Objective To review the diagnosis and surgical therapeutic methods of adrenocorti-cal hyperplasia disease. Methods One hundred and eighty adrenocortical hyperplasia patients (74 males and 106 females with a mean age of 40 years) were retrospectively analyzed. The patients were divided into hypereortisolism (n=152) and aldosteronism (n=28) according to secretion. Data of clinical characteristic, endocrine and image examination were collected. All patients were treated by operation. Results Of these patients, 107 had Cushing disease (CD), 28 had adrenocorticotropin independent macronodular adrenal hyperplasia (AIMAH), 19 had ectopic adrenocorticotropin adrenal hyperplasia (EAAH), 4 had primary pigmented nodular adrenocortical hyperplasia (PPNAH), 28 had DOI: 10.3760/cma.j.issn. 1000-6702.2009.05.002idiopathic hyperaldosteronism (IHA). 24 h urinay free cortisol (24hUFC) excretion of CD, AIMAH, EAAH and PPNAH were 95.2-535.7 μg (mean, 287.6 μg), 24.8-808.2 μg (mean, 307.9 μg), 102.5-3127.0 μg (mean, 852.5 μg), 243.8-1124.6 μg (mean. 564.3 μg). The proportion loss of the serum cortisol circadian rhythm were 99% (102/103), 92% (11/12), 100% (17/17), 100% (4/ 4), respectively. Low- and high-dose dexamethasone suppression tests (DDST) failed to suppress cor-tisol secretion in AIMAH, PPNAH and EAAH groups, but HDDST was suppressed in CD group. Of the 28 IHA cases, 17 had hypokalemia and 15 had high urine kalium (>30 mmol/24 h). The results of plasma renin activity and serum aldosterone accorded with the diagnosis. Unilateral adrenalectomy were operated in 102 hypercortisolism cases, and 24hUFC of CD, AIMAH, EAAH and PPNAH were 56.2-233.5 μg (mean, 157.4 μg), 22.5-418.5 μg (mean, 117.9 μg), 116.5-1137.0 μg (mean, 756.7 μg), 124.6-422.6 μg (mean, 164.3 μg) 1 week after operation. The blood pressure was nor-mal in 8 paitents and droped in 13 patients for IHA after unilateral adrenalectomy. 24hUFC were nor-mal in 55 patients after bilateral adrenalectomy for hypercortisolism. One hundred and six patients were followed up for 4-158 months, the Cushing syndrome ameliorated and blood pressure dropped. Conclusions Unilateral adrenalectomy is the first choice for adrenocortical hyperplasia disease which needs operation. The operation mode of the contralateral adrenal gland is based on the hyperplasia types and clinical observation.

Cushing syndrome (CS) is a series of clinical symptoms and signs caused by long-term exposure to abnormally high levels of glucocorticoids.It is divided into two types of adrenocorticotropic hormone(ACTH) dependent and ACTH-independent CS.There are great differences in the target gland treatment of different causes of the disease, but the basic principle is to make a clear diagnosis.It is very important to treat primary lesions for ACTH-dependent CS.The adrenal gland will be resected when the treatment of primary lesions is not satisfied or the diagnosis is not clear.Adrenal sparing surgery is recommended for the definitive diagnosis adenomas of CS or subclinical CS.There is a lot of controversy about the treatment of ACTH-independent adrenal gland hyperplasia and primary pigmented nodular adrenal gland, and the individualized treatment scheme has been recognized.

Objective: To investigate the clinical effects of perforating branch flaps of medial vastus muscle in repairing secondary wounds in donor sites of free anterolateral femoral perforator flaps. Methods: From August 2014 to December 2016, 12 patients (8 males and 4 females, aged 35-72 years) with skin and soft tissue defects of extremities associated with tendon and bone exposure were treated in Hanzhong Central Hospital. The sizes of the primary wounds after debridement were 10 cm×8 cm-22 cm×14 cm, and the wounds were repaired with 12 cm×10 cm-24 cm×16 cm free anterolateral femoral perforator flaps. The anterolateral femoral donor sites, which were 8.0 cm×4.0 cm-14.0 cm×7.5 cm in the secondary wounds after skin extensional suture, were repaired with perforating branch flaps of medial vastus muscle in the size of 9.0 cm×5.0 cm-15.0 cm×8.5 cm. The medial femoral donor sites were sutured directly. Results: All the perforating branch flaps of medial vastus muscle and free anterolateral femoral perforator flaps survived in 12 patients. Following up for 6 to 12 months, the medial femoral perforator flaps had good local shape and texture. The flaps of 8 patients without cutaneous nerve transection were sensitive. The sensation of the flaps of the other 4 patients gradually recovered, and the functions of the ipsilateral knee joints were normal. Conclusions The medial femoral perforator flap has a stable anatomy and abundant blood supply, which can be used to repair the secondary wound in the donor site of the free anterolateral femoral perforator flap conveniently. It is safe and easy to be popularized. Moreover, it has a good shape and function after operation.

Objective To study the expression of growth inhibitor gene p33ING1 in bladder transitional cell carcinoma(TCC) and its clinical significance.Methods The expression of p33ING1 was examined in 68 cases(58 men and 10 women) of TCC and 12 cases of normal bladder tissues(controls) by immunohistochemistry method.The mean age of the 68 cases at diagnosis was 65 years.The pathological grading showed grade Ⅰ in 16 cases,Grade Ⅱ in 22 and Grade Ⅲ in 30.The clinical staging showed T_1 in 25 cases,T_2 in 17,T_3 in 14 and T_4 in 12.Of the 68 cases,47 developed relapse.Results The p33ING1 was strongly expressed in all the 12 cases of normal bladder tissues with a positive rate of 100%;whereas the overall positive rate of p33ING1 in the 68 cases of bladder carcinoma was 33.8%(23/68).The positive rates of p33ING1 in grade Ⅰ,Ⅱ and Ⅲ cases of bladder carcinoma were 75.0%(12/16),31.8%(7/22) and 13.3%(4/30),respectively;the differences of positive rates between grade Ⅰ and grade Ⅱ,grade Ⅰ and grade Ⅲ cases was significant(P

Objective To evaluate the effect of mitotane as a preoperative adjuvant therapy in advanced adrenocortical carcinoma.Methods Mitotane was used in 2 patients as preoperative adjuvant therapy.Case 1,a 24-year-old woman had Cushing's symptom for 1 year and presented with edema in both legs for 1 month.Computed tomography (CT) showed a solid mass in the right adrenal.It was about 10.0 cm×7.8 cm with calcification,and the boundary of tumor was not clear.Enhanced CT scan showed heterogeneous enhancement and there was a suspicious filling defect in the inferior vena cava.Biopsy of adrenal neoplasm was made and pathological result was adrenocortical tissue.The patient was diagnosed as right adrenocortical carcinoma with tumor thrombus in vena cava.Mitotane was used for 6 months.Case 2,a 42-year-old woman who underwent left adrenal adenoma resection 9 years ago,presented with Cushing's syndrome for 2 years,and found masses in left retroperitoneal and abdominal wall for 6 months.CT showed multiple nodular soft tissues with pitting calcification and fusion in it,locating between retroperitoneal spleen and kidney.The biggest section was approximately 8 cm× 12 cm,with a largest diameter of 14 cm.Enhanced scan showed uneven density wiht CT value of about 32 HU.Nodule shadows which were obviously strengthened were found in front of the right side of the diaphragmatic muscle.And some heterogeneous enhanced nodule shadows could be seen in front of the right abdominal peritoneal and muscle layer.The patient was diagnosed as right adrenocortical carcinoma with multiple metastases.Mitotane was used for treatment for 5 months.Results Both of their Cushing's symptom were significantly improved by taking drugs.The main side effects were nausea,vomiting,dizziness,fatigue,diarrhea and so on,but all could be alleviated after drug dosage adjustment.CT reexamination showed the volume of the primary lesion and metastases were significantly reduced.The postoperative pathological report after remove of the tumor and metastases showed adrenocortical carcinoma.Followed up for 42 and 7 months,the patients had no recurrence.Conclusions Mitotane is effectiveas preoperative adjuvant therapy in adrenocortical carcinoma and metastases.It can reduce the lesions significantly,relieve the Cushing's symptom,and provide opportunity for surgical treatment.

Objective To explore the feasibility and to improve the efficiency of testing circulating tumor cells(CTC)in patients with renal cell carcinoma by Cell-search System(CSS).Methods Eight patients with renal cell carcinoma hospitalized in the PUMC urology department for further clinical evaluation in Jan.to Jun.2012 were enrolled in this study.There were 5 males and 3 females with mean age of 64 (57-70)years.There were 2 cases in clinical stage T3N0M0 and 6 cases in T4N0M1;3 cases were treated with radical nephrectomy,5 cases were treated with targeted therapy drugs.7.5 ml peripheral blood samples from these patients were collected and saved in test tube at the temperature of 15 ℃-30 ℃.The tests had to be done within 96 hrs after the blood sample drawn.We selected fluorescent anti-CKS/18/19 antibodies as CTC makers for renal cell carcinoma.Then the number of CTC was quantitative detected by CSS.The feasibility and detection rate of testing CTC in patients with RCC by CSS was evaluated,and the improvement of method was discussed.Results Quantitative test results of CTC in 8 patients with renal cell carcinoma were negative.25 CK+CD45 + double positive cells were found in one T4N0M1 patient's peripheral blood.The detection rate of the CTC in TNM stage T3N0M0 and T4N0M1 renal cell carcinoma patients using CSS with epithelial tumor cell maker anti-CK8/18/19 was zero.Conclusions Anti-CK8/18/19 antibodies can not be used in testing circulating tumor cells in patients with renal cell carcinoma by Cell-search System.

Objective To evaluate the value of targeted second-generation sequencing (NGS) in the genetic diagnosis of tuberous sclerosis complex (TSC) associated with renal complications.Methods The clinical data of 43 patients (with 33 patients of definite diagnosis and 10 patients of possible diagnosis)with tuberous sclerosis complex associated with renal complications were analyzed.There were 26 females and 17 males with a mean age of 28 (10 ～ 48) years ranging from 10 to 48 years old.All patients had renal complications,including 39 renal angiomyolipomas,3 renal cysts and 1 renal cell carcinoma.Written informed consents were signed,and 5ml peripheral blood was drawn for DNA extraction.Mutations of TSC1 and TSC2 genes were detected by the NGS technology,and then confirmed by Sanger sequencing.Results TSC1 or TSC2 pathogenetic mutants were identified in 39 patients (90.7％),which were consistent with clinical phenotypes and two non-significant mutations were identified in two individuals,while no mutation was detected in the other two cases.Fourteen novel mutations were reported for the first time,including 8 frameshift mutation,3 deletion mutation,2 nonsense mutation and 1 splicing mutation.Conclusion NGS is an accurate and less time-consuming technology in detecting TSC1 or TSC2 gene mutation,which has great value in genetic diagnosis of tuberous sclerosis complex associated with renal complications.

Objective To investigate the effect of has-miR-663 on proliferation and apoptosis of human adrenal cortical cell line SW-13.Methods Based on the first stage study,in adrenocorticotropinindependent macronodular adrenal hyperplasia (AIMAH),hsa-miR-663 was up-regulated.The synthetic hsa-miR-663 mimics and inhibitors were transfected the SW-13 cell line.Real-time quantitative PCR (RT-PCR) tested the effect of the transfection.According to the results of RT-PCR,cell line was divided into five groups,including blank group,mimics NC group,inhibitor NC group,mimics group,inhibitor group.Then proliferating of the cell was tested by MTT and the apoptosis by Annexin V-FITC/PI.Result By RT-PCR,mimics group showed that has-miR-663 was over-expressed,and inhibitor group were knocked down.MTTshowed that,compared with NC group and Blank group,SW-13 cell line proliferated faster in mimics group and slower in inhibitor group.Annexin V-FITC/PI showed that apoptosis of SW-13 cell line was not different in all the five groups.Conclusion Up-regulating has-miR-663 could accelerate proliferation of adrenal cortical cells.The microRNA might play some role in the pathogenesis of AIMAH.

Objective To investigate the clinical characteristics of tuberous sclerosis-associated huge renal angiomyolipoma,and to summarize the experiences of diagnosis and treatment.Methods The clinical data of 9 patients with tuberous sclerosis-associated huge renal angiomyolipoma treated in Peking Union Medical College Hospital from January 2009 to June 2014 was analyzed retrospectively,including 4 males and 5 females.The age at onset and diagnosis of tuberous sclerosis was 0 to 7 years (mean 2.3 years) and 2 to 30 years (mean 19.6 years),respectively,and the diagnosis age of renal angiomyolipoma was 14 to 35 years (mean 25.2 years).In the 9 patients,8 cases had facial angiofibromas,7 cases had hypomelanotic macules,6 cases had ungual fibromas and epilepsy,and 5 cases had family history.All the patients underwent abdominal ultrasound examination,CT scan and serum creatinine testing,and 3 cases received renal dynamic imaging to assess renal lesions and functional status.Results Imaging examinations showed typical renal angiomyolipomas,which were bilateral and multiple lesions with the maximum diameter greater than 10 cm in every patient,and with 2 patients had a history of acute retroperitoneal hemorrhage.The levels of serum creatinine were normal in all patients,and 3 patients had unilateral glomerular filtration rate of 44.4-99.1 ml/min (mean 62.9 ml/min).One patient underwent unilateral partial nephrectomy,2 patients underwent selective renal artery embolization,1 received blood transfusion due to acute bleeding,1 received rapamycin treatment,and 4 patients underwent close follow-up.Conclusions Tuberous sclerosis-associated renal angiomyolipomas are often bilateral and multiple.Increase of the lesion diameter may only enhance the risk of bleeding with small effect on renal function.Therapeutically,close follow-up and mTOR inhibitor are the preferred treatment options,surgery or selective renal arterial embolization is used only for the possible malignancy,acute bleeding or patients with high risk of bleeding.

Objective To analyze the characteristics of adrenal metastasis from renal cell carcinoma (RCC),and explore the principles to deal with the ipsilateral adrenal gland during radical nepbrectomy.Methods The data of adrenal metastasis in 19 patients with RCC from January 2003 to December 2012 was retrospectively analyzed,including 8 cases with ipsilateral adrenal metastasis,5 with contralateral adrenal metastasis,and 6 with bilateral adrenal metastasis,respectively.The relationship between RCC location,diameter,TNM stage and adrenal metastases was evaluated,and the clinical features of adrenal metastasis were summarized.Results Diameters of RCC ranged from 1.6 cm to 12.6 cm in the 19 cases,and the average diameter was 6.3 cm.The mean diameter of primary RCC,metastasized to the ipsilateral,contralateral and bilateral adrenal gland,was 5.6,6.5 and 7.1 cm,respectively.Among the 9 patients whose primary RCC located in the upper pole,the number of patients with bilateral,ipsilateral and contralateral adrenal metastasis was 3,3,3,respectively.Of the 7 patients with RCC located in the middle and lower pole,the number of patients with bilateral,ipsilateral and contralateral adrenal metastasis was 3,2,2,respectively.Three patients with diffused RCC in the whole kidney occurred ipsilateral adrenal metastases.RCC and adrenal metastasis were simultaneously found in 9 patients,with the average diameter of the primary RCC of 7.4 cm and the clinical stage of T2b-4N0-1 M1.Adrenal metastasis were found in 10 patients during the postoperative follow-up,with the average diameter of the primary RCC of 5.3 cm and the clinical stage of T1a-4N0M0.Nine patients underwent surgery,8 received non-surgical treatment,and 2 had no treatment.Fourteen patients had an average follow-up of 31.9 months,with 1,3 and 5-year survival rates of 84.2％,26.3％ and 15.8％,respectively.Conclusions Adrenal metastasis from RCC may be associated with the diameter and TNM of the primary tumor,regardless of the location.For the patients with normal adrenal gland observed in pre-operative imaging,ipsilateral adrenalectomy is not recommended.