Clinical imaging features and prognosis of von Hippel-Lindau syndrome associated with pancreatic lesions / 中华消化外科杂志

Objective:To investigate the clinical imaging features and prognosis of von Hippel-Lindau (VHL) syndrome associated with pancreatic lesions.Method:The retrospective case-control study was conducted. The clinicopathological data of 161 patients with VHL syndrome who were admitted to Peking University First Hospital from September 2010 to August 2022 were collected. There were 83 males and 78 females, with age of onset as 27.0(range, 8.0-66.0)years. Observation indicators: (1) imaging results of VHL syndrome associated with pancreatic lesions; (2) clinical characteristics of VHL syndrome associated with pancreatic lesions; (3) comparison of clinicopathological factors in patients with VHL syndrome associated with pancreatic cystic lesions; (4) comparison of clinicopathological factors in patients with VHL syndrome associated with pancreatic neuroendocrine neoplasms (pNENs). (5) Treatment and prognosis of patients with VHL syndrome associated with pancreatic lesions. Measurement data with normal distribution were represented as Mean± SD, and comparison between groups was conducted using the independent sample t test. Measurement data with skewed distribution were represented as M(range), and comparison between groups was conducted using the non-parameter test. Count data were described as absolute numbers, and comparison between groups was conducted using the chi-square test. Results:(1) Imaging results of VHL syndrome associated with pancreatic lesions. Of the 161 patients with VHL syndrome, there were 151 patients associated with pancreatic lesions and 10 patients not associated with pancreatic lesions. Of the 151 patients with VHL syndrome associated with pancreatic lesions, there were 136 patient with pancreatic cystic lesions and 34 patients with pNENs, 22 patients with both pNENs and pancreatic cystic lesions, and the type of pancreatic lesions could not be accurately determined in 3 cases. (2) Clinical characteristics of VHL syndrome associated with pancreatic lesions. The age of onset in 151 patients with VHL syndrome associated with pancreatic lesions was 33.0(range, 14.0-68.0)years. Cases with gene site mutation of exon 1, exon 2, exon 3 and other types of gene site was 51, 16, 43 and 41, respectively. There were 116 patients of VHL type 1 and 35 patients of VHL type 2. There were 92 patients with family history of VHL syndrome and 59 patients without family history of VHL syndrome. There were 127 patients combined with renal cell carcinoma, 112 patients combined with central nervous system lesions, 46 patients combined with retinal hemangioblastoma. Patients may combined with multiple lesions. (3) Comparison of clinicopathological factors in patients with VHL syndrome associated with pancreatic cystic lesions. The age of onset, VHL syndrome type (VHL1 type, VHL2 type) and cases combined with renal cell carcinoma were 32.5(range, 14.0-68.0)years, 110, 26 and 115 in 136 patients with VHL syndrome associated with pancreatic cystic lesions, versus 22.0(range, 8.0-64.0)years, 13, 12 and 14 in 25 patients with VHL syndrome not associated with pancreatic cystic lesions, showing significant differences in the above indicators between them ( Z=-3.384, χ2=9.770, 10.815, P<0.05). (4) Comparison of clinicopathological factors in patients with VHL syndrome associated with pNENs. The age of onset, gene mutation sites (exon 1, exon 2, exon 3, other types of gene site) and VHL syndrome type (VHL1 type, VHL2 type) were 33.5(range, 14.0-64.0)years, 12, 5, 14, 3 and 18, 16 in 34 patients with VHL syndrome associated with pNENs, versus 27.0(range, 9.0-66.0)years, 41, 12, 32, 42 and 105, 22 in 127 patients with VHL syndrome not associated with pNENs, showing significant differences in the above indicators between them ( Z=-4.030, χ2=8.814, 13.152, P<0.05). (5) Treatment and prognosis of patients with VHL syndrome associated with pancreatic lesions. Of the 161 patients with VHL syndrome, 3 patients underwent surgical treatment, and the remaining patients were followed up. All 161 patients with VHL syndrome were followed up for 6 (range, 1-12)years, in which 15 patients died and 146 patients alive during the follow-up. The follow-up time of 3 patients undergoing surgical treatment was 4, 14, 9 years, respectively, and all of them were alive. Conclusions:The clinical imaging features of pancreatic lesions related to VHL syndrome are cystic lesions and pNENs, which with the characteristics of multiple lesions and benign tumors. Such patients usually do not requiring surgical treatment and have good prognosis.

Analysis of risk factors for intraoperative hemodynamic instability in adrenal incident pheochromocytoma / 中华泌尿外科杂志

Objective To evaluate the risk factors for intraoperative hemodynamic instability (HI) in patients with adrenal incident pheochromocytoma.Methods Perioperative clinical parameters of patients undergoing surgery for adrenal incident pheochromocytoma at the First Hospital of Peking University from January 2001 to July 2018 were analyzed.There were 39 males and 41 females,with mean age of 45.1 years (13-76 years old).The median tumor length was 5.1 cm (1.5-14.0 cm),with 25 cases (31.3％) on the left side,55 cases (68.8％) on the right side.There were 37 cases combined with coronary heart disease or diabetes or BMI≥24 kg/m2.Patients were divided into hemodynamic instability (HI group) and hemodynamic stability group (HS group) by whether intraoperative hemodynamic instability occurred.The differences of demographic characteristics and clinical parameters between the two groups were compared.Logistic regression analysis was done for seeking the risk factors for hemodynamic instability during surgery.Results There were 54 cases (67.5％) in the HS group and 26 cases (32.5％) in the HI group.Univariate analysis showed that there was no significant difference in age [(44.06 ± 13.58) years old vs.(47.35 ± 16.11) years old],combined with coronary heart disease or diabetes or BMI≥24 kg/m2 [50.0％(27/54) vs.38.5％ (10/26)],tumor long diameter [median 5.0 cm(1.5-14.0 cm) vs.6.0cm(1.5-13.5 cm)],tumor location [left:29.6％ (16/54) vs.34.6％ (9/26)],preoperative catecholamine test positive [44.4％ (20/45) vs.50.0％ (10/20)],open surgery [27.8％ (15/54) vs.34.6％ (9/26)]and preoperative non-alpha blockers[13.0％ (7/54) vs.30.8％ (8/26)] between HS group and HI group (P ＞ 0.05).Further logistic regression analysis was used to analyze the risk factors of intraoperative hemodynamic instability.Multivariate analysis found that patients who preoperative non-alpha blockers before surgery were independent risk factor for HI (OR =4.574,95 ％ CI 1.273-16.432,P =0.020).Conclusions Preoperative non-alpha blocker in patients with adrenal incidental pheochromocytoma could be independent risk factor for intraoperative hemodynamic instability.Therefore,it is recommended that patients with adrenal incidental tumors,especially those who fail to rule out pheochromocytoma,take preoperative alpha blockers.

Risk factors for positive surgical margin after laparoscopic radical prostatectomy / 中国肿瘤临床

Objective: To identify risk factors for positive surgical margin after laparoscopic radical prostatectomy. Method: The study retrospectively analyzed the records of 177 patients with prostate cancer who eventually underwent laparoscopic radical prostatecto-my from January 2016 to December 2017 in Peking University First Hospital. Age, prostate volume, prostate-specific antigen (PSA) be-fore needle biopsy, number of positive cores, positive percentage of needle biopsy and biopsy, and postoperative Gleason scoreand pathological stage were analyzed. Results: The overall positive surgical margin rate was 32.2% (57/177). Age, prostate volume, PSA be-fore needle biopsy, positive percentage of biopsy, and postoperative Gleason score were not significantly different (P>0.05). The study demonstrated significant differences between the number of positive cores, positive percentage of needle biopsy, and pathological stage (P<0.05). Multiple logistic regression revealed that the pathological stage was an independent factor affecting the positive surgi-cal margin rate (odds ratio, 1.616; 95% confidence interval, 1.062-2.459). Conclusions: The number of positive cores, positive percent-age of needle biopsy, and pathological stage significantly correlated with a positive surgical margin. The postoperative pathological T stage is an independent factor affecting positive surgical margins.

Clinical value of fluorescence in situ hybridization positive of exfoliated urothelial cells in urothelial carcinoma / 北京大学学报(医学版)

Objective: To analyze the clinical pathologic characteristics of cases with fluorescence in situ hybridization (FISH) positive of exfoliated urothelial cells, so as to evaluate the clinical utility of FISH in the diagnosis of urothelial carcinoma (UC).Methods: A total of 271 cases of FISH positive in Department of Urology of Peking University First Hospital from Apr.2012 to Sep.2015 were recruited in this study.Retrospective analysis was made on their clinical data.For FISH analysis, labeled probes specific for chromosomes 3, 7, 17, and the p16 (9p21) gene were used to assess chromosomal abnormalities indicative of malignancy.The positive predict values (PPV) of all the techniques were analyzed.Results: Of the 271 patients, 207 cases were UC, 7 cases were non-UC, and 57 cases were benign diseases.The PPV of FISH in detecting UC was 76.4%, while the 95% confidence interval (CI) 71.3% to 81.5%.In the cohort of FISH positive, this value was similar to that of urinary cytology (PPV 86.8%, 95% CI: 78.5%-95.0%).The PPV of FISH was lower than that of cystoscopy and ureteroscopy (PPV 96.1%, 95% CI: 91.7%-100.0%).There were significant differences between this study and the PPV of FISH reported abroad (PPV 53.9%, χ2=33.048, P<0.001).Of all the UC with FISH positive, bladder cancer showed an earlier pathological stage versus renal pelvic carcinoma and ureteral carcinoma, with significance (χ2=5.894, P=0.015, and χ2=13.601, P<0.001, respectively).However, no difference was found in the size, pathological stage and pathological grade of tumors between the urinary cytology positive group and the urinary cytology negative group.The rate of high-grade UC in ureteral carcinoma of FISH positive was 92.3%, much higher than that of ureteral carcinoma reported domestically.Conclusion: The PPV of FISH in detecting UC is higher relatively, with a better clinic value for Chinese patients.The ureteral carcinoma with FISH positive obtains a higher pathological grade, which is of great guiding significance for UC.

Percutaneous biopsy of the renal masses under ultrasound:a single-center 14 years experience / 北京大学学报(医学版)

Objective: To assess the diagnostic rate, safety and clinical application of percutaneous renal masses biopsy for advanced renal cell carcinoma patients.Methods: In this retrospective study, we collected the data of renal masses from the patients who underwent renal masses biopsy under ultrasound from April 2001 to December 2014 in Peking University First Hospital.A total of 75 patients who were undiagnosed or diagnosed with advanced renal cell carcinoma by the imageological method were enrolled in this study.The patient and lesion characteristics such as tumor size, pathology of tumor, histologic subtype, pathological grade, biopsied location and biopsied cores were recorded and analyzed.Results: Among all the 75 patients, biopsy was diagnostic in 64 cases (85.3%) and non-diagnostic in 11 cases (14.7%).Of the 64 diagnostic biopsies, 60 were malignant, including 37 (61.7%) renal cell carcinoma (RCC), 13 (21.7%) urothelial carcinoma and 10 (16.7%) other malignant masses.Of all the RCC subjects, 24 suffered from clear cell RCC, 5 papillary RCC, 3 collecting duct carcinomas, 1 unclassified RCC and 4 unknown subtypes.The 11 non-diagnostic biopsied samplings included inflammatory, blood and extrarenal tissue and normal renal tissue.The proportion of collecting duct carcinoma in RCC was 10.8% and the proportion of squamous carcinoma in urothelial carcinoma was 23.1%, which were both higher than the previous research findings.For the male and female groups, non-diagnostic yields were 6.5% and 30.4%, respectively (P=0.022).Of all the 75 patients, 13 renal cell carcinoma patients underwent the surgical treatment and got the results of postoperative pathology.Comparing preoperative biopsy pathological diagnosis with postoperative pathological diagnosis, we found the diagnostic correct rates for benign and malignant lesions, pathological subtype and pathological grade were 100%, 81.8% and 60%, respectively.Mild macroscopic hematuria occurred in 1 case after RMB and there were no serious complications in all the cases.Conclusion: Percutaneous renal masses biopsy under ultrasound with a high diagnostic rate which can define the histologic subtype of renal cell carcinoma.With targeted therapy, more and more patients whose evaluation suggests local advanced disease or metastatic tumors adopt renal tumor biopsy to define the histologic subtype, which could avoid unnecessary surgical treatment.

Evaluation of efficacy and safety of sunitinib regimen in 22 patients with metastatic renal cell carcinoma: at least 12-month follow-up / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>Sunitinib has been proved an effective new option for treatment of metastatic renal cell carcinoma (mRCC). Analysis of clinical data of 22 patients, who were exposed to sunitinib for at least 1 year, was conducted to evaluate the long-term efficacy and safety of sunitinib for the treatment of mRCC.</p><p><b>METHODS</b>A total of 54 patients with mRCC were treated with sunitinib malate, 50 mg/d orally, on a 4-weeks-on and 2-weeks-off dosing schedule in Peking University First Hospital. Treatment continued until disease progression, unacceptable adverse events (AEs), or death. Among them, 22 patients continued treatment for at least 1 year. The clinical data of these 22 patients were prospectively collected for analysis. AEs were assessed according to the National Cancer Institute Common Terminology Criteria for Adverse Events, Version 3.0. Tumor response was evaluated in accordance with the Response Evaluation Criteria in Solid Tumors.</p><p><b>RESULTS</b>Median progression-free survival was 19.5 months until last follow-up. The best efficacy results achieved were complete response, partial response, and stable disease for 2, 9, and 11 patients, respectively. Objective response rate was 50%. The most common AEs were hand-foot syndrome (95%) and hypertension (91%). Other common AEs were thyroid-stimulating hormone elevation (82%), platelet decrease (77%), and loss of appetite (77%). Only one patient withdrew from treatment for cardiac infarction. Another nine patients experienced dose modifications or short-term suspensions.</p><p><b>CONCLUSION</b>Long-term exposure to sunitinib malate showed encouraging efficacy in the treatment of mRCC. At the same time, the tolerability was good.</p>

Analysis of clinicopathological characteristics and survival time of penile cancer after surgery / 中华泌尿外科杂志

ObjectiveTo analyze the clinicopathological characteristics and survival time of penile cancer after surgery.MethodsThe clinicopathological data of 93 cases of penile cancers patients treated from January 2002 to December 2010 were collected retrospectively.The Kaplan-Meier method was used to draw the survival function and calculate the survival rate.Log-rank test was further used to compare survival difference.ResultsThe median age of the 93 patients was 51 years ( range,23 to 82).Squamous carcinoma was most common with 87 eases (93.5％) in our group.Sixty-one patients were successfully followed up and the median follow up duration was 28 months(range,2 to 89 months).All 3 cases of verrucous carcinoma had progression free survive after surgery after follow up of 12,19,and 67 months.In the 55 patients with penile invasive squamous carcinoma,11 patients died of metastatic disease with a median survival time of 10 months ( range,2 to 24 months).Two years cumulative survival rate was 75.7％ (95％ CI 63.0％ to 88.4％ ).Six cases of 12 patients with lymph node metastasis died of penile cancer while only 5 cases of 43 patients without lymphnode metastasis died.The survival difference was significant (log rank,P =0.000).ConclusionsSquamous carcinoma is the most common type,and patients with lymphnode metastasis have poor prognosis.Verrucous carcinoma has good prognosis.

Analysis of grant support of National Natural Science Foundation of China in Peking University First Hospital during the period of 11th Five-Year Plan and discussion on the management method / 中华医学科研管理杂志

During the period of 11th Five-Year Plan,the Natural Science Foundation of China Peking University First Hospital acquired has improved in the total amount and project types.An analysis was made on the status of grant application and support in Peking University First Hospital as well as on the management method of the grant,offering some suggestions for the general hospitals on the management and development of Natural Science Foundation.

Efficacy and safety of sunitinib in the treatment of metastatic renal cell carcinoma / 中华医学杂志(英文版)

<p><b>BACKGROUND</b>The tyrosine kinase inhibitors (TKIs) sunitinib, the first targeted agent for the first line treatment of metastatic renal cell carcinoma (RCC), targets the vascular endothelial growth factor (VEGF) pathway. The objective of this study was to investigate the efficacy and safety of sunitinib in treating metastatic clear-cell RCC and to confirm if hypertension is an effective predictive factor.</p><p><b>METHODS</b>A total of 36 patients with metastatic RCC were enrolled between June 2008 and December 2010. Among them 29 cases were first line therapy and 7 cases were in progression on first-line cytokine or sorafinib therapy. The pathology of all patients was confirmed predominant in clear cell type. Sunitinib mono-therapy was administered in repeated 6-week cycles of daily oral therapy for 4 weeks, followed by 2 weeks off in 34 patients; and 3 patients were administered with 37.5 mg/d continuously until disease progression or unacceptable toxicities occurred. Overall response rate and safety were evaluated. We divided patients into Group A and Group B according to the blood pressure level.</p><p><b>RESULTS</b>The median follow-up was 15 months (10 cycles, range 1.5 - 30.0 months (1 - 20 cycles)). Ten patients (29.4%) achieved partial responses (PR); 23 patients (67.6%) demonstrated stable disease (SD) lasting ≥ 2 cycles. Seventeen patients (50%) developed progressive disease (PD) during follow-up. The median progression-free survival (PFS) was 15 months (range 3.0 - 28.5) months. A total of 9 patients died; the overall survival has not been reached; the median survival time of the deceased patients was 13 months (range 7 - 24) months. The most common adverse events were hand-foot syndrome (77.8%), thrombocytopenia (75.0%), hypertension (61.1%) and diarrhea (46.0%). Most adverse events were reversible by treatment interruption. Twenty-two patients (61.1%) developed hypertension; and hypertension was associated with a long time to disease progression and long overall survival (P = 0.004, 0.000, respectively).</p><p><b>CONCLUSIONS</b>The results of this study demonstrate the efficacy and manageable adverse event profile of sunitinib as a single agent in first- or second-line therapy for patients with metastatic clear cell RCC. Further, sunitinib-associated hypertension may be a strong predictive marker for treatment efficacy in metastatic RCC.</p>

Efficacy and safety of sunitinib in treating metastatic renal cell carcinoma: a single center 37 cases report / 中华泌尿外科杂志

Objective To evaluate the efficacy and safety of sunitinib in the treatment of metastatic renal cell carcinoma (RCC). Methods A total of 37 patients with metastatic RCC were treated with between June 2008 and April 2010, including 28 males and 9 females. The median age was 52 (17-74) years. All patients received a pathologic diagnosis of RCC, which consisted of 1 papillary cell carcinoma and 36 clear cell carcinomas, 4 of which accompanied with partial sarcoma differentiation. Thirty cases were treated with first line therapy and 7 cases showed progression on first-line cytokine or sorafinib therapy. Sunitinib monotherapy was administered in repeated 6-week cycles of daily oral therapy for 4 weeks, followed by 2 weeks off in 34 patients, while another 3 patients received 37. 5 mg Qd continuously until disease progression or unacceptable toxicities occurred. Overall response rate and safety were evaluated. Results The median follow up was 12 months (8 cycles),range 1.5-19. 5 months (1-13 cycles). 26.5% (9/34) patients achieved partial responses, 70.6%(24/34) patients demonstrated stable disease over≥3 months and 1 (2. 9%) patient developed progressive disease. The objective response rate was 26.5%, and the disease control rate was 97. 1%.The 12 months' overall survival rate was 95.8% (23/24), and 12 months' progression-free survival rate was 62.5 % (15/24). The most common treatment-related adverse events were thrombocytopenia (30 cases, 81.1%), thyroid dysfunction (18/22, 81.8%) ,hand-foot syndrome (27 cases, 73.0%),neutropenia (23 cases, 62.2%) and hypertension (18 cases, 48.6%). The major grade 3 adverse events included thrombocytopenia (8 cases, 21.6%), hand-foot syndrome (4 cases, 10.8%) and diarrhea (2 cases, 5. 4%). Most adverse events were ameliorated by treatment interruption. Ten (27.0%) patients had dose decrement or drug discontinuation and 1 patient quit the treatment for intolerable fatigue. Conclusion The efficacy and manageable adverse event profile of sunitinib as a single agent in first- or second-line therapy for patients with metastatic RCC.

Clinical study of Sunitinib in the treatment of metastatic renal clear cell carcinoma: a single center 23 cases experience / 中华外科杂志

<p><b>OBJECTIVE</b>To evaluate the efficacy and safety of Sunitinib in the treatment of metastatic renal clear cell carcinoma in our institution.</p><p><b>METHODS</b>A total of 23 patients with metastatic clear cell RCC were enrolled from June 2008 to June 2009, male 16, female 7, median age 52 years. Twenty cases were treated by using Sunitinib as first-line therapy and 3 cases as second-line therapy. All pathological diagnosis was clear cell carcinoma. Sunitinib monotherapy was administered in repeated 6-week cycles of daily oral for 4 weeks, followed by 2 weeks off, until disease progression or intolerable toxicities occurred. Overall response rate and safety were evaluated.</p><p><b>RESULTS</b>The median follow-up were 7.5 months (5 cycles). Four of 23 patients (17.4%) were treated with Sunitinib achieved partial responses (PR); 18 patients (78.3%) demonstrated stable disease (SD); 1 patient (4.3%)developed progressive disease (PD) during the study. Seventeen patients received treatment over 6 months (5 cycles). The 6 months' overall survival rate was 100%, 6 months' progression-free survival rate was 88.2%. The most commonly reported grade 3 adverse events included hand-foot syndrome (13.0%), thrombocytopenia(8.7%), diarrhea (4.3%)and fatigue (4.3%). Most grade 3 adverse events were ameliorated by dose-adjustment or treatment interruption.</p><p><b>CONCLUSION</b>The results of this study demonstrate the efficacy and manageable adverse-event profile of Sunitinib as a single therapy in first-line or second-line therapy for patients with metastatic clear cell RCC.</p>

Differential transcription of Bcl-2 and Bax through the cell cycle in prostate cancer cell line / 中华外科杂志

<p><b>OBJECTIVE</b>To investigate the differential expression of apoptosis associated gene Bcl-2 and Bax through cell cycle and its possible clinical meaning.</p><p><b>METHODS</b>The prostate cancer cell line PC-3 was synchronized in M, G1, S and G2 phase using modified thymine deoxyriboside blockage and high pressure N2O technique. The efficiency of synchronization was detected by flow-cytometry. RT-PCR and Western blot methods were used to examine the expression of Bcl-2 and Bax in mRNA and protein level.</p><p><b>RESULTS</b>The synchronized rate of M, G1, S and G2 phase were 92.1%, 87.0%, 80.2% and 75.9% respectively. Bcl-2 was constitutively expressed through the cell cycle, but both the mRNA and protein expression level of Bcl-2 were very high in the G1 phase, dramatically decreased in M, S and G2 phase. The expression level of Bax had no change through the cell cycle.</p><p><b>CONCLUSIONS</b>Cell cycle could influence the expression level of Bcl-2 significantly but not Bax, these might have some clinical relevance.</p>

Relationship between von Hipple-Lindau tumor suppressor gene mutations and the prognosis of Chinese sporadic clear cell renal cell carcinoma / 中华泌尿外科杂志

Objective To evaluate the relationship between von Hippel-Lindau (VHL) gene mutations and the prognosis of Chinese sporadic clear cell renal cell carcinoma (CCRCC). Methods VHL gene mutations were detected by polymerase chain reaction (PCR), direct DNA sequencing in 74 paired specimens of sporadic CCRCC and normal kidney. The mutation findings, pathologic fea-tures and prognostic characteristics were analyzed. Results Fifty-one patients (68.9%) were in clinical stage T1, 9 patients (12.2%) were in T2 and 14 patients (18.9%) were in T3. In the aspect of pathological grade, 15 patients (20.3%) were in G1, 50 patients (67.6%) were in G2 and 9 pa-tients (12.2%) were in G3. VHL gene mutations were detected in 40 of 74 cases (54.1%). There were no significant differences of VHL gene mutations in different pathological grade and clinical stage of CCRCC, P=0.915, P=0.237, respectively. All patients were followed up for 34 to 107 months (mean 71 months). Seven patients died of CCRCC. Another 11 patients developed CCRCC related me-tastasis. Five-year tumor-free survival was 78%. The incidence of death or metastasis of CCRCC in VHL gene mutation group (15.0%, 6/40) was significant lower than non-mutation group (35.3%,12/34), P=0.043. Logistic regression results showed that lower tumor pathological grade and stage, better patients' prognosis would be (P=0.016, P=0.024, respectively). However, VHL gene muta-tion was related with better prognosis (P=0.033). This association was stronger among patients with high grade tumors (G3, P=0.048). Conclusions There are frequent VHL gene mutations in spo-radic Chinese CCRCC. Tumors' pathological grade and clinical stage are the important parameters for prognosis. However, VHL mutations are strongly associated with better cancer specific survival for CCRCC patients. These associations are relatively stronger in patients with high grade tumors.

The surgical treatment and prognosis of bilateral sporadic renal tumors / 中华外科杂志

<p><b>OBJECTIVE</b>To evaluate the surgical management and prognostic characteristics of bilateral sporadic renal tumors (BSRTs).</p><p><b>METHODS</b>Twenty-three cases of BSRTs were identified and retrospectively reviewed. The presenting clinical findings, surgical treatment, and prognosis were evaluated.</p><p><b>RESULTS</b>Twelve patients had synchronous tumors and 11 had asynchronous tumors. BSRTs comprised 1.8% of all RCC cases with a male-to-female ratio of 6.67:1 in the same term. The mean age of patients was 56 years. Seventeen patients were preformed bilateral operation (10 had synchronous and 7 had asynchronous tumors), 4 patients were preformed unilateral operation (1 had synchronous and 3 had asynchronous tumors), and 2 patients were not performed operation (asynchronous tumors). Twenty-one patients were followed up (11 had synchronous tumors and 10 had asynchronous tumors, mean 27 months). One and 5-years cancer specific survival rate of BSRTs were 84.6% and 33.5% respectively. The rate of renal failure of synchronous BSRTs (18%) was lower than that of asynchronous BSRTs (70%) after operation (P=0.030).</p><p><b>CONCLUSIONS</b>Prognosis of BSRTs is poor. The key in the treatment of BSRTs is to detect and resect tumor earlier. Our results indicate that nephron sparing surgery should be performed in order to obtain optimal functional preservation of the renal remnant with complete local excision of tumor.</p>

Treatment and prognosis of scrotal extramammary Paget's disease: a report of 23 cases / 中华男科学杂志

<p><b>OBJECTIVE</b>To evaluate the clinical manifestation, management and prognostic characteristics of scrotal extramammary Pagets disease (EMPD).</p><p><b>METHODS</b>Twenty-three cases of EMPD were identified and retrospectively reviewed, and the clinical findings, surgical treatment, pathologic features and prognostic characteristics were evaluated. The patients ranged in age from 49 to 72 years (mean 62. 4 years). The diagnoses were delayed by 12 to 132 months (67. 6 on average) in 9 cases at Stage A1 , 12 at A2, 1 at B and 1 at D. The rate of initial misdiagnosis was 91.3% (21/23). Twenty of the patients underwent operations, 2 received radiotherapy and 1 chemotherapy.</p><p><b>RESULTS</b>Twenty-two patients of the total number were followed up for 7 to 223 months, averaging at 119, therapy and 1 chemotherapy. of whom 12 remained cancer free, 1 died of tumor, 3 died of intercurrent disease, 4 experienced local recurrence, 1 relapsed with inguinal lymph node metastasis and 1 developed inguinal lymph node metastasis exclusively. Those with relapses received the second surgery, and 5 of them survived without cancer and 1 died of intercurrent disease.</p><p><b>CONCLUSION</b>The primary treatment of choice for scrotal EMPD is radical excision. Precise, histological examination before and during the operation is of crucial importance. The positive excisal margins are one of the key causes of local recurrence, and the involvement of dermis by Pagets cells may suggest possible metastasis. Scrotal EMPD tends to occur as a slowly growing lesion, mainly in the elderly, and has a good prognosis in cases of non-invasion. Otherwise the prognosis is poor.</p>

The expression of hypoxia inducible factor-1,2 alpha in sporadic clear cell renal cell carcinoma and their relationships to the mutations of von Hippel-Lindau gene / 中华外科杂志

<p><b>OBJECTIVE</b>To evaluate the expression of hypoxia inducible factor (HIF)-1alpha, 2alpha in sporadic clear cell renal cell carcinoma and their relationships to the mutations of von Hippel-Lindau (VHL) gene.</p><p><b>METHODS</b>Mutations of VHL gene, expression of HIF-1alpha and 2alpha were detected by polymerase chain reaction (PCR), direct DNA sequencing and immunohistochemistry in 77 cases of Chinese sporadic clear cell renal cell carcinoma (CCRCC). The stage was pT(1)N(0)M(0)in 55 patients (71%), pT(2)N(0)M(0) in 7 patients (9%), pT(3)N(0)M(0) in 14 patients (18%), and pT(4)N(0)M(0) in 1 patient (1%). The classification according to the tumor nuclear grading system showed 15 carcinomas (19%) of tumor nuclear grade 1, 56 (73%) of tumor nuclear grade 2 and 6 (8%) of tumor nuclear grade 3.</p><p><b>RESULTS</b>None of the VHL gene mutations were found in all the normal tissue specimens. VHL gene mutations were detected in 40 (52%) cases of CCRCC. The positive rate of HIF-2alpha (81%) was higher than that of HIF-1alpha (66%) (chi(2) = 23.310, P < 0.01); The positive rate of HIF-1alpha and HIF-2alpha in the cases of mutations (98% and 93% respectively) was higher than that of them in non-mutations (32% and 68% respectively) (chi(2) = 36.386, 7.617, P < 0.01); The correlation between HIF-1alpha and VHL gene mutations was closer than that between HIF-2alpha and VHL gene mutations (partial correlation coefficiency was 4.481 and 2.027 respectively, P < 0.01). The expression of HIF-1alpha and 2alpha in different pathological grade and stage of CCRCC showed no significant difference (P > 0.05).</p><p><b>CONCLUSIONS</b>Our study suggests that VHL gene mutations are frequent in sporadic CCRCC, and the high expression of HIF-1alpha and 2alpha are found in the group of VHL mutations. However, we have not found significant correlation between the expression of HIF-1alpha and 2alpha and pathological grade and stage of CCRCC in our study.</p>

Analysis of biallelic inactivation of the von Hippel-Lindau tumor suppressor gene VHL in patients of renal cell carcinoma patient / 中华外科杂志

<p><b>OBJECTIVE</b>To investigate biallelic inactivation of the von Hippel-Lindau tumor suppressor gene (VHL) in patient of renal cell carcinoma (RCC) patient.</p><p><b>METHODS</b>We extracted tumor and normal DNA from 41 RCC patients. Mutation of VHL gene from tumor tissue was detected from tumor tissue by polymerase chain reaction (PCR) and direct sequencing. Two single nucleotide polymorphism (SNP) sites located in VHL gene were analyzed by PCR restriction fragment length polymorphism, and loss of heterozygosity (LOH) was analyzed for VHL gene by comparing between tumor with normal tissue.</p><p><b>RESULTS</b>Mutation and LOH of VHL gene was found in 51% (21/41) and 42% (8/19) of RCC patients respectively. LOH was highly associated with mutation positive tumors (r = 0.78) and VHL biallelic inactivation was detected in 37% of RCC patients.</p><p><b>CONCLUSION</b>Biallelic inactivation of VHL gene occurs in RCC due to VHL mutation and LOH, and its frequency rate is 37%.</p>

Analysis of two single nucleotide polymorphisms in von Hippel-Lindau gene and detection of loss of heterozygosity in Chinese sporadic renal cell carcinoma / 中华医学遗传学杂志

<p><b>OBJECTIVE</b>To exam two single nucleotide polymorphism(SNP) in VHL gene and intragenic loss of heterozygosity (LOH) of VHL gene in 79 Chinese sporadic renal cell carcinomas(RCCs), and to analyze the relationships between VHL LOH and clinicopathological parameters.</p><p><b>METHODS</b>The authors extracted tumor and normal tissue DNA and detected two genotypes of intragenic SNP sites, rs779805 in the 5'terminal and rs 1642742 in the 3'terminal of VHL gene by polymerase chain reaction-restriction frament length polymorphism, then analyzed VHL LOH by comparing tumor tissue versus normal tissue in heterozygosities. Subsequently the relationships between VHL LOH and clinicopathological parameters of RCCs were analyzed.</p><p><b>RESULTS</b>The computed heritage parameters of two SNPs, included genotype frequency, allele frequency, heterozygosity, and polymorphism information content. Twenty-nine heterozygosities were detected in 79 RCCs. LOH was found in 41.4%(12/29) of RCCs. No significant relationships between VHL LOH and age, sex, tumor stage, pathological grade were found.</p><p><b>CONCLUSION</b>LOH of VHL gene is an important genetic event in Chinese sporadic renal carcinoma, and the LOH frequency is 41.4%. VHL LOH has no influence on stage and grade of RCC.</p>

Primary artery erectile dysfunction: one case report / 中华男科学杂志

<p><b>OBJECTIVE</b>To evaluate the relationship between the deformation of penile artery and the primary artery erectile dysfunction, and to improve the treatment and diagnosis of primary artery erectile dysfunction.</p><p><b>METHODS</b>One case of primary artery erectile dysfunction was presented with its primary clinic data.</p><p><b>RESULTS</b>The dorsal artery of the penis was thin and the bilateral penile arteries were lacking by arteriography. The implantation of a penile prosthesis significantly improved the patient's erectile function.</p><p><b>CONCLUSION</b>The primary artery erectile dysfunction is a relatively rare disease. The possibility of primary artery erectile dysfunction should be kept in mind. Penile prosthesis implantation is an effective means for the treatment of primary artery erectile dysfunction.</p>

Report of a case of penile epithelioid sarcoma / 中华男科学杂志

<p><b>OBJECTIVE</b>To enhance the knowledge and the effect of the diagnosis and treatment of primary epithelioid sarcoma of the penis.</p><p><b>METHODS</b>One rare case of primary epithelioid sarcoma of the penis was studied with regard to its primary clinical process and characteristics, differential diagnosis and method of treatment.</p><p><b>RESULTS</b>An operation was performed on the penis to treat the epithelioid sarcoma. The diagnosis was confirmed by immunohistological and pathological techniques. There was no evidence of relapse during the three-year follow-up after operation.</p><p><b>CONCLUSION</b>The possibility of primary epithelioid sarcoma of the penis should be considered if a mass or induration of the proximal penis and the symptoms of urethremphraxis are found. Total phallectomy could be chosen as an appropriate method of treatment. Unless adenopathy is palpable, node dissections are not recommended.</p>