The oncologic impact of a delay between diagnosis and radical nephroureterectomy due to diagnostic ureteroscopy in upper urinary tract urothelial carcinomas: results from a large collaborative database.

OBJECTIVES: According to the current upper urinary tract urothelial carcinomas (UTUC) guidelines, ureteroscopic evaluation (URS) is recommended to improve diagnostic accuracy and obtain a grade (by biopsy or cytology). However, URS may delay radical surgery [e.g., nephroureterectomy (RNU)]. The objective of this study was to evaluate the influence of URS implementation before RNU on patient survival. METHODS: A French multicentre retrospective study including 512 patients with nonmetastatic UTUC was conducted between 1995 and 2011. Achievement of ureteroscopy (URS), treatment time (time between imaging diagnosis and RNU), tumour location, pT-pN stage, grade, lymphovascular invasion (LVI) and the presence of invaded surgical margins (R+) were evaluated as prognostic factors for survival using univariate and multivariate Cox regression analyses. Cancer-specific survival (CSS), recurrence-free survival (RFS) and metastasis-free survival (MFS) were calculated using the Kaplan-Meier method. RESULTS: A total of 170 patients underwent ureteroscopy prior to RNU (URS+ group), and 342 did not undergo URS (URS-). The median treatment time was significantly longer in the URS+ group (79.5 vs. 44.5 days, p = 0.04). Ureteroscopic evaluation was correlated with ureteral location and lower stage and tumour grade (p = 0.022, 0.005, 0.03, respectively). Tumour stage, LVI+ and R+ status were independently associated with CSS (p = 0.024, 0.049 and 0.006, respectively). The 5-year CSS, RFS and MFS did not differ between the two groups (p = 0.23, 0.89 and 0.35, respectively). These results were confirmed for muscle-invasive (MI) UTUC (p = 0.21, 0.44 and 0.67 for CSS, RFS and MFS, respectively). CONCLUSIONS: Despite the increased time to radical surgery, diagnostic ureteroscopy can be systematically performed for the appraisal of UTUC to refine the therapeutic strategy without significantly affecting oncological outcomes, even for MI lesions.

Assessment of oncologic control obtained after open versus laparoscopic nephroureterectomy for upper urinary tract urothelial carcinomas (UUT-UCs): results from a large French multicenter collaborative study.

BACKGROUND: The purpose of this study was to compare the postsurgical survival of UUT-UC patients treated with ONU and LNU. METHODS: Using a multi-institutional, national, retrospective database, we identified patients with UUT-UC who underwent radical nephroureterectomy by open access (ONU) or by the minimally invasive alternative (LNU). Survival curves were estimated using Kaplan-Meier method. A multivariate Cox model was used to evaluate the association between surgical approach and disease recurrence. RESULTS: Overall, 609 patients were included (ONU = 459 and LNU = 150). The median age was 69.8 years (range 61.9-76), and the male-to-female ratio was 2:1. Postoperative complications occurred in 80 patients, with no significant difference between ONU and LNU on the whole (P = 0.64). The median follow-up was 27 months. There was no difference between the 2 procedures in the 5-year CSS or 5-year RFS. Moreover, the 5-year CSS (P = 0.053) and 5-year RFS (P = 0.9) for cases with locally advanced disease (pT3/pT4) were similar between ONU and LNU. In the multivariate analysis, the surgical procedure used was not found to be associated with survival. The main limitation of the study is its retrospective design, which is the result of the rarity of the disease. CONCLUSIONS: There is no evidence that oncological outcomes for LNU are inferior to those for open surgery, provided that the appropriate precautionary measures are taken.

A proportion of hereditary upper urinary tract urothelial carcinomas are misclassified as sporadic according to a multi-institutional database analysis: proposal of patient-specific risk identification tool.

UNLABELLED: What's known on the subject? and What does the study add? Hereditary non-polyposis colorectal cancer (HNPCC), also known as Lynch syndrome, is an autosomal dominant multi-organ cancer syndrome. Upper urinary tract urothelial carcinomas belong to HNPCC-related tumours and rank third within this group after colorectal and endometrial cancer. However, many urologists are not aware of this association and it is presumed that some hereditary cancers are misclassified as sporadic and that their incidence is underestimated. Consequently, family members of patients with upper urinary tract urothelial carcinomas secondary to HNPCC may be denied appropriate surveillance and early detection. A significant proportion of patients (21.3%) with newly diagnosed upper urinary tract urothelial carcinomas may have underlying HNPCC. Demographic and epidemiological characteristics suggest different mechanisms of carcinogenesis among this population. Recognition of such potential is essential for appropriate clinical and genetic management of patients and family. In order to help to identify these patients, we propose a patient-specific checklist. OBJECTIVE: • To identify, based on previously described clinical criteria, hereditary upper urinary tract urothelial carcinomas (UUT-UCs) that are likely to be misclassified as sporadic although they may belong to the spectrum of hereditary non-polyposis colorectal cancer (HNPCC) associated cancers. PATIENTS AND METHODS: • We identified, using established clinical criteria, suspected hereditary UUT-UC among 1122 patients included in the French national database for UUT-UC. • Patients were considered at risk for hereditary status in the following situations: age at diagnosis <60 years with no previous history of bladder cancer; previous history of HNPCC-related cancer regardless of age; one first-degree relative with HNPCC-related cancer diagnosed before 50 years of age or two first-degree relatives diagnosed regardless of age. RESULTS: • Overall, 239 patients (21.3%) were considered to be at risk of hereditary UUT-UC. • Compared with sporadic cases, hereditary cases are more likely to be female (P= 0.047) with less exposure to tobacco (P= 0.012) and occupational carcinogens (P= 0.037). A greater proportion of tumours were located in the renal pelvis (54.5% vs 48.4%; P= 0.026) and were lower grade (40% vs 30.1%; P= 0.015) in the hereditary cohort. • The overall, cancer-specific and recurrence-free survival rates were similar in both cohorts. • We propose a patient-specific risk identification tool. CONCLUSIONS: • A significant proportion (21.3%) of patients with newly diagnosed UUT-UC may have underlying HNPCC as a cause. • Recognition of such potential and application of a patient-specific checklist upon diagnosis will allow identification and appropriate clinical and genetic management for patient and family.

Active Surveillance for Biopsy Proven Renal Oncocytomas: Outcomes and Feasibility.

OBJECTIVES: To report the outcomes and feasibility of active surveillance (AS) of biopsy-proven renal oncocytomas. METHODS: Multicentric retrospective study (2010-2016) in 6 academic centers that included patients with biopsy-proven renal oncocytomas who were allocated to AS (imperative or elective indication) with a follow-up ≥1 year. Imaging was performed at least once a year, by CT-scan or ultrasound or MRI. Conversion to active treatment (surgical excision or ablative treatment) was at the discretion of the urologist. The primary endpoint was renal tumor growth (cm/year). Secondary outcomes included accuracy of biopsy, incidence, and reason to change AS to active treatment. RESULTS: Eighty-nine patients were included: Median age 67 years (26-89) and median tumor size 26 mm [15-90] on diagnosis. During a mean follow-up of 43 months'' (median 36 [12-180]), mean tumor growth was 0.24 cm/year. No predictive factors (demographical, radiological or histologic) of tumor growth could be identified. Conversion from AS to active treatment occurred in 24 patients (27%) (13 surgical excisions, 11 ablative procedures), in a median time of 45 (12-76) months'' after diagnosis. Tumor growth was the main indication to convert AS to active treatment (58%) with 8% of the patients opting to discontinue AS. No patient had metastatic progression nor disease-specific death. The correlation between biopsy and surgical specimen was 92%. CONCLUSION: Active surveillance for biopsy-proven renal oncocytomas was oncologically safe and patient adherence was high. No predictive factor for tumor growth could be identified but the tumor growth rate was low, and biopsy efficacy was high.

Anatomical repair of a congenital aneurysm of the distal abdominal aorta in a newborn.

Congenital (primary) neonatal abdominal aortic aneurysm (AAA) is an extremely rare truncular arterial abnormality among numerous congenital vascular malformations. Only seven cases have been reported as congenital origin in newborns. This report presents the case of a male infant in whom a 33-mm congenital AAA was diagnosed prenatally and was successfully treated 10 days after birth without exogenous graft material or aneurysmorrhaphy. Follow-up study at 39 months demonstrated excellent clinical, ultrasound scan, and computed tomography scan findings. Anatomic reconstruction with native vessels is the preferred surgical technique to ensure the child's potential for harmonious growth.

Nephrectomy improves overall survival in patients with metastatic renal cell carcinoma in cases of favorable MSKCC or ECOG prognostic features.

OBJECTIVES: The role of cytoreductive nephrectomy (CN) in the treatment of patients harboring metastatic renal cell carcinoma (mRCC) has become controversial since the emergence of effective targeted therapies. The aim of our study was to compare the overall survival (OS) between CN and non-CN groups of patients presenting with mRCC in the era of targeted drugs and to assess these outcomes among the different Memorial Sloan-Kettering Cancer Center (MSKCC) and The Eastern Cooperative Oncology Group (ECOG) performance status subgroups. METHODS AND MATERIALS: A total of 351 patients with mRCC at diagnosis recruited from 18 tertiary care centers who had been treated with systemic treatment were included in this retrospective study. OS was assessed by the Kaplan-Meier method according to the completion of a CN. The population was subsequently stratified according to MSKCC and ECOG prognostic groups. RESULTS: Median OS in the entire cohort was 37.1 months. Median OS was significantly improved for patients who underwent CN (16.4 vs. 38.1 months, P<0.001). However, subgroup analysis demonstrated that OS improvement after CN was only significant among the patients with an ECOG score of 0 to 1 (16.7 vs. 43.3 months, P = 0.03) and the group of patients with good and intermediate MSKCC score (16.8 vs. 42.4 months, P = 0.02). On the contrary, this benefit was not significant for the patients with an ECOG score of 2 to 3 (8.0 vs. 12.6 months, P = 0.8) or the group with poor MSKCC score (5.2 vs. 5.2, P = 0.9). CONCLUSIONS: CN improves OS in patients with mRCC. However, this effect does not seem to be significant for the patients in ECOG performance status groups of 2 to 3 or poor MSKCC prognostic group.

Influence of previous or synchronous bladder cancer on oncologic outcomes after radical nephroureterectomy for upper urinary tract urothelial carcinoma.

OBJECTIVE: The objective of the study was to evaluate the effect of a history of bladder cancer (BC) or synchronous BC on the prognosis and survival of patients who have undergone radical nephroureterectomy (RNU). METHODS AND MATERIALS: Using a multi-institutional, retrospective database, we identified 662 patients with upper urinary tract urothelial carcinoma (UUT-UC) treated by radical nephroureterectomy, between 1995 and 2010. We analyzed clinicopathologic characteristics and outcomes according to the history of BC or concomitant BC or both, at the time of diagnosis. BC was evaluated as a prognostic factor for bladder recurrence and survival. RESULTS: Overall, 83 (12.5%) patients had previous BC, 62 (9.4%) exhibited concomitant BC, and 75 (11.3%) presented with both previous and current BC. A history of BC was less seen in women and nonsmokers (P<0.0001 and P = 0.013, respectively). The patients with associated BC had more tumors located in the ureter (P<0.0001), as well as more multiple locations in the upper tract (P<0.0001). The tumors without concomitant BC were more likely to be associated with locally advanced stages (P = 0.024). At a median follow-up time of 37.3 months, 31.4% of patients experienced BC recurrence and 2.9% developed contralateral upper tract tumor. Using multivariate analyses, the previous or synchronous BC (P = 0.01) and positive surgical margins (P = 0.03) are independent prognostic factors for BC recurrence. The metastasis-free survival and cancer-specific survival rates did not significantly differ according to the associated BC status. CONCLUSIONS: In patients without previous or concomitant BC, the upper tract tumors are more frequently localized in the renal pelvis and are associated with a more invasive status at the time of diagnosis. Nevertheless, the presence of UUT-UC without previous or synchronous BC did not significantly affect the survival rates after nephroureterectomy.