1. Surgical Treatment And Analysis Of Prognosis For Renal Tumor With Vena Cava Tumour Thrombus And Establishment Of Cox Model 2. Clinical Genetics Investigation In Chinese Kindred With ACTH-independent Macronodular Bilateral Adrenal Hyperplasia 3. Design

[Objective]To study the therapeutic effects of surgical treatment for renal tumor with vena cava tumor thrombus(VCTT),and typing system of VCTT.Screen prognostic factors and establish postoperative Cox model of renal tumor with VCTT by survival analysis.[Methods]A retrospective analysis of the cases of renal tumor with VCTT from Jan.1 1993 to Apr.2008 was performed.The Cox model was used for multivariate analyses. Survival curve was draw by Kaplan-Meier method,and Cox model was established.[Results]The postoperative median survival time of renal cell carcinoma of VCTT is 30.8 month.1-year and 5-year survival rate is 77%and 43%.The new typing system of VCTT designed by Prof.Xiao was applied reliably.And it can be guide to select modus operandi definitely.The alone prognostic factors in postoperative renal tumor with VCTT were wall of vein invaded by tumor and tumor size,otherwise tumor invading extent,necrosis of tumor center,lymph node metastasis were important prognostic factors.Establish Cox model of postoperative renal tumor of VCTT,and to classify two groups(high-dangerous group and low-dangerous group)by means of prognostic index(PI).[Conclusion]Surgical treatment is preferred option in the patients with renal tumor with VCTT.It was profit to selection of modus operandi that the new typing system of VCTT designed by Prof.Xiao.The alone prognostic factors in postoperative renal tumor with VCTT were wall of vein invaded by tumor and tumor size.The Cox model can accurately identify patients with different prognosis,and a high PI correlates with high risk and poor prognosis,thus representing a useful tool in the treatment of the disease. Introduction and Objective:ACTH-independent macronodular adrenal hyperplasia(AIMAH)is an endogenous form of adrenal Cushing syndrome characterized by massive multiple bilateral adrenocortical nodules.So far few familial cases have been reported,and the majority of AIMAH cases are sporadic.By reporting these cases on a family of five generations with ten infected members,our research aims to analyze the pedigree and karyotype as well as study the inheritance pattern of familial AIMAH.Methods:This research was based on the first case of AIMAH family in China, which is also the largest AIMAH ancestry ever known to the world.With the approval from The Ethics Committee,we developed the further study of the family.After obtaining informed consent from all consenting study participants in the family,history-taking,medical examination,endocrine examination and conventional imaging are performed.The samples of blood and excision tissue were collected for the purpose of making future karyotype and SNP analysis. Our pedigree analysis explored the inheritance pattern.Results:This family of five generations had 48 members in total.Among 28 members who are alive and available for screening,five of them exhibited the clinically apparent Cushing's syndrome and four were sub-clinical since the first three generation,and the ratio between male and female was 4:5.The result of the Karyotype analysis showed normal on the twelve family members including affected,normal and sub-clinical.Conclusions:This study indicated the following results:(1)Familial AIMAH is a single gene inheritance disease,conforming to a delayed autosomal dominant hereditary pattern.(2)The onset of the disease generally starts during 50 to 60 age set.The typical clinical features are Cushing syndrome,with the presence of bilateral adrenal macronodular masses in imaging.(3)The sub-clinical AIMAH is defined as the absence of clinical signs of CS,with slight to normal plasma cortisol and bilateral adrenal macronodular masses.All these valuable resources and the abundant survey work have provided us with reliable data to conduct genetic linkage studies and locate the genes for familial AIMAH.