中文定稿－肾上腺意外瘤

nullnullAACE/AAES Adrenal Incidentaloma Guidelines, Endocr Pract. 2009;15(Suppl 1)Adrenal Incidentaloma RevealedAdrenal Incidentaloma Revealed Normal wishbone shape most apparent on patient’s left Image: Grainger & Allison’s Incidentally discovered adrenal mass Image: eMedicine, all others Grainger & Allison’s Diagnostic RadiologynullThe incidence of adrenal incidentaloma, a term coined　in reference to the phenomenon of detecting an otherwise unsuspected adrenal mass on radiologic imaging 肾上腺意外瘤(adrenal incidentaloma)特指临床上无明确症状和体征而在腹部影像检查时意外发现的肾上腺肿物。 1982年Geelhoed提出这一命名，现已为国内、外学者所接受null不包括因为肿瘤而进行的影像学检查null对肾上腺皮质和髓质功能评价，目的在于： ① 有助于分辨肿瘤的起源、鉴别其良恶性、选择治疗策略； ②指导合理的围手术期处理，降低围手术期死亡率，如约占肾上腺意外瘤5％ 的静息性嗜铬细胞瘤，近一半患者血压正常，对未行术前准备的患者进行手术切除危险性增大；约5％ 的肾上腺意外瘤出现亚临床Cushing综合征，对这些患者在围手术期需要补充糖皮质激素； ③指导术后随访，提高远期生存率。国际内科学杂志2008,vol35 肾上腺疾病的早期诊断与治疗策略 张少玲，程桦nullnull评估肾上腺占位，需要注意三个问题 1)内分泌功能 2)恶性征象 3)既往恶性肿瘤病史null内分泌功能评估nullthe remaining incidentalomas were ganglioneuromas,　myelolipomas, or benign cysts null因此 术前需要严格的生化及影像学的评估 亚临床库欣综合征 术后的替代 嗜铬细胞瘤 术前术后的危险 醛固酮瘤 与无功能腺瘤的鉴别 1 亚临床库欣综合征的筛查1 亚临床库欣综合征的筛查R3. 所有意外瘤必须进行皮质醇增多症的筛查。 过夜小剂量地塞米松抑制试验被认为是最简便的筛查试验。null临床高度怀疑皮质醇增多症的患者，如有高血压、肥胖、糖尿病、骨质疏松，可以3项检查（唾液皮质醇，地塞米松抑制试验，尿游离皮质醇）进行筛查 (Grade C; BEL 3).nullR4. 产生皮质醇腺瘤术后可能需要6-18个月的外源性的皮质醇替代治疗(Grade C; BEL 3).nullR5. SCS没有典型的库欣综合征的表现，过夜法小剂量地塞米松抑制试验抑制后皮质醇高于5.0 Ug/dL，低ACTH和硫酸脱氢表雄酮也可以支持诊断 (Grade D; BEL4). 5.4. Screening Tests 5.4. Screening Tests 5.4.1. Late-Night Salivary Cortisol The late-night salivary cortisol test is the most recent assay to be used for screening for Cushing syndrome and is now available through most laboratories. Patients with a regular sleep pattern can collect a specimen of saliva at bedtime at home and then bring or mail the samples to the laboratory for testing. Several investigators have shown that elevated nighttime cortisol levels appear to be the earliest and most sensitive markers for Cushing syndrome (24 [EL 4], 25 [EL 4]), with sensitivity and specificity approaching90% to 95% (26 [EL 3]).null5.4.2. 过夜法1mg地塞米松抑制试验 ： 1 mg of dexamethasone at 11 pm 11点口服1mg地塞米松 determination of a fasting plasma cortisol level between 8 am and 9 am the following day. 次日8am至9am之间查空腹血浆皮质醇 Suppression of the plasma cortisol level to <1.8 µg/dL has the best negative predictive value for Cushing syndrome (5 [EL 4]). 过夜法阳性则需要进一步进行三个筛查试验（唾液皮质醇，经典法小剂量地塞米松抑制试验，尿游离皮质醇）null5.4.3. 24-Hour UFC 24小时UFC不受皮质激素结合蛋白影响，更能反映皮质醇的合成情况，升高4倍考虑诊断库欣综合征；不足3倍需要考虑假性皮质醇增多症，如慢性焦虑、忧郁、酒精成瘾、肥胖或者某些药物影响，则需要进一步进行确诊试验。AdenomaAdenomaRadiologic appearance: Usually small (<3cm), homogeneous, limited enhancement Rarely can appear heterogeneous due to focal areas of necrosis or hemorrhage Low attenuation Majority of adenomas are non-functional (51-82%) Most common functional adenoma: sub-clinical hypercortisolism Cushing inducing adenomas are usually larger than 3 cm in diameter？？ A: Contrast CT of L adrenal adenoma B: T1 C: T2null第二个异常的HPA轴的功能试验，比如2日法地塞米松抑制试验也许需要作为SCS诊断的确诊试验 (Grade B; BEL 2). nullR6. 亚临床库欣综合征的患者的肾上腺切除手术远期获益能进一步得到之前，对于高血压恶化、糖耐量异常、血脂异常和骨质疏松的患者则需要手术治疗(Grade D; BEL 4).nullR7. 推荐术前糖皮质激素的治疗和术后的HPA轴的恢复的评估(Grade C; BEL 3).2 嗜铬细胞瘤的诊断2 嗜铬细胞瘤的诊断R8. 怀疑有嗜铬细胞瘤的患者需要检查血尿去甲肾上腺素和肾上腺素及 代谢产物等(Grade A; BEL 1).nullR9. (Grade C; BEL 3). ¼的嗜铬细胞瘤患者有RET、VHL、琥珀酸脱氢酶基因突变，与遗传综合征有关，需要进行基因检测及咨询尤其是年轻患者或者有肾上腺外嗜铬细胞瘤的患者nullR10. 所有嗜铬细胞瘤均需要手术治疗(Grade C; BEL 3).nullR11. 为了术中血液动力学的稳定，嗜铬细胞瘤患者均需要在术前使用α受体阻滞剂 (Grade C; BEL 3).nullR12. 由于有10%－15%的嗜铬细胞瘤患者术后有复发，故需要长期的随访(Grade B;BEL 2).PheochromocytomaPheochromocytomaRadiologic appearance Non-contrast CT, pheo appears similar in density to surrounding soft tissue Often have fluid filled centers due to necrosis Calcification in 12% of cases Usually large, average size 5cm. T1: usually low signal intensity T2: usually have high signal intensity, though not specific to pheo Rule of 10’s 10% are malignant >10% are extra-adrenal >10% are bilateral >10% are familial >10% are not associated with HTN A: T1 B: T2 shows high signal intensity typical of Pheochromocytomanull嗜铬细胞瘤的核素检查 MIBG 奥曲肽显像 PET，多巴胺 3 原发性醛固酮增多症诊断3 原发性醛固酮增多症诊断R14. 肾上腺意外瘤原醛的确诊是盐负荷下24小时尿醛固酮不被抑制 (Grade C; BEL 3).nullnullR15. 原醛的分型需对所有患者进行高分辨CT和在四十岁以上的患者进行AVS (Grade C; BEL 3).null10-15分钟后静脉增强剂延迟显像，良性的肾上腺病变通常增强至80-90HU,超过50％造影剂被清除，转移瘤、癌或者嗜铬细胞瘤则不是。 (8 [EL 2]).null嗜铬细胞瘤经常增强至100HU,与腺瘤鉴别 在CT 平扫时，良性病变的CT值可能达到20-40HU,在脂肪含量低的腺瘤中被发现比较多，而如前造影剂清除50%则可以诊断为腺瘤，nullR16. In patients with primary aldosteronism and a unilateral source of aldosterone excess, laparoscopic total adrenalectomy is the treatment of choice because it yields excellent outcomes with low associated morbidity relative to open approaches 相对于开放手术，腹腔镜肾上腺切除手术死亡率低，应该作为单侧醛固酮增多的原醛的治疗首选 (Grade C; BEL 3).nullR17. IHA特醛不是手术适应症，而是予以选择性或者非选择性盐皮质激素的受体阻滞剂 (Grade A; BEL 1).null4 性激素肿瘤4 性激素肿瘤 除非有很明显的雄激素增多的表现，性激素检测不是必须的良恶性的评估良恶性的评估R18. 有相关的影像学特征和直径大于4cm的肾上腺结节为肾上腺癌的机会增加，需要予以切除(Grade C; BEL 3).nullnonfunctioning adrenal tumors ≥4 cm should be considered for surgical resection. 即使是无功能肾上腺肿瘤，直径≥4 cm也是手术的指征在影像学检查的各项肿瘤特征中，肿瘤的大小被认为是最有鉴别意义的，因而也是决定手术与否的最重要指标。很多文献报道意外瘤越大，其恶性的可能性也就越大。4 cm以上的肿瘤中恶性和良性的比例为8：l 。有人以肿瘤大于5 cm作为判断肿瘤恶性的指标，发现其敏感性为93％ ，特异性为64％。Bertherat J，Mosnier-Pudar H，Bertagna X．Adrenal incidentalomas[J]．Curt Opin in Oncol，2002，14：58—63．Radiologic Evaluation Size ConsiderationsRadiologic Evaluation Size Considerations几乎所有的< 4 cm 病变是良性的 If mass 60% are benign adenomas If mass 4.1-6cm 6% are adrenal cortical CA < 15% are benign adenomas If mass > 6cm 25% are adrenal cortical CA 有限的随访发现5-25% 的无功能结节直径增大，但是有3-4%的结节却缩小 小于3cm的病变有内分泌功能的可能性小（？？？） 10年内无功能结节转化为有功能结节的概率<20% Radiologic Evaluation Appearance of Benign LesionsRadiologic Evaluation Appearance of Benign LesionsSmooth borders Low attenuation on CT Benign adenomas generally contain low attenuation intracellular lipid Hounsefield units (HU): standardized attenuation values abbreviated as HU) Criteria: Lesion <10 HU on non-contrast CT highly specific for benign adenoma, nearly 100% Criteria: 1 h post-contrast CT with attenuation < 30 HU can be used to identify adrenal masses as adenomas MRI: no more effective than CT T2 signal intensity similar to that of liver Signal drop on chemical-shift imaging Intracellular lipid, as in adenomas, may show a loss of signal on the out-of-phase images compared with the in-phase images双回波同去相位磁共振成像nullR19. 术前必须排除嗜铬细胞瘤 (Grade C; BEL 3).nullR20. 需要排除皮质醇增多症，术前进行糖皮质激素替代 (Grade D; BEL 4)nullR21. 怀疑肾上腺皮质癌患者需要进行开放性手术 (Grade C; BEL 3).nullR22. 有恶性肿瘤病史的患者需要怀疑肾上腺转移癌，这不是符合肾上腺意外瘤的 （Grade C;BEL 3).nullR23. 极少数情况下需要行活检明确病理治疗的 (Grade D; BEL 4).nullR24. 穿刺活检前也是需要排除嗜铬细胞瘤 (Grade C; BEL 3).nullR25.、 双侧肾上腺转移癌有时出现肾上腺皮质功能不足 (Grade D; BEL 4).nullR26. 肾上腺转移瘤极少情况下孤立肾上腺转移的病变是进行肾上腺切除的指征 (Grade C; BEL 3).null原发灶多来源于肺、乳腺、结肠和肾肿瘤及黑色素瘤，其大小介于良性肿瘤和原发恶性肿瘤之间，多为双侧 有其他部位恶性肿瘤的患者如发现有肾上腺意外瘤，则该意外瘤的恶性可能性高达38％ Lim PO，Rodgers P，Cardale K，et a1．Potentially high preva·lenee of primary aldosteronismina primary·care population[J]．Lancet，1999，353：40—47null如可疑病例可以做B-US及CT引导下作细针穿刺，结合临床内分泌检查及影像学表现，其准确性达75％ ～85％⋯。 然而，细针穿刺有利于鉴别良性肿瘤和转移瘤，却难以鉴别皮质腺瘤及皮质癌 ’ 。 有些学者认为B-us及CT引导下作细针穿刺只用于那些有肾上腺以外恶性肿瘤而其肾上腺意外瘤的良恶性又不明确的患者 Linos DA，Stylovmlos N。How accurate is computed tomography in predietlng the size of adrenal tumors：A retrospective study[J]．Arch Esp Uml，1998，51(3)：227—240Adrenal CarcinomaAdrenal CarcinomaRare malignancy with poor prognosis 50% are hormonally active, usually cortisol Account for 5-10% of Cushing’s syndrome Androgen secreting tumors are nearly always malignant, rarely adenoma Radiologic appearance Most >6cm at presentation Usually heterogeneous with areas of necrosis, hemorrhage, and calcification Contrast CT showing large partly calcified, heterogeneously enhancing Left adrenal cancer MyelolipomaMyelolipomaRare, benign, asx, non-functioning neoplasm composed of fat and bone marrow tissue Radiologic appearance A: US showing a hyperechoic mass lying above the right kidney B: CT of same mass, composed mostly of fat C: T1 showing similar density w/peri-renal fat D: showing suppression of fat signal from within massAdrenal CystAdrenal CystUncommon, usually unilateral structure, more common in women Radiologic appearance A: CT B: T1 C: T2 shows uniform high signal intensity of fluid-filled cyst肾上腺结核2003年6月24日*肾上腺结核肾上腺转移瘤2003年6月24日*肾上腺转移瘤Contrast-enhanced CT scan. Right adrenal metastasis from esophageal carcinoma. Liver metastases (arrowheads).nullnull　腹主动脉瘤2003年6月24日*　腹主动脉瘤CT of large abdominal aortic aneurysm with calcified wall.原发腹膜后恶性肿瘤 　　（腹膜后恶性间叶组织肿瘤，）2003年6月24日*原发腹膜后恶性肿瘤 　　（腹膜后恶性间叶组织肿瘤，）abnullHistoplasmosis组织胞浆菌病Histoplasmosis组织胞浆菌病nullR2. (Grade C; BEL 3). 未达到手术标准的意外瘤在将来3-6个月随之1-2年每年重新影像学评估。 内分泌评估在随诊五年内每年一次null随诊患者中出现最多的内分泌活性最常见SCS 增大1cm或者出现内分泌功能则考虑手术 目前缺乏超过5年后仍然稳定、无功能的肾上腺结节的随访的循证医学证据nullnullnullnull谢谢您的倾听！