种罕见但侵袭性内分泌肿瘤的异常表现及其及时诊断和治疗的重要性。
    We report a case of a 22-year-old female with pedal edema, hypokalemia, and hypertension. On suspicion of hyperaldosteronism, further workup was pursued, which only revealed a low serum adrenocorticotropic hormone (ACTH) and an inappropriately normal cortisol level after a 1-mg dexamethasone suppression test, suggestive of primary hypercortisolism. CT of the chest, abdomen, and pelvis revealed a left adrenal mass. Based on the clinical findings and biochemical abnormalities, we were expecting this tumor to be aldosterone-secreting, but both serum aldosterone and renin levels were normal in our patient. Eventual surgical resection confirmed initial suspicions of malignancy, as it was found to be adrenal cortical carcinoma. This case highlights the unusual presentation of this rare but aggressive endocrinologic neoplasm and the importance of its prompt diagnosis and treatment.
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  • 文章类型: Case Reports
    胃憩室是一种罕见的现象,通常无症状且偶然遇到。由于胃憩室和左肾上腺之间的相对接近,它们可能在计算机断层扫描(CT)上模仿肾上腺肿块。出于这个原因,胃憩室的首选诊断方法是上消化道系列检查或内窥镜直接观察。本报告描述了一种罕见的情况,即模仿肾上腺脓肿的胃憩室,感染明显扩散到肺的左下叶。
    Gastric diverticula are a rare phenomenon that is typically asymptomatic and encountered incidentally. Due to the relative proximity between the gastric diverticula and the left adrenal gland, they may mimic adrenal masses on computed tomography (CT). For this reason, the preferred diagnostic methods for gastric diverticula are upper gastrointestinal series or direct visualization on endoscopy. The present report describes an unusual case of a gastric diverticulum mimicking an abscess of the adrenal gland with the apparent spread of infection to the left lower lobe of the lung.
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  • 文章类型: Case Reports
    绝经后女性的雄激素过多症可能来自卵巢或肾上腺来源,并可能构成具有挑战性的诊断困境。我们介绍了一名66岁的绝经后高雄激素血症伴男性化的女性,肾上腺偶发瘤,同时发现了两个极其罕见的卵巢肿瘤,包括双侧Leydig细胞肿瘤和Brenner肿瘤。实验室测试显示睾丸激素和雄烯二酮升高,硫酸脱氢表雄酮正常(DHEAS)。对1mg过夜地塞米松抑制试验的反应表明睾酮持续升高和雄烯二酮的不完全抑制。计算机断层扫描(CT)扫描显示左侧肾上腺结节和卵巢外观不明显。盆腔超声没有显示右侧卵巢有卵巢肿瘤,左侧卵巢未见。肾上腺和卵巢静脉取样表明卵巢是睾酮的来源。鉴于卵巢静脉采样结果,内分泌学和妇科肿瘤学之间的多学科讨论得出结论,双侧输卵管卵巢切除术(BSO)是诊断和治疗的下一个最佳步骤.进行腹腔镜BSO检查。组织病理学显示双侧Leydig细胞肿瘤和左卵巢Brenner肿瘤。术后1年随访,脱发改善,睾酮水平恢复正常。此案例强调了诊断途径和跨学科合作在管理绝经后女性高雄激素血症的罕见临床情景中的重要性。就像我们的情况一样,外科医生可能对切除外观正常的卵巢犹豫不决。虽然在这种情况下存在的三种肿瘤类型来自不同的组织并表现出不同的组织学特征,这种独特的三合会的存在促使人们考虑潜在的统一致病机制。
    Hyperandrogenism in postmenopausal females may arise from either ovarian or adrenal sources and can pose a challenging diagnostic dilemma. We present the case of a 66-year-old female with postmenopausal hyperandrogenism with virilization, adrenal incidentaloma, and concurrent finding of two extremely rare ovarian tumors, including bilateral Leydig cell tumor and Brenner tumor. Laboratory tests showed elevated testosterone and androstenedione and normal dehydroepiandrosterone sulfate (DHEAS). Response to 1 mg overnight dexamethasone suppression test demonstrated persistently elevated testosterone and incomplete suppression of androstenedione. Computed tomography (CT) scan showed a left adrenal nodule and an unremarkable appearance of the ovaries. The pelvic ultrasound did not show an ovarian tumor on the right ovary, and the left ovary was not seen. Adrenal and ovarian vein sampling suggested the ovaries as the source of the testosterone. Given the ovarian vein sampling results, a multidisciplinary discussion between endocrinology and gynecologic oncology concluded that bilateral salpingo-oophorectomy (BSO) was the next best step for diagnosis and management. Laparoscopic BSO was performed. Histopathology showed bilateral Leydig cell tumors and a left ovarian Brenner tumor. At one-year postoperative follow-up, alopecia improved, and testosterone level normalized. This case highlights the importance of diagnostic pathways and interdisciplinary collaboration in managing rare clinical scenarios of hyperandrogenism in postmenopausal females. As in our case, surgeons may be hesitant to remove normal-appearing ovaries. While the three presented tumor types in this case arise from distinct tissues and exhibit different histological characteristics, the presence of such a unique triad prompts consideration of potential unifying pathogenic mechanisms.
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