Nephron sparing surgery in tumours greater than 7cm.

INTRODUCTION: Partial nephrectomy (PN) is the gold standard treatment for renal cell carcinomas under 4cm. No robust data exists to recommend PN for tumours>7cm (cT2). The objective of this work is to evaluate the results of PN for cT2 tumours. PATIENTS AND METHODS: All patients who underwent PN or radical nephrectomy (RN) for cT2 tumours between 2000 and 2013 at our institution have been included. Patient demographics, postoperative data including renal function, morbidity, mortality and oncologic outcomes were reviewed retrospectively and compared using χ2 test, Mann-Whitney test, Kaplan-Meier method and log rank test. RESULTS: We included 130 patients, 49 (38%) in the PN group and 81 (62%) in the RN group, with a median follow-up of 42 months [19-69]. Variation of postoperative renal function at day 5 and last recorded value was significantly different between the groups (P=0.03 and P<0.001). The PN group had a significantly higher complication rate as compared with RN group (37% versus 14%, P=0.002). There were no significant differences between the two groups for overall, recurrence free and specific survival (P=0.55, P=0.55, P=0.24, respectively). In univariate analysis, the type of surgery (PN versus RN) was not associated with a significant difference of oncologic outcome (margins, survival). CONCLUSION: PN can be offered for cT2 tumours with oncological outcomes similar to RN. Despite an increased morbidity, it remains acceptable with the demonstrated advantage of preservation of renal function. LEVEL OF EVIDENCE: 4.

Simple and complex renal cysts in adults: Classification system for renal cystic masses.

Ultrasound and slice imaging are currently the key modalities for diagnosing simple renal cysts. Benign simple renal cysts are defined by a set of strict criteria. Any cystic lesions that do not meet these criteria should be classified as "atypical" or "complex" and considered as suspicious. For over three decades, the Bosniak classification has been the central tool for diagnosing renal cystic masses larger than 1 cm. The Bosniak classification is based upon the results of a well-established pre- and post-contrast computed tomography (CT) protocol. Lesions that are difficult to classify or that remain indeterminate after CT can benefit from a multimodal diagnostic approach combining CT, non-contrast-enhanced and contrast-enhanced ultrasound, and magnetic resonance imaging.

[Sporadic kidney cancer of young subjects: Study of the clinical and pathological features of a bicentric cohort]. / Le cancer du rein sporadique du sujet jeune : étude des particularités cliniques et anatomopathologiques d'une cohorte bicentrique.

INTRODUCTION: The epidemiology of kidney cancer is evolving with a net increase in the incidence of renal tumors, globally, and in young people in particular. OBJECTIVE: To evaluate the incidence and clinical and pathological characteristics of sporadic renal tumors in young subjects and their risk factors. MATERIAL AND METHODS: A retrospective study aimed at collecting clinical, epidemiological and anatomopathological information from the 118 patients aged 18 to 40 treated for a sporadic kidney tumor in two Parisian university hospital centers between 2003 and 2013. RESULTS: Our study showed a very significant increase in the incidence of renal tumors in our 11 years of decline (P=6.10-15). The mode of discovery also seems to have evolved with a majority of tumors (67 %), due to the considerable growth of imaging in recent decades. We also showed a different pathological distribution compared to the literature with a significant increase in the number of papillary tumors (16.9 %) and chromophobes (15.2 %), in addition to a decrease in the number of carcinomas (43.2 %) as well as the appearance of a new pathological entity of particular clinical severity: renal carcinoma related to translocation Xp11.2 (15.3 %) (P<10-5). Among the risk factors, hypertension seems to be a definite risk factor while tobacco and obesity do not have a significant influence. CONCLUSION: Our study showed a marked increase in the incidence of renal tumors with specific clinical and epidemiological features in a population of young subjects. The role and importance of oncogenetic management as well as the study of environmental factors could lead to the identification of new risk factors and corollary to their prevention. LEVEL OF EVIDENCE: 4.

[Cancer genetic screening for patient with multiple renal tumors: What is the current practice for what results?]. / Dépistage oncogénétique des patients atteints de tumeurs rénales multiples : quelle pratique pour quels résultats ?

AIM: To evaluate in a high volume center the practice and the performance of cancer genetic screening for patients with multiple renal tumors without a predisposition to kidney tumors known at the time of surgery. METHOD: All patients treated for multiple renal tumors from January 2000 to December 2013 in our center were included. Patients with a family history of renal cell carcinoma, a kidney disease or a genetic predisposition to renal cancer known at the time of surgery were excluded from the analysis. Our list of patients was retrospectively compared to the records from PREDIR (PREDisposition to Kidney Tumors) center of Île-de-France, which regionally centralizes the care of patients with kidney tumors associated with a genetic predisposition. RESULTS: One hundred and thirty-six patients were included. Twenty-six patients of 136 (19%) were referred to PREDIR center: 23 followed the screening and 3 did not show up in consultation. Of the 23 patients screened, three genetic predispositions to kidney tumors were identified (13%). Of the 95 patients with synchronous tumors, 48% with more than 3 tumors benefited from cancer genetics investigation against 13% of those presenting only two tumors. CONCLUSION: Our study shows that cancer genetic investigations are not routinely offered to patients with multiple kidney tumors, conversely to existing guidelines. Urologists must be aware of this screening. LEVEL OF EVIDENCE: 4.

Multimodal management of primary adenocarcinoma of the female urethra: About four cases.

PURPOSE: To retrospectively analyse female patients treated for urethral adenocarcinoma, modalities of treatment and long-term outcomes. PATIENTS: Four cases of primary female urethral adenocarcinoma were treated in the departments of urology and radiation-oncology at Georges-Pompidou and Necker hospitals (France) over a 7-year period. RESULTS: All of them underwent surgery, with three presenting stage pT3-pT4 and one a positive cytology on inguinal node. Three patients received adjuvant cisplatin-based chemoradiotherapy up to 60Gy, and one preoperative chemoradiotherapy at 45Gy. Two recurrences were observed: one local relapse occurred at 9months from the diagnosis and was treated by anterior pelvic exenteration followed by chemoradiotherapy, with no recurrence. One tumour relapsed both at the local level and on distant metastatic sites at 9months from the diagnosis, and died 21months after this progression. Median survival and progression-free survival are respectively 4.2years and 13months. Three patients are alive at 7, 4.5 and 3years from diagnosis. CONCLUSION: Female urethral adenocarcinoma is a very rare entity and often present in locally advanced stages. Initial extensive surgery with pelvic exenteration should be considered, followed by chemoradiotherapy according to the surgical margins and lymph nodes involvement.

Tubulocystic renal cell carcinoma: a new radiological entity.

UNLABELLED: Tubulocystic renal cell carcinoma (TC-RCC) is a recently identified renal malignancy. While approximately 100 cases of TC-RCC have been reported in the pathology literature, imaging features have not yet been clearly described. The purpose of this review is to describe the main radiologic features of this rare sub-type of RCC on ultrasound, computed tomography (CT), and magnetic resonance imaging (MRI), based jointly on the literature and findings from a multi-institutional retrospective HIPAA-compliant review of pathology and imaging databases. Using a combination of sonographic and CT/MRI features, diagnosis of TC-RCC appeared to be strongly suggested in many cases. KEY POINTS: • Tubulocystic renal cell carcinoma is a new entity with typical imaging features • Diagnosis of tubulocystic renal cell carcinoma can be suggested preoperatively by imaging • Cystic renal lesions with high echogenicity may correspond to tubulocystic carcinoma.

RANK/OPG ratio of expression in primary clear-cell renal cell carcinoma is associated with bone metastasis and prognosis in patients treated with anti-VEGFR-TKIs.

BACKGROUND: Bone metastases (BMs) are associated with poor outcome in metastatic clear-cell renal carcinoma (m-ccRCC) treated with anti-vascular endothelial growth factor tyrosine kinase inhibitors (anti-VEGFR-TKIs). We aimed to investigate whether expression in the primary tumour of genes involved in the development of BM is associated with outcome in m-ccRCC patients treated with anti-VEGFR-TKIs. METHODS: Metastatic clear-cell renal cell carcinoma patients with available fresh-frozen tumour and treated with anti-VEGFR-TKIs. Quantitative real-time PCR (qRT-PCR) for receptor activator of NF-kB (RANK), RANK-ligand (RANKL), osteoprotegerin (OPG), the proto-oncogene SRC and DKK1 (Dickkopf WNT signalling pathway inhibitor-1). Time-to-event analysis by Kaplan-Meier estimates and Cox regression. RESULTS: We included 129 m-ccRCC patients treated between 2005 and 2013. An elevated RANK/OPG ratio was associated with shorter median time to metastasis (HR 0.50 (95% CI 0.29-0.87); P=0.014), shorter time to BM (HR 0.54 (95% CI 0.31-0.97); P=0.037), shorter median overall survival (mOS) since initial diagnosis (HR 2.27 (95% CI 1.44-3.60); P=0.0001), shorter median progression-free survival (HR 0.44 (95% CI 0.28-0.71); P=0.001) and mOS (HR 0.31 (95% CI 0.19-0.52); P<0.0001) on first-line anti-VEGFR-TKIs in the metastatic setting. Higher RANK expression was associated with shorter mOS on first-line anti-VEGFR-TKIs (HR 0.46 (95% CI 0.29-0.73); P=0.001). CONCLUSIONS: RANK/OPG ratio of expression in primary ccRCC is associated with BM and prognosis in patients treated with anti-VEGFR-TKIs. Prospective validation is warranted.

Double-echo gradient chemical shift MR imaging fails to differentiate minimal fat renal angiomyolipomas from other homogeneous solid renal tumors.

OBJECTIVES: The purpose of this retrospective study was to evaluate the diagnostic performance of double-echo gradient chemical shift (GRE) magnetic resonance (MR) imaging for the differentiation of angiomyolipomas with minimal fat (mfAML) from other homogeneous solid renal tumors. METHODS: Between 2005 and 2010 in two institutions, all histologically proven homogenous solid renal tumors imaged with computed tomography and MR imaging, including GRE sequences, have been retrospectively selected. A total of 118 patients (mean age: 61 years; range: 20-87) with 119 tumors were included. Two readers measured independently the signal intensity (SI) on GRE images and calculated SI index (SII) and tumor-to-spleen ratio (TSR) on in-phase and opposed-phase images. Intra- and interreader agreement was obtained. Cut-off values were derived from the receiver operating characteristic (ROC) curve analysis. RESULTS: Twelve mfAMLs in 11 patients were identified (mean size: 2.8cm; range: 1.2-3.5), and 107 non-AML tumors (3.2cm; 1-7.8) in 107 patients. The intraobserver reproducibility of SII and TSR was excellent with an intraclass correlation coefficient equal to 0.99 [0.98-0.99]. The coefficient of correlation between the readers was 0.99. The mean values of TSR for mfAMLs and non-mfAMLs were -7.0±22.8 versus -8.2±21.2 for reader 1 and -6.7±22.8 versus -8.4±20.9 for reader 2 respectively. No significant difference was noticed between the two groups for SII (p=0.98) and TSR (p=0.86). Only 1 out of 12 mfAMLs and 11 of 107 non-AML tumors presented with a TSR inferior to -30% (p=0.83). CONCLUSION: In a routine practice, GRE sequences cannot be a confident tool to differentiate renal mfAMLs from other homogeneous solid renal tumors.

Renal papillary carcinoma: CT and MRI features.

PURPOSE: To describe the CT and MRI appearances of papillary renal cell carcinoma. MATERIALS AND METHODS: Retrospective study of 102 papillary carcinomas in 79 patients, 81 tumors examined by CT and 56 by MRI. Tumor size, homogeneity and contrast enhancement were recorded. RESULTS: The most common presentation of papillary renal cell carcinoma was a small homogeneous hypovascular tumor both on CT and MRI. Eighty-nine percent of lesions were hypointense on T2 weighted images compared to the renal parenchyma. Seventeen percent of the lesions did not significantly enhance with contrast on CT. All of the lesions examined on MRI had a significant enhancement percentage. Calcifications were rare and only seen in 7% of cases (CT). The second most common presentation was a bulky necrotic tumor. In addition, atypical types of disease were found which were difficult to diagnose, including infiltrating tumors and tumors with a fatty component. CONCLUSION: A homogeneous hypovascular renal tumor which is hypointense on T2 weighted images should suggest a diagnosis of papillary carcinoma. Some papillary carcinomas do not enhance significantly on CT. MRI is then required to diagnose the renal tumor.