肾上腺意外瘤指南课件.ppt

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1、肾上腺意外瘤指南第1页,此课件共61页哦OutlinevvDefinitionvvPrevalencevvAnatomy and Physiology ReviewvvDiagnostic WorkupsvvConclusions第2页,此课件共61页哦Definitionvv“Mass lesion greater than 1 cm in diameter discovered“accidentally”during a radiographic examination performed for indications other than an evaluation for adren

2、al disease.”Management of the clinically inapparent adrenal mass(incidentaloma).NIH State-of-the-Science Conference Statement Feb 4-6,2002.第3页,此课件共61页哦PrevalencevvAutopsies:87,065 cases:6%with adrenal adenomasvvAbdominal CT(61,054 CT scans reviewed):4%with adrenal adenomasvvNow approaches the 8.7%in

3、cidence reported in autopsy series 第4页,此课件共61页哦Incidence Increases with AgeEndocrine and Metabolism Clinics of North America.2000;29(1):159-185第5页,此课件共61页哦Three Main QuestionsvvIs the adrenal mass hormonally active?vvIs the mass benign or malignant?vvDoes the patient have a history of a previous mal

4、ignant lesion?Is it metastatic?第6页,此课件共61页哦Anatomyhttp:/shutterbug.ucsc.edu/sealion/view_photo.php?set_albumName=album265&id=Adrenal第7页,此课件共61页哦Anatomyhttp:/shutterbug.ucsc.edu/sealion/view_photo.php?set_albumName=album265&id=Adrenal第8页,此课件共61页哦AnatomyPrimary AldosteronismCushings SyndromeDHEA-sPheo

5、chromocytoma第9页,此课件共61页哦Frequency of FindingsvvMulticenter study of 1096 casesNonfunctioning adenoma:85%Nonfunctioning adenoma:85%Subclinical Cushings syndrome:9%Subclinical Cushings syndrome:9%Pheochromocytoma:4%Pheochromocytoma:4%Aldosteronomas:2%Aldosteronomas:2%Mantero et al.85(2):637.(2000)第10页

6、,此课件共61页哦Frequency of FindingsAllolio,B.,Adrenal Incidentalomas.Adrenal Disorders,ed.C.G.Margioris AN.2001,Totowa:Humana Press Inc.第11页,此课件共61页哦A summary of the literaturevNonfunctioning adenoma Approximately 80%vSubclinical Cushing syndrome(SCS),5%vPheochromocytoma 5%vAldosteronoma 1%vadrenocortica

7、l carcinoma(ACC)5%vMetastatic lesion 2.5%vGanglioneuromas,myelolipomas,or benign cysts第12页,此课件共61页哦考虑是否手术治疗之前准确的功能诊断非常必要 v嗜铬细胞瘤要进行认真的术前准备以避免术中和术后的发作和死亡。v原发性醛固酮增多症的患者需要明确是否存在肾上腺皮质增生及无功能的肾上腺腺瘤。肾上腺源性Cushing综合征的患者在行切除术后可能发生肾上腺皮质功能不全,激素的替代以及增减治疗需要非常仔细。亚临床Cushing综合征的患者是否需要手术治疗仍存在争议。v肾上腺皮质癌的患者手术前需要外科医师和内分泌

8、科医师或肿瘤科医师共同协商决定切除的方式,因为首次切除的效果是生存率的主要预测因素。v超过4cm的肾上腺无功能瘤可以考虑切除。小的髓脂肪瘤或良性的囊肿一般影像学检查即可确诊,通常不需要治疗,除非有症状可以考虑手术治疗。第13页,此课件共61页哦Algorithm for the evaluation and management of an adrenal incidentaloma*Reimage in 3 to 6 months and annually for 1 to 2 years;repeat functional studies annually for 5 years.If m

9、ass grows more than 1cm or becomes hormonally active,then adrenalectomy is recommended.第14页,此课件共61页哦Hyperfunctioning Hormonal EvaluationvvSubclinical Cushings SyndromevvPheochromocytomavvPrimary AldosteronismvvSex hormone-secreting adrenocortical tumors第15页,此课件共61页哦Subclinical Cushings SyndromevvHyp

10、ercortisolism without clinical manifestations of Cushings syndrome vvMost frequent hormonal abnormality in adrenal incidentalomas 第16页,此课件共61页哦Subclinical Cushings SyndromevvCentral obesityvvFacial roundingvvBuffalo humpvvEasy bruisingvvPurple striaevvProximal muscle weaknessvvEmotional/cognitive ch

11、anges第17页,此课件共61页哦Subclinical Cushings SyndromevvIncrease risk for:HypertensionDyslipidemiaImpaired glucose tolerance Type 2 DMAtherosclerosisOsteoporosis?Tauchmanova L,et.al.Patients with subclinical Cushings syndrome due to adrenal adenoma have increase cardiovascular risk.JCEM 2000;85:1440.第18页,此

12、课件共61页哦Subclinical Cushings SyndromevvBiochemical abnormalitiesElevated urine free cortisolLow or suppressed ACTH Blunted diurnal variation No cortisol suppression after 1 mg overnight dexamethasone suppression test-BEST SCREENING TEST!1.Mantero F,et al.1.Mantero F,et al.Hormone ResHormone Res 47:28

13、4289,1997 47:284289,19972.Montwill J,et al.The O/N DST is the procedure of choice for screening for Cushings 2.Montwill J,et al.The O/N DST is the procedure of choice for screening for Cushings syndrome.syndrome.SteroidsSteroids 1994;59:2296 1994;59:2296第19页,此课件共61页哦Dexamethasone Suppression Testvv1

14、 mg dexamethasone at 11PM1 mg dexamethasone at 11PMvvMeasure cortisol at 8 AM the next morningMeasure cortisol at 8 AM the next morningNormal:cortisol 1.8Normal:cortisol 30 and PAC 20 ng/dLPAC/PRA 30 and PAC 20 ng/dLvv90%spec and sensitivity for PA90%spec and sensitivity for PAvvIf screening test is

15、 positive-need to confirm with saline suppression test,adrenal venous sampling and imaging第35页,此课件共61页哦midnight salivary cortisol,or a 2-day low-dose dexamethasone suppression testmidnight salivary cortisol,or a 2-day low-dose dexamethasone suppression test第36页,此课件共61页哦第37页,此课件共61页哦Hyperfunctioning

16、Hormonal EvaluationvvSubclinical Cushings SyndromevvPheochromocytomavvPrimary AldosteronismvvSex hormone-secreting adrenocortical tumors第38页,此课件共61页哦Sex hormone-secreting Adrenocortical TumorsvvRarevvTypically occur in the presence of clinical manifestations(hirsutism or virilization)第39页,此课件共61页哦Hi

17、rsutism第40页,此课件共61页哦Sex hormone-secreting Adrenocortical TumorsvvRarevvTypically occur in the presence of clinical manifestations(hirsutism or virilization)vvRoutine screening for excess androgens and estrogens is not warranted第41页,此课件共61页哦Hormonal Workup Summaryvv3 hormonal tests necessary for work

18、up of adrenal incidentaloma:1 mg overnight dexamethasone suppresion testPlasma or urinary fractionated metaneprinesPlasma aldosterone concentration and plasma aldosterone concentration/plasma renin activity ratio(PAC/PRA).第42页,此课件共61页哦TreatmentvvAll patients with documented pheochromocytoma and All

19、patients with documented pheochromocytoma and primary aldosteronism should undergo surgeryprimary aldosteronism should undergo surgeryvvNo prospective,randomized trials for Subclinical Cushings Syndrome but concensus is to proceed with surgery if the patient is young第43页,此课件共61页哦Three Main Questions

20、vvIs the adrenal mass hormonally active?vvIs the mass benign or malignant?vvDoes the patient have a history of a previous malignant lesion?Is it metastatic?第44页,此课件共61页哦Primary Adrenal CarcinomavvVery rare:5 cases per 1 million populationvvSmall size corresponds to better prognosisvv5 year survivalO

21、verall:16%Localized disease(stage I and II):42%Metastases:5.3%第45页,此课件共61页哦Imagingcomplex solid and cystic,calcified mass第46页,此课件共61页哦Patient with Known Malignancyvv10-40%of patients with known malignancy have adrenal metastases at autopsyvvMost common primaryBreastLungKidneyMelanomaLymphoma第47页,此课件

22、共61页哦Assessment of Malignant PotentialvvSizevvImaging Phenotype(features)第48页,此课件共61页哦SizevvProbability of malignancy increases with sizeIn a study involving 887 patients with adrenal incidentalomas,90%of patients with adrenal carcinomas has tumor 4 cm(National Italian Study Group,National Italian S

23、tudy Group,1997)1997)adrenal carcinomas 2%(4cm)2%(6cm)25%(6cm)第49页,此课件共61页哦SizevvMayo Clinic Study342 Patients with adrenal incidentaloma retrospectively evaluatedTumor diameter averaged 2.5 cmMost malignant tumors measured 5 cmIncidentally discovered adrenal tumors:an institutional perspective.Herr

24、era MF;Grant CS;van Heerden JA;Sheedy PF;Incidentally discovered adrenal tumors:an institutional perspective.Herrera MF;Grant CS;van Heerden JA;Sheedy PF;Ilstrup DM.Surgery 1991 Dec;110(6):1014-21 Ilstrup DM.Surgery 1991 Dec;110(6):1014-21 第50页,此课件共61页哦SizevvConsensus StatementMass 6 cm should be re

25、movedMass 4 cm can be monitoredMass between 4-6 cm:Criteria other than size should be used to dictate surgery vs.monitoringManagement of the clinically inapparent adrenal mass(incidentaloma).NIH State-of-the-Science Conference Statement Feb 4-6,2002.第51页,此课件共61页哦Assessment of Malignant PotentialvvSi

26、zevvImaging Phenotype第52页,此课件共61页哦Image Phenotype-CT ScanvvHounsfield unit(HU)-semiquantitative method for measuring x-ray attenuationWater=0 HUAdipose tissue=-20 to-150 HUKidney=20 to 50 HUBone=1000 HUvvLipid rich mass are benignvvHU10 on unenhanced CT=benign adenoma 100%第53页,此课件共61页哦Image Phenotyp

27、e-CT ScanvvRetrospective analysis of 151 patients with adrenal massesvvHU10 or a combination of tumor size 4cm and HU 60%at 10 min=no cancervvWash out 60%at 10 min=high risk for malignant lesion第55页,此课件共61页哦Imaging-metastases第56页,此课件共61页哦MRIvvEqually effective as CTvvAdenomas are isointense with the

28、 liver on T2 weighted imagesvvCarcinomas are hyperintense compared to the liver on T2 weighted images第57页,此课件共61页哦FNAvvCytology from FNA cannot distinguish benign adrenal mass vs.malignantvvIt can distinguish adrenal tissue from metastasesvvFNA is useful only in distinguishing adrenal tumor from met

29、astasis and infectionvvNeed to rule out pheochromocytoma before FNA第58页,此课件共61页哦Follow UpvvWill the mass become hypersecretory?vvWill the mass become malignant?第59页,此课件共61页哦ConclusionsvvAll patients with an incidentaloma should have a 1-mg All patients with an incidentaloma should have a 1-mg dexame

30、thasone suppression test,a plasma/urinary dexamethasone suppression test,a plasma/urinary fractionated metanephrines and a aldosterone/renin fractionated metanephrines and a aldosterone/renin levellevelvvA homogenous mass with low attenuation value(HU10)A homogenous mass with low attenuation value(HU6 cm need to have the tumor removed,those with tumor 4 cm are typically monitoredvvTumors between 4 to 6 cm need to consider other criteria other than size第61页,此课件共61页哦

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