Sum of High-Risk Gene Mutation (SHGM): A Novel Attempt to Assist Differential Diagnosis for Adrenocortical Carcinoma with Benign Adenoma, Based on Detection of Mutations of Nine Target Genes.

There has been no research on applying gene detection to differential diagnosis of adrenocortical carcinoma (ACC). We attempted to explore a novel auxiliary method for differential diagnosis between ACC with benign adrenocortical adenoma (ACA), based on mutations of target genes in tissues. Nine genes were chosen as target genes, including TP53, CTNNB1, ARMC5, PRKAR1A, ZNRF3, RB1, APC, MEN1, and RPL22. Exons sequencing of target genes were performed in 98 cases of tissue samples by FastTarget technology, including 41 ACC tissues, 32 ACA tissues, and 25 normal adrenal gland tissues. Significant mutations were detected and identified, and the clinical information was collected, for further comparative analysis and application to assist differential diagnosis of ACC. We identified 132 significant gene mutations and 227 significant mutation sites in 37 ACC tissues, much more than ACA and normal adrenal gland tissues. Mutation rates of 6 genes in ACC tissues were obviously higher than ACA tissues, including ZNRF3, ARMC5, TP53, APC, RB1, and PRKAR1A, regarded as high-risk genes. The sum of mutated high-risk genes detected in each sample was denominated sum of high-risk gene mutation (SHGM), and the rates of SHGM > 0 and SHGM > 1 in ACC tissues were 73.0% and 62.2%, respectively, both obviously higher than those in ACA tissues, with significant statistic differences. Especially for 8 cases of ACC with diameter < 5 cm, SHGM > 0 and SHGM > 1 were found in 6 samples (75%) and 4 samples (50%), respectively. However, no relevance was found between SHGM and clinical characteristics of ACC. We identified 6 high-risk genes in ACC tissues, with significantly higher mutation rates than ACA or normal adrenal gland tissues. The sum of mutated high-risk genes detected in ACC tissues was denominated SHGM, which was potential to assist the differential diagnosis of ACC with ACA, especially for the small-size ACC.

Subclinical left ventricular systolic dysfunction detected by two-dimensional speckle tracking echocardiography in patients with pheochromocytoma and paraganglioma and preserved ejection fraction.

BACKGROUND: Excessive catecholamine leads to pressure overload and left ventricular (LV) remodeling. The goal of this study was to explore subclinical LV systolic dysfunction and the mechanism of preserved left ventricular ejection fraction (LVEF) in patients with pheochromocytoma and paraganglioma using two-dimensional speckle tracking echocardiography. METHODS: A total of 48 patients with pheochromocytoma and paraganglioma and preserved LVEF and 38 age- and gender-matched volunteers were studied. Echocardiographic parameters including LVEF, and global peak longitudinal and circumferential strains were measured. The correlation between echocardiographic parameters and blood pressure as well as biochemical parameters was analyzed. RESULTS: LVEF was similar between patients with pheochromocytoma and paraganglioma and controls. The amplitude of LV longitudinal strain was decreased, and the amplitude of LV circumferential strain was increased in the pheochromocytoma and paraganglioma group (P = .003 and P = .009). LV mass index and blood pressure were positively correlated with 24-hour urinary norepinephrine (r = .696, P < .0001; r = .470, P = .0007). The amplitude of LV longitudinal strain reduced with increase in blood pressure, 24-hour urinary norepinephrine and LV mass index (r = -.305, P = .035; r = -.506, P = .0002; r = -.680, P < .0001). CONCLUSIONS: This study revealed that excessive norepinephrine in pheochromocytoma and paraganglioma was associated with increased blood pressure and LV mass. The LV longitudinal strain was decreasing with increase in blood pressure and LV mass index. The enhanced LV circumferential strain might be the mechanism of compensation to maintain the normal LVEF in these patients.

Preliminary Application of WCX Magnetic Bead-Based Matrix-Assisted Laser Desorption Ionization Time-of-Flight Mass Spectrometry in Analyzing the Urine of Renal Clear Cell Carcinoma.

Objective To evaluate the application of weak cation exchange (WCX) magnetic bead-based Matrix-Assisted Laser Desorption Ionization Time-of-Flight Mass Spectrometry (MALDI-TOF MS) in detecting differentially expressed proteins in the urine of renal clear cell carcinoma (RCCC) and its value in the early diagnosis of RCCC.Methods Eleven newly diagnosed patients (10 males and 1 female, aged 46-78, mean 63 years) of renal clear cell carcinoma by biopsy and 10 healthy volunteers (all males, aged 25-32, mean 29.7 years) were enrolled in this study. Urine samples of the RCCC patients and healthy controls were collected in the morning. Weak cation exchange (WCX) bead-based MALDI-TOF MS technique was applied in detecting differential protein peaks in the urine of RCCC. ClinProTools2.2 software was utilized to determine the characteristic proteins in the urine of RCCC patients for the predictive model of RCCC. Results The technique identified 160 protein peaks in the urine that were different between RCCC patients and health controls; and among them, there was one peak (molecular weight of 2221.71 Da) with statistical significance (P=0.0304). With genetic algorithms and the support vector machine, we screened out 13 characteristic protein peaks for the predictive model. Conclusions The application of WCX magnetic bead-based MALDI-TOF MS in detecting differentially expressed proteins in urine may have potential value for the early diagnosis of RCCC.

Human urothelial carcinoma cell response to Sunitinib malate therapy in vitro.

OBJECTIVES: Bladder transitional cell carcinoma (TCC) is one of the most common solid malignancies in China. This study examined the antitumor effect and underlying mechanism of action of sunitinib malate in human bladder TCC in vitro. METHODS: Bladder TCC cell lines 5637 and BIU87 were maintained in 1640 medium and T24 cell lines in DMEM/F12 medium. All 3 cell lines were then exposed to graded concentrations (0.625-20 µmol/L) of sunitinib malate, sorafenib and cisplatin for 24-96 hours to determine the sensitivities to each drug. Cell viability was measured by the MTT [3-(4,5-dimethylthiazol-2-yl)-2,5-diphenyltetrazolium] assay, and apoptosis was analyzed by flow cytometry. Cell apoptotic morphology was observed by a fluorescence microscope after DAPI (4',6-diamidino-2-phenylindole) staining. Protein concentrations were measured by western blot. RESULTS: Sunitinib malate showed a concentration-dependent inhibitory effect on the 5637, T24 and BIU87 cell lines with IC50's of 1.74 µmol/L, 4.22 µmol/L, and 3.65 µmol/L, respectively. Cisplatin also exhibited good antitumor activity, but whereas sorafenib suppressed proliferation of the cells at concentrations of 10 µmol/L or higher, there was practically no response at lower concentrations. Sunitinib malate treatment resulted in an accumulation of cells in the sub-G1 phase, especially with the T24 and BIU87 cell lines, which induced apoptosis of the cells. CONCLUSIONS: Sunitinib malate exerted marked inhibitory activity against bladder cancer cells. The cell growth inhibitory effect of the drug was related to induction of apoptosis. These results suggest that clinical application of sunitinib-based therapy for advanced bladder cancer is possible.

Clinical features and retroperitoneal laparoscopic resection of adrenal schwannoma in 19 patients.

OBJECTIVE: To explore the diagnostic and therapeutic principles of the rare adrenal schwannoma. METHODS: We objectively analyzed the detailed clinical data of 19 patients with adrenal schwannoma treated in a large comprehensive medical center in China between January 2009 and March 2014, including general information, clinical manifestations, imaging, adrenal-related endocrine tests, treatment, pathology, and follow-up records. RESULTS: Patients included 16 females and 3 males ranging from 23 to 66 years of age (mean, 48.3 ± 12.6 years), of whom 15 were discovered incidentally. Four patients reported mild abdominal discomfort. Each patient had one unilateral lesion, ranging in diameter from 4.0 to 8.8 cm (mean, 6.2 ± 1.2 cm). Lesions were hypoechoic in 16 patients and cystic-solid in 3 patients. Computed tomography (CT) scanning revealed soft tissue density, mostly with calcifications and clear boundaries. Average values in normal and enhanced CT were 28.7 ± 7.2 Hounsfield units (HU) and 59.7 ± 5.4 HU, respectively. Indicators of adrenal-related endocrine tests were normal, except that catecholamines levels were slightly low in 2 patients with excessively large lesions. All patients underwent uncomplicated retroperitoneal laparoscopic surgery, with subsequent pathology confirmation of adrenal schwannoma. The patients have been followed for 6 months to 4 years, with no recurrence. CONCLUSION: Our results demonstrate that the onset of adrenal schwannoma is always occult, without typical clinical manifestations and adrenal-related endocrine abnormalities, and the pre-operative diagnosis depends primarily on imaging features of hypoechoic calcifications and mild enhancement. Diagnosis can be confirmed by postoperative pathology. Even with large diameter lesions, retroperitoneal laparoscopic surgery is still the preferred approach, with good prognosis.

Effects of sunitinib malate on growth of human bladder transitional cell line T24 in vitro.

OBJECTIVE: To investigate the growth-inhibitory effect of sunitinib malate on human bladder transitional cell carcinoma (TCC) in vitro. METHODS: Human bladder TCC cell line T24 was cultured and exposed to graded concentrations of sunitinib malate for 72 hours in vitro to determine the sensitivities to drug. Cell viability was measured by MTT assay. Cell apoptotic morphology was observed by fluorescence microscope following DAPI staining. Band expressions of Fas, Fas ligand, poly (ADP-ribose) polymerase (PARP) and ß-actin were analyzed by Western blot. Wound healing process of T24 cells exposed to sunitinib malate was assayed. RESULTS: Sunitinib malate exerted a concentration-dependent and time-dependent inhibitory effect on the T24 cell lines. Fluorescence microscopy showed that small vacuoles appeared in the nuclei of T24 cells and the vacuoles were bigger with higher drug concentrations. The expressions of Fas ligand and PARP in T24 cells treated with sunitinib malate exhibited a concentration-dependent increase. Moreover sunitinib malate suppressed the wound healing process in a concentration-dependent manner. CONCLUSION: Sunitinib malate exerted marked inhibitory activity against bladder cancer cell line T24.

Outcomes of T3a Prostate Cancer with Unfavorable Prognostic Factors Treated with Brachytherapy Combined with External Radiotherapy and Hormone Therapy.

OBJECTIVE: To evaluate the outcomes of T3a prostate cancer with unfavorable prognostic factors treated with permanent interstitial brachytherapy combined with external radiotherapy and hormone therapy. METHODS: From January 2003 to December 2008, 38 patients classified as T3a prostate cancer with unfavorable prognostic factors were treated with trimodality therapy (brachytherapy + external radiotherapy + hormone therapy). The prescription dose of brachytherapy and external radiotherapy were 110 Gy and 45 Gy, respectively. The duration of hormone therapy was 2-3 years. The endpoints of this study included biochemical failure-free survival (BFFS), distant metastasis-free survival (DMFS), cancer-specific survival (CSS), and overall survival (OS). Survival curves were calculated using the Kaplan-Meier method. The Log-rank test was used to identify the prognostic predictors for univariate analysis. RESULTS: The median follow-up was 71 months. The serum pre-treatment prostate-specific antigen (PSA) level ranged from 10.0 to 99.8 ng/ml (mean 56.3 ng/ml), the Gleason score ranged from 5 to 9 (median 8), and the percentage of positive biopsy cores ranged from 10% to 100% (mean 65%). The 5-year BFFS, DMFS, CSS, and OS rates were 44%, 69%, 82%, and 76%, respectively. All biochemical failures occurred within 40 months. The percentage of positive biopsy cores was significantly correlated with BFFS, DMFS, and OS (all P=0.000), and the Gleason score with DMFS (P=0.000) and OS (P=0.001). CONCLUSIONS: T3a prostate cancer with unfavorable prognostic factors presents not so optimistic outcome. Hormone therapy should be applied to prolong the biochemical progression-free or metastasis-free survival. The percentage of positive biopsy cores and the Gleason score are significant prognostic factors.

Clinical Characteristics and Outcome of Gleason Score 10 Prostate Cancer on Core Biopsy Treated by External Radiotherapy and Hormone Therapy.

OBJECTIVE: To evaluate the clinical characteristics and outcomes of patients with Gleason score 10 prostate cancer treated by external radiotherapy and hormone therapy. METHODS: From January 2003 to March 2014, 1832 patients with prostate cancer were treated, among which 9 patients (represented 0.49%) were identified as Gleason score 10 disease on prostate core biopsy without distant metastases when first diagnosed. All 9 patients were treated by whole pelvic external radiotherapy (The whole pelvic dose was 50.0 Gy and the boost dose ranged from 76.2 to 78.0 Gy) and long-term hormone therapy. We assessed the clinical characteristics, treatment outcomes and treatment toxicities. Survival curves were calculated using the Kaplan-Meier method. RESULTS: The median follow-up was 4.8 years. Six patients' pre-treatment prostate-specific antigen (PSA) levels were lower than 20.0 µg/L and three patients' pre-treatment PSA levels were higher than 70.0 µg/L. The median percentage of positive biopsy cores was 91%. Three, four and two cases were classified as T2c, T3a and T3b stage, respectively. Three cases were assessed as N1 stage. The 5-year biochemical failure-free survival, distant metastasis-free survival, cancer specific survival and overall survival rates were 28.6%, 57.1%, 66.7% and 57.1%, respectively. Five patients experienced grade 1-2 acute gastrointestinal toxicities and six patients complained of grade 1-2 acute genitourinary toxicities. No bone fracture or cardiovascular disease was detected. CONCLUSIONS: Gleason score 10 prostate cancer on core biopsy is usually combined with other high risk factors. The pre-treatment PSA levels lie in two extremes. Timely and active treatments are urgent needed because unfavourable oncological outcomes are often presented.

[Prognostic factors and associated models for metastatic renal cell carcinoma treated with targeted therapy].

Since 2006, tyrosine kinase inhibitors and anti-angiogenic drugs have revolutionized the treatment of metastatic renal cell carcinoma by improving progression-free survival and overall survival. The prognostic factors in metastatic renal cell carcinoma treated by targeted therapy include anatomical, histological, clinical, biological, and molecular parameters. The accuracy of these prognostic factors are not high when applied alone. A renal cancer prognostic system that combines all these prognostic factors can improve the risk assessment of renal cancer and prognosis prediction, and thus guide clinical decision-making.

[Clinical character of metanephric adenoma of the kidney: a case report].

To investigate the clinicopathological characteristics of metanephric adenoma (MA), we analyzed the clinical and pathological data of metanephric adenoma. A 39-year old woman with asymptomatic right kidney tumor for 4 years was admitted to the hospital. A tumor with the largest diameter of 28 mm in the right kidney was homogeneously enhanced on CT. The tumor was distinctly increased as compared with 4 years before. The diagnosis was right kidney tumor. Nephron-sparing surgery was given after general anaesthesia. And the relative literature was reviewed. The tumor was homogeneous, with integrity tegument, and a grey cutting surface. Histopathologically, the tumor cells formed an adenoid or papillary pattern and contained psammoma bodies. Immunohistologically, they were positive for AE1/AE3, vimentin and WT1, negative for CK7, EMA and RCC. Pathological diagnosis was metanephric adenoma. The follow-up data of 24 months were available and without recurrence. MA is peculiar. It is difficult to get the final diagnosis of MA only by imaging characters. Nephron-sparing surgery is eligible for the treatment of MA. Considering the uncertainty of the biological behavior and cellular origin of MA, a long-term active surveillance is necessary.

[Perioperative treatment of patients with pheochromocytoma combined severe catecholamine cardiomyopathy: 5 cases report].

OBJECTIVE: To discuss perioperative treatment and cardiac function changes after medicine and surgical treatment of pheochromocytoma patients with severe catecholamine cardiomyopathy. METHODS: Five pheochromocytoma patients with severe catecholamine cardiomyopathy in our hospital for the past 5 years were studied, their general characteristics, clinical manifestations, diagnosis tests summarized and the cardiac function changes before and after the treatment evaluated. RESULTS: All the 5 patients were diagnosed definitely before operation. After medication, left ventricular ejection fraction (LVEF) of the 5 patients improved and 4 patients' LVEF increased 7-10 days after operation compared with after medication, and one patient's LVEF improved after 3 months. CONCLUSION: Pheochromocytoma patients with severe catecholamine cardiomyopathy should have adequate medication, and their abnormal cardiac function would be reversed after surgical treatment.

[Complications of laparoscopic surgery: a 11-year single institute experience].

OBJECTIVE: To analyze the complications of laparoscopic surgery for the past 11 years in the Urology Department of Peking Union Medical College Hospital. METHODS: The laparoscopic surgery of 5 386 cases from Jan. 2002 to Dec. 2012 was statistically analyzed. The patient's gender, age, surgery mode, blood loss, complications, total length of hospital stay, postoperative hospital stay and other data were analyzed. RESULTS: There were 5 386 cases in this group aged 8-86 years (the mean age: 49.0 years; 2 651 cases were male and 2 735 female). Of all the cases, 2 411 were of adrenal surgery, accounting for 44.7%, 167 of retroperitoneal tumor resection , accounting for 3.1%, 763 of radical nephrectomy surgery, accounting for 14.2%, 441 of partial nephrectomy surgery, accounting for 8.2%, 784 of renal cyst surgery, accounting for 14.6%; 279 of semi-urinary tract resection, accounting for 5.2%; 74 of renal pelvis and ureter plasty, accounting for 1.4%; 141 of radical prostatectomy surgery, accounting for 2.6%, 71 of cystectomy surgery, accounting for 1.3% and 255 of other surgery, accounting for 4.7%. The blood loss was 10-3 000 mL (average: 97.5 mL ) and the surgery requiring blood transfusion treatment involved 270 patients. The total hospital stay was 3-133 days (average: 18 days) and postoperative hospital stay was 2-35 days (average: 6.3 days). All kinds of complications occured in 346 cases (6.42%), of which 198 were of peritoneal injury, 12 of pleural injury; 72 of subcutaneous emphysema, 24 of urinary fistula, 15 of inferior vena cava injury, 6 of pancreatic fistula 10,of lymphatic fistula, 8 of wound delayed healing and 1 of, intestinal fistula. The incidence of the complications and blood transfusion showed a downward trend according to stratified analysis each year. CONCLUSION: The laparoscopic surgery coveres almost all treatment of urological diseases. The complications are different in different types of surgery at different times, but generally no serious complications occurr. The incidence of complications is in a downward trend as the laparoscopic technique level improves.

[Clinical analysis of tuberous sclerosis complex complicated with renal angiomyolipoma: a report of 22 cases].

OBJECTIVE: To explore the diagnosis and treatment of tuberous sclerosis complex complicated with renal angiomyolipoma. METHODS: The clinical data of 22 patients with tuberous sclerosis complex complicated with renal angiomyolipoma were analyzed retrospectively. RESULTS: There were 12 males and 10 females with a mean age of 23 (1-46) years. All of them had bilateral multiple renal angiomyolipomas. The mean tumor size was 8.5 (0.7-18.0) cm in diameter. The presence or absence of clinical symptoms showed a significant correlation with tumor size.Eight patients with angiomyolipoma under 4 cm in diameter were continuously monitored at an outpatient clinic. There were 8 patients with lesions of 4-10 cm.Five of them underwent nephron-sparing surgeries and another 3 monitored at an outpatient clinic. There were 6 patients with tumor over 10 cm. Three of them underwent transcatheter arterial embolization and one case took rapamycin after embolization.One patient with concurrent renal cell carcinoma underwent partial nephrectomy. Chronic renal failure occurred in one case dying of pulmonary lymphangiomyomatosis with serious hemoptysis. During a mean follow-up of 25 months, neither severe renal hemorrhage nor symptomatic aggravation was found.In the case of rapamycin, there was 10% reduction in the size of angiomyolipoma. CONCLUSIONS: Most cases of tuberous sclerosis complex are complicated with bilateral multiple renal angiomyolipoma. The small lesions under 4 cm in diameter may be monitored at an outpatient clinic.For those larger (>4 cm) or symptomatic ones, medication, embolization or surgery is necessary.

[Clinical diagnosis and treatment of spontaneous urinary extravasation caused by primary lower ureteral tumor].

OBJECTIVE: To explore the clinical characteristics of spontaneous urinary extravasation caused by primary lower ureteral cancer. METHODS: Eight cases of spontaneous urinary extravasation caused by the obstruction of primary ureteral cancer from January 2005 to June 2010 from Department of Urology, Peking Union Medical College Hospital, were reported. There were 5 males and 3 females with an age range of 56-81 years old. Six cases presented with an onset of acute flank pains and 2 cases complained of flank discomforts with a lower fever. All cases had peripelvic fluid collection of varying levels on computed tomography (CT) scan and extravasation of contrast medium was found in delayed enhanced CT. The tumors of ureter were detected in 6 cases through preoperative imaging examinations while another 2 cases had no evidence of tumor. RESULTS: Unilateral nephroureterectomy was performed in 6 cases, including 5 cases undergoing retroperitoneal laproscopic surgery and 1 case with open surgery. In 5 laproscopic operations, the mean operative duration was 152 (120 - 235) minutes and the mean estimated intraoperative blood loss 130 (100 - 430) ml. The patient of open nephroureterectomy had an operative duration of 175 minutes and an intraoperative blood loss of 200 ml respectively. One patient underwent a local resection of ureteral carcinoma. A 81-year-old patient was diagnosed of tumor by ureteroscopic biopsy and accepted neither surgery or adjunctive therapy. All patients had a pathological diagnosis of urothelial carcinoma. One patient received local radiotherapy and chemotherapy with gemcitabine after nephroureterectomy while another one had local radiotherapy only. During a follow-up period of 6-36 months, CT showed that five cases undergoing nephroureterectomy were free of recurrence. One patient with local lymphatic metastasis had a local recurrence, developed multiple metastases at Month 3 and died of exhaustion at Month 8 post-operation. The patient with a local resection had a local bladder recurrence at Month 6 and underwent unilateral nephroureterectomy and radical cystectomy after local radiotherapy and neoadjuvant chemotherapy. Tumor progression was found in the patient without surgery. No implantation metastasis was found. CONCLUSIONS: Spontaneous urinary extravasation caused by the obstruction of primary lower ureteral cancer is rare and mostly peripelvic. Biopsy may exclude tumors for those patients with no obvious cause of ureteral obstruction. And radical operation is a preferred option.

[Expression of SDHB, EPAS1 and MIB-1 in Zuckerkandl paragangliomas].

OBJECTIVE: To explore the significance of succinate dehydrogenase B (SDHB) mutation and EPAS1 overexpression in Zuckerkandl paragangliomas (PGL) and examine their correlations with malignant infiltration and metastasis. METHODS: From March 2008 to July 2011, the clinical profiles of 16 Zuckerkandl PGL patients were analyzed retrospectively. For increased diagnostic specificity, a complex immunohistochemical panel of tissue microarray was performed for SDHB, EPAS1 and MIB-1. Positive expression identified as a granular cytoplasmic staining. Greater than or equal to 50% as strongly positive (+++), 1% to 10% as weakly positive (+). RESULTS: Tissue microarray immunohistochemical staining showed SDHB immunoreactivity in the cytoplasm, whereas EPAS1 and MIB-1 in the nuclear of tumor cells. Positive expression of EPAS1 in which 13 cases of Zuckerkandl PGL. And high expression strongly associated with malignancy. SDHB mutation of 7 cases are all EPAS1 positive staining. Non-gene mutation 9 cases tumor specimens, 6 cases were EPAS1 positive expression (P < 0.05). CgA positive expression in 11 cases benign Zuckerkandl PGL, strongly positive in 4 malignant cases (4/4). MIB-1 below 1% in 12 cases of benign Zuckerkandl PGL. And in 4 malignant cases, MIB-1 was about 3%. Malignant neoplasms had significantly higher EPAS1, CgA and MIB-1 expression compared to benign counterparts (P < 0.05). CONCLUSIONS: The SDHB mutation causes the EPAS1 over expression in PGL and correlation with higher positive expression of CgA and MIB-1. It may be one of the mechanisms of malignant invasiveness and metastasis in PGL.

Rare case of reninoma with double inferior vena cava.

A 16-year-old boy suffered from headaches and dizziness for 2 years. He was found to have remarkably elevated blood pressure (BP) of 180/110 mmHg. Laboratory findings showed a low level of serum potassium and markedly increased plasma renin activity. A solid mass at the periphery of the right kidney and double inferior vena cava (IVC) were detected by abdominal computer tomography (CT). Right partial nephrectomy via laparoscopy was performed on the patient. The histologic and electron microscopic findings comfirmed a diagnosis of juxtaglomerlar cell tumor. The patient had no headache or dizziness with normal BP after surgery.

Diagnosis and treatment of primary adult renal sarcoma.

OBJECTIVE: To investigate the clinical characteristics of primary adult renal sarcoma. METHODS: A total of 1654 cases with adult renal tumors were treated during 1985 to 2009 in Peking Union Medical College Hospital. Of all, 17 cases were diagnosed as primary renal sarcoma and underwent radical nephrectomy. The clinical features of 17 such patients were retrospectively analyzed. RESULTS: The first symptom of 10 (59%) cases in all renal sarcomas was abdominal mass. The pathological diagnosis was leiomyosarcoma (7 cases), rhabdomyosarcoma (2 cases), malignant fibrous histiocytoma (2 cases), low-differentiated sarcoma (2 cases), chromophobe renal cell carcinoma coexisting with liposarcoma (1 case), fibrosarcoma (1 case), embryonic sarcoma (1 case) and leiomyosarcoma (1 case). One patient died of tumor thrombus of the inferior vena cava during surgery. Finally, 15 cases were regularly followed up for 4 to 60 months. Till now, 1 had tumor-free survival for 9 months, and the other 14 cases died 2-38 months after the operation with a median survival time of 18 (range, 5-60) months. The median survival time of leiomyosarcoma group was 28 (range, 11-60) months, and 2 cases of malignant fibrous histiocytoma died 4 and 8 months after the operation respectively. CONCLUSIONS: The primary renal sarcoma has the clinical symptom similar with advanced renal cell carcinoma and has poor prognosis. Leiomyosarcoma might have relative good prognosis.

[Diagnosis and treatment for adrenal ganglioneuroma].

OBJECTIVE: To evaluate the clinical characteristics,diagnosis and treatment for adrenal ganglioneuroma. METHODS: Thirty cases of adrenal ganglioneuroma at our hospital from 1999 to 2010 were reviewed. Their clinical, radiological, laboratory and pathologic findings as well as follow-up data were analyzed retrospectively. RESULTS: There were 12 males and 18 females with an age range of 7 - 63 (23.8 ± 11.4) years old. Tumor size ranged from 2.5 cm to 15 cm. Fourteen (46.7%) cases were found incidentally without any overt symptom. And the presenting symptoms and signs of the others were paroxysmal headache and palpitation (n = 7, 23.3%), hypertension (n = 5, 16.7%) and flank or abdominal pain (n = 4, 13.3%). All cases underwent open or laproscopic operations and their diagnosis of adrenal ganglioneuroma was confirmed pathologically. An oval or crescentic well-defined mass, low or intermediate attenuation on CT (computed tomography) scan was found in most cases. Tumors partially or completely surrounded major blood vessels in 9 cases while 7 cases had discrete punctuate calcification. The 24-hours urinary catecholamine and urinary free cortisol output were within a normal range. Seventeen cases underwent a (99)Tc-octreotide scan and negative results were found in 15 cases. Seven cases had a (131)I-Metaiodobenzylguanidine ((131)I-MIBG) scan and all results were negative. Twenty-one cases were diagnosed empirically as adrenal ganglioneuroma. One case was found pre-operatively with multiple bone metastases. Among all cases, either open operation (n = 11) or retroperitoneal laparoscopic excision (n = 19) was performed successfully. All recovered smoothly. One patient with pre-operative bone metastasis developed progression. No recurrence was detected after a follow-up period of 1 - 11 years. CONCLUSION: Mostly benign in nature, adrenal ganglioneuromas is often found incidentally without specific clinic symptoms. A relatively accurate preoperative diagnosis can be made on the basis of a detailed history and the relevant imaging and laboratory results. Surgical resection of tumor is a preferred choice with a fairy good prognosis.

[Vasculogenic mimicry and mosaic vessels and targeted therapy in renal cell carcinoma].

Renal cell carcinoma is one of the most common malignant tumors of urinary system. The annual incidence rate is approximately 17.9/100 000 populations, and there is a continually rising trend in number of new diagnosis. Metastatic and high-risk renal cell cancer is associated with a poor prognosis and is resistant to traditional chemotherapy and/or radiotherapy. Although cytokine-based therapies (interferon and interleukin-2) have been widely used, their effectiveness remained unsatisfactory due to their low response rates and short survival. Drugs targeting anti-angiogenesis pathways have shown benefits in relapse-free survival. In this review, we introduce the recent advances in the treatment of renal cancer, especially the application of vasculogenic mimicry and mosaic vessels. Although targeted therapies with anti-angiogenic properties have proposed new treatment criteria for advanced renal cell carcinoma, new drugs or new combinations are needed to improve the clinical efficacy and minimize adverse effects.