肾上腺偶发瘤是由于怀疑肾上腺疾病以外的原因而在成像中检测到的肾上腺肿块。在大多数情况下,肾上腺偶发瘤是无功能的肾上腺皮质腺瘤,但也可能需要治疗性干预,包括肾上腺皮质癌。嗜铬细胞瘤,产生激素的腺瘤,或转移。这里,我们提供了第一个国际的修订版,关于偶发瘤的跨学科指南。我们遵循了建议评估的分级,关于对偶发瘤治疗至关重要的4个预定义临床问题的开发和评估系统和更新的系统评价:(1)如何评估恶性肿瘤的风险?;(2)如何定义和管理轻度自主皮质醇分泌?(3)谁应该接受手术治疗,应该如何进行?(4)如果肾上腺偶发瘤未通过手术切除,应进行什么随访?选定的建议:(1)每个肾上腺成像都需要。最近的进展现在可以区分风险类别:未增强CT上Hounsfield单位(HU)≤10的同质病变是良性的,不需要任何额外的成像,而与大小无关。所有其他患者都应该在多学科专家会议上讨论,但只有>4cm且不均匀或HU>20的病变才有足够高的恶性肿瘤风险,因此手术将是常规的治疗选择.(2)每个患者都需要进行彻底的临床和内分泌检查,以排除激素过量,包括测量血浆或尿中的肾上腺素和1mg过夜地塞米松抑制试验(应用血清皮质醇≤50nmol/L的截止值[≤1.8µg/dL])。最近的研究提供了证据,表明大多数患者没有明显的库欣综合征的临床症状,但在地塞米松>50nmol/L(>1.8µg/dL)后血清皮质醇水平增加了发病率和死亡率的风险。对于这种情况,我们提出术语“轻度自主皮质醇分泌”(MACS)。(3)所有患有MACS的患者应筛查潜在的皮质醇相关合并症,这些合并症可能与皮质醇有关(例如,高血压和2型糖尿病),以确保这些得到适当的治疗。(4)对于合并有相关疾病的MACS患者,应考虑采用个体化的手术治疗方法。(5)手术干预的适当性应该以恶性肿瘤的可能性为指导,激素过量的存在和程度,年龄,一般健康,患者偏好。我们为放射学发现可疑恶性肿瘤的肾上腺肿块应考虑哪种手术方法提供指导。(6)无症状的患者通常不需要手术,无功能的单侧肾上腺肿块和影像学检查的明显良性特征。此外,我们为非手术患者的随访提供建议,双侧偶发瘤患者的治疗,对于肾上腺外恶性肿瘤和肾上腺肿块的患者,以及年轻和老年肾上腺偶发瘤患者。最后,我们提出了未来的10个重要研究问题。
Adrenal incidentalomas are adrenal masses detected on imaging performed for reasons other than suspected adrenal disease. In most cases, adrenal incidentalomas are nonfunctioning adrenocortical adenomas but may also require therapeutic intervention including that for adrenocortical carcinoma, pheochromocytoma, hormone-producing adenoma, or metastases. Here, we provide a revision of the first international, interdisciplinary
guidelines on incidentalomas. We followed the Grading of Recommendations Assessment, Development and Evaluation system and updated systematic reviews on 4 predefined clinical questions crucial for the management of incidentalomas: (1) How to assess risk of malignancy?; (2) How to define and manage mild autonomous cortisol secretion?; (3) Who should have surgical treatment and how should it be performed?; and (4) What follow-up is indicated if the adrenal incidentaloma is not surgically removed? Selected Recommendations: (1) Each adrenal mass requires dedicated adrenal imaging. Recent advances now allow discrimination between risk categories: Homogeneous lesions with Hounsfield unit (HU) ≤ 10 on unenhanced CT are benign and do not require any additional imaging independent of size. All other patients should be discussed in a multidisciplinary expert meeting, but only lesions >4 cm that are inhomogeneous or have HU >20 have sufficiently high risk of malignancy that surgery will be the usual management of choice. (2) Every patient needs a thorough clinical and endocrine work-up to exclude hormone excess including the measurement of plasma or urinary metanephrines and a 1-mg overnight dexamethasone suppression test (applying a cutoff value of serum cortisol ≤50 nmol/L [≤1.8 µg/dL]). Recent studies have provided evidence that most patients without clinical signs of overt Cushing\'s syndrome but serum cortisol levels post dexamethasone >50 nmol/L (>1.8 µg/dL) harbor increased risk of morbidity and mortality. For this condition, we propose the term \"mild autonomous cortisol secretion\" (MACS). (3) All patients with MACS should be screened for potential cortisol-related comorbidities that are potentially attributably to cortisol (eg, hypertension and type 2 diabetes mellitus), to ensure these are appropriately treated. (4) In patients with MACS who also have relevant comorbidities surgical treatment should be considered in an individualized approach. (5) The appropriateness of surgical intervention should be guided by the likelihood of malignancy, the presence and degree of hormone excess, age, general health, and patient preference. We provide guidance on which surgical approach should be considered for adrenal masses with radiological findings suspicious of malignancy. (6) Surgery is not usually indicated in patients with an asymptomatic, nonfunctioning unilateral adrenal mass and obvious benign features on imaging studies. Furthermore, we offer recommendations for the follow-up of nonoperated patients, management of patients with bilateral incidentalomas, for patients with extra-adrenal malignancy and adrenal masses, and for young and elderly patients with adrenal incidentalomas. Finally, we suggest 10 important research questions for the future.