The malignant rhabdoid tumor (RT) is one of the most aggressive childhood neoplasm. RTs are characterized by the presence of inactivating mutations in the SMARCB1 (hSNF5/INI1/BAF47) gene – a tumor suppressor localized in 22q11.2. Up to 30 % of RTs caused by germline mutations of this gene, to date those cases are considered as a manifestation of the rhabdoid tumor predisposition syndrome type 1 (RTPS1). We have analyzed the SMARCB1 mutations by polymerase chain reaction and subsequent Sanger sequencing in 18 patients with RT in different localizations for improving of genetic laboratory diagnostics of the RTPS1, as well as searching of genotype-phenotype correlations in this disease. Three patients had de novo nonsense-mutations c.157C→T (p.R53*), c.669_670del (p.C223*) and c.843G→A (p.W281*), confirming RTPS1, which were associated with RT in the kidney, early age at diagnosis (median 2.6 months) and poor prognosis. Identification of germline SMARCB1 mutations in the patients with RTs is essential to assess the risk of metachronous tumors and for genetic counseling of other family members.

Objective. Study of 90 day complications after solitary kidney resection and determination of their dependence on resection complexity level in accordance with the RENAL nephrometry scoring system.

Materials and methods. Seventy (70) resections due to solitary kidney tumors were performed in 65 patients (34 males and 31 females). Mean age was 59.1 years (40–75 years). Fifty-five (55) patients had renal cell carcinoma, 10 patients had benign tumors. According to the RENAL scoring system, in 17 (24 %) cases resection complexity was 4–6 points, in 21 (30 %) cases it was 7–9 points, in 32 (45 %) cases it was 10–12 points. Mean tumor diameter was 4.4 (1.5–10.0) cm. Kidney resection without ischemia was performed in 3 patients, with segmental ischemia – in 39 cases. In 28 (40 %) cases general ischemia was used, its mean duration was 18 (6–48) minutes. Local hypothermia was used in 5 patients. Statistical analysis was performed using SPSS Statistics 16.

Results. Analysis of 90 day complications revealed no complications in 41 (58.6 %) cases, severity grade I complications were observed in 2 (2.9 %) cases, grade II – in 20 (28.6 %), grade III – in 6 (8.6 %); there were no grade IV and V complications. Hemodialysis wasn’t performed. Complications were significantly more frequent when total RENAL points were higher (Mann–Whitney U-test, р = 0.001); a positive correlation between RENAL score and presence of complications was observed (Spearman’s rho 0.411; p <0.001). A dependence between complications severity and RENAL score (Mann–Whitney U-test, р = 0.004) and a positive correlation between complications severity and RENAL score (Spearman’s rho 0.432; p <0,001) were revealed.

Conclusion. Forty-five percent (45 %) of kidney resections were of maximum complexity according to the RENAL scoring system. In 60 % of cases resection was performed without general ischemia. Analysis of the results of solitary kidney resection demonstrated high efficacy and safety of the procedure and dependence of the frequency and severity of complications on complexity level per the RENAL scoring system.

Aim: to evaluate results of partial nephrectomy for patients with a solitary kidney performed at the N.N. Blokhin Cancer Center.

Materials and methods: medical data of 131 patients with solitary kidney tumors underwent partial nephrectomy at the Department of Urology of the N.N. Blokhin Russian Cancer Research Center from 1980 to 2015 were collected. Median age was 57 (26–75) years, a maleto-female ratio was 1:1.2. Median RENAL score was 7.0 ± 2.4. In all cases, chronic kidney disease was initially diagnosed (stage III – 30 (22.9 %), stage IV–V – 0 (0 %)). Median follow-up for all patients was 55 (6–386) months.

Results. The rate of intraoperative complications was 9.2 % (12/131), the rate of postoperative complications was 26.9 % (35/131) (severity grade II – 23 (17.6 %), III – 10 (7.7 %), IV – 1 (0.8 %), V – 1 (0.8 %)). Acute renal failure was observed in 69 (52.7 %) cases, acute dialysis was necessary in 6 (4.6 %) patients. Progression of chronic kidney disease was observed in 58 (44.6 %) patients, hemodialysis was demanded for 2 (1.5 %) patients. Histological study revealed renal cell carcinoma in 124 (94.6 %) samples, positive surgical margins were in 2 (1.5 %) samples. No local recurrences in the resection area were observed. Overall 5- and 10-year survival for all patients with renal cell carcinoma was 89.6 and 72.0 %, specific survival was 93.5 and 81.3 %, recurrence-free survival was 85.3 and 62.2 % respectively.

Conclusion. Partial nephrectomy is an effective method of treatment in of solitary kidney tumors associated with satisfactory functional and oncological outcome

The article presents results of treatment of patients with localized renal cell carcinoma using the original technique of open partial nephrectomy. This technique involves the use of intraoperative ultrasonoscopy to define and mark the boundaries of the tumor and the method of creating a zone of local ischemia to resection the tumor without the use of total renal ischemia. The developed methodology in the comparative analysis of the immediate and remote results significantly (p > 0.05) reduces the amount of intraoperative blood loss without increasing the duration of the operation, reduces the risk of development of acute disorders of renal function in the early postoperative period, without compromising kidney function in the late postoperative period. This technique is comparable to the classical open radical resection of the kidney, does not affect the overall, oncospecific and relapse-free survival. 

This study is a retrospective analysis of the combined treatment of patients with metastatic renal cell carcinoma who had undergone spinal surgery. Objective: to evaluate the effect of the type of surgery on the spine, as well as targeted therapy on outcomes, quality of life and long-term outcomes of patients with mRCC in the spine. According to our data the survival of patients treated with targeted therapy was significantly higher than in patients without it. The use of targeted therapy in conjunction with surgery and sometimes teletherapy (DLT), increases survival and improves quality of life of patients with mRCC in the spine. 

The majority of patients with metastatic renal cell carcinoma (mRCC) are in the groups of favorable or moderate prognosis per the MSKCC (MemorialSloan-KetteringCancerCenter) criteria, but in 20–30 % of poor-prognosis patients aggressive, rapidly progressing mRCC is observed. Despite improved prognosis in mRCC patients due to targeted therapy, this patient group remains hard to treat with overall survival 6 months) was higher in the temsirolimus (32.1 %) and combination (28.1 %) groups compared to the IFN-α group (15.5 %; р<0,001).

The standard method of treatment of patients with metastatic renal cell carcinoma is targeted therapy. Selection of drugs for these patients has remained a complex task. In the absence of predictors of effectiveness, clinical trials should serve as a reference point. 

A comparative analysis of the results of diagnosis of cancer metastases in the lymph nodes using a method of one-step nucleic acids amplification (OSNA) and morphological methods was performed. Comparable sensitivity and specificity of OSNA and histological examination of a lymph node were observed. 

A separate group of patients who underwent radical prostatectomy, make patients with isolated recurrence of cancer in the lymph nodes, limited by regional and / or retroperitoneal lymph nodes. Alternatively, treatment of this patient group can be used open or laparoscopic lymphadenectomy (robot-assisted). The analysis of literature indicate an increase in disease-free survival of these patients during lymphadenectomy. Imaging techniques such as multiparametric magnetic resonance imaging and positron emission tomography, allow more accurately identify lymph nodes in patients with biochemical recurrence of prostate cancer after radical prostatectomy. 

Objectives. Analyzed short-and long-term results of radiation therapies (brachytherapy ¹²⁵I, conformal radiation therapy, stereotactic robotic radiotherapy) of patients with prostate cancer.

Materials and methods. In each treatment the patients were divided into 2 groups received the combined treatment in combination with neoadjuvant hormone therapy without its implementation. The immediate results of treatment were assessed according to the level of total prostatic specific antigen, prostate volume, residual urine, change according to the International Prostatic Symptom Score (IPSS).

Results. The dynamics of these indicators comparable in both groups, which is reflected in the regression of the primary tumor, as well as the quality of life of patients. Five-year overall survival and oncospecific survival in the group of patients after stereotactic radiotherapy is 100 %, the remaining groups – of more than 90–95 %.

Conclusion. The obtained results testify to the effectiveness of the proposed methods of treatment of patients with localized prostate cancer. 

Introduction. The 6-month depot formulation of leuproreline acetate 45 mg (Eligard, Astellas Pharma) was shown to reduce the levels of prostate-specific antigen (PSA) and testosterone and to be well tolerated in patients with advanced prostate cancer (PCa) in clinical trials as well as other depot formulations of leuproreline acetate (1- and 3-month). However, clinical trials are limited by strict patient inclusion and exclusion criteria.

Objective of this study, sponsored by Astellas Pharma Russia, was to assess whether the efficacy and tolerability of the 6-month leuprorelin depot formulation could be confirmed in a broad and heterogeneous patient population encountered in daily clinical practice in the Russian Federation.

Materials and methods. A non-interventional multicenter study (a observational program) was conducted in male patients with advanced PCa (T3–4, N+/M+, or with progression after local treatment) to whom Eligard 45 mg had been prescribed. Patients were followed every 6 months up to 24 months of treatment. Fifty three uro-oncologists from out-patient clinics in the Russia participated in the study.

Results. The study enrolled 640 patients, of which 524 met inclusion/exclusion criteria. Mean age of the patients was 69.0 ± 8.6 years old (from 46 to 96 years old). In most cases hormonal therapy with Eligard 45 mg was performed for locally advanced PCa (50 % of patients), distant metastases were detected in 15 % subjects only, nodal involvement was detected in 12 % patients. Patients with initially localized prostate cancer (41.4 %) were prescribed Eligard 45 mg because of progression of their disease after local treatment or inability to receive any other treatment. Eligard 45 mg monotherapy was used in 92.75 % cases. Only 7.25 % patients received combined hormonal therapy with Eligard 45 mg and bicalutamide, flutamide and zoledronic acid. Mean serum PSA level was reduced by 81,7 % from 38,47 ng/ml at baseline to 7.05 ng/ml after 24 months of treatment, while mean testosterone level was reduced by 88 % from 92.12 to 11.03 ng/dl. Testosterone level below 50 and 20 ng/dl was reached by 97 and 88 % of patients. There was a change in mean quality of life based on EQ-5D-5L questionnaire: mean value of health status according to visual scale increased from 76.15 ± 14.10 to 78.22 ± 15.99 mm. The number of adverse drug reactions was low and amounted to 1.41 % of all patient population. Asthenia, headache, arterial hypertension, dizziness and nausea were the most commonly reported adverse drug reactions.

Conclusions. These results suggest that the 6-month leuprorelin acetate depot formulations are well tolerated and reliably lower serum PSA and testosterone levels in daily clinical practice in patients with advanced PCa. These data are consistent with efficacy and tolerability results from clinical trials. 

 Introduction. Histological grading system is an important prognostic factor of bladder cancer. Grading of urothelial carcinoma has been a matter of debate since the three-grade system was introduced in 1973.

Objective. Optimization of the grading system for urothelial carcinoma.

Materials and methods. An analysis of literature devoted to evaluation of diagnostic significance, variability and interobserver reproducibility of the existing classifications of urothelial cancer of the bladder proposed in 1973, 1998, 1999 and 2004.

Results. The classification proposed in 1973 is the most popular and time honored method of grading bladder tumors. In 1998 it was modified by the International Society of Urological Pathology. In 1999 the World Health Organization (WHO) approved a new classification which preserved the three-grade system but differed from the previous ones. According to this new classification, tumors could fall into the following categories: papilloma, papillary urothelial neoplasm of low malignant potential, urothelial carcinoma of I, II, and III malignancy grade. The definition of papilloma was identical in all of these classifications. In 2004 a new WHO classification was introduced in which non-invasive urothelial tumors were subdivided into papilloma, papillary urothelial neoplasm of low malignant potential and low and high grade carcinoma. All of the proposed grading systems had a certain level of subjectivity and interobserver reproducibility, but reproducibility between unfamiliar pathologists was considerably higher than in groups of pathologists who had studied or worked together. Importantly, the 2004 WHO classification aimed to provide a detailed explanation of histological criteria for each diagnostic category and therefore improve reproducibility between different pathologists. However, no improvement of reproducibility in comparison with the 1973 WHO classification was observed. Moreover, among the pathologists better reproducibility of the 1973 WHO classification was registered compared to the 1999 and 2004 classifications. Reproducibility of the papillary urothelial neoplasm of low malignant potential diagnosis was only 48 %. At the same time, reproducibility of the 1973 WHO classification too has its problems. The biggest criticism is ambiguity in the diagnostic criteria of the 3 grades of urothelial carcinoma.

Conclusions. Standardization of the grading system of superficial bladder cancer allows to validate comparison between treatment outcomes in different centers. Introduction of the 2004 classification is the first step to treatment and monitoring standardization, but all of the classifications proposed by the WHO have shortcomings caused by considerable heterogeneity of papillary urothelial neoplasms. Significant interobserver reproducibility between papillary urothelial neoplasm of low malignant potential and low grade papillary urothelial carcinoma shows inadvisability of creating a separate diagnostic category for papillary urothelial neoplasm of low malignant potential. 

Objectives. Levels and expression of matrix metalloproteases 2 (MMP-2), MMP-9, MMP-14 and tissue inhibitor of metalloproteinases 1 (TIMP-1), TIMP-2 were studied by immunohistochemistry in tumor and surrounding tissues in 73 patients with superficial bladder cancer (BC) ТаN0M0 and Т1N0M0 depending on the disease characteristics.

 Results. The study showed the initial increase in the number of tumor cells expressing MMP-2, MMP-9 and MMP-14 and their tissue inhibitors, compared to histologically unchanged tissues and tumor-adjacent tissues. The number of MMP-9 producing tumor cells was maximal. Tissue expression of TIMP-1 and TIMP-2 in tumor cells in superficial BC patients was higher than in unchanged tissues and in tumor-adjacent tissues. The ratio of MMP and TIMP expression differed, and the proteolytic potential of MMP was higher than that of TIMP.

Conclusion. The study demonstrated the influence of the primary bladder cancer stage (from Ta to T1), tumor differentiation grade (from G1 to G2) and prognosis of the disease course (from favorable to intermediate) on the enhancing expression of MMP-9 and TIMP-1 in tumor tissues in superficial BC. 

Considerable life expectancy after radical prostatectomy dictates a necessity to preserve high quality of life of these patients. Conversely, attention is paid to functional results of the surgery, including preservation of the erectile function and quality of urination. Urinary obstruction in the postoperative period, caused primarily by postoperative vesicourethral anastomotic strictures, not only negatively impacts patients’ health but also has a pronounced maladaptation effect. The main factors of maladaptation are weak urine stream; frequent, often painful urination; episodes of acute urine retention. Currently, there are no strict guidelines for prevention of vesicourethral anastomotic strictures or for selection of an optimal method of correction of this postoperative complication of radical prostatectomy. This study highlights the main existing theories concerning development of vesicourethral anastomotic strictures and effectively used methods/ regimens for its treatment. 

One of clinical and anatomical types of melanoblastoma is melanoma of the glans penis appearing on the skin or mucosa of the penis (external opening of the urethra or ureter, internal or external layer of the foreskin). It’s a rare disease, and most of specialists are unfamiliar with it, including oncologists and urologists. Thus, the conducted research has practical importance as it may improve diagnostics and treatment of patients with this oncological pathology. 

Adrenal myelolipoma is a rare benign tumor with characteristic histological and radiological picture. Significant tumor size, suspicion of malignancy, and danger of bleeding are indications for laparoscopic adrenalectomy. In this article, we present 2 clinical cases of laparoscopic adrenalectomy for adrenal myelolipoma. 

In the era of targeted therapy, the attention of researchers is focused on the role of neoadjuvant targeted therapy in the treatment of patients with renal cell carcinoma. The advantages of neoadjuvant therapy for advanced renal cell carcinoma include: detection of patients with primary refractory tumors, recurring tumors, simple onset of systemic therapy. One of the most popular and suitable for neoadjuvant targeted therapy is sorafenib. The use of this drug, in our opinion, can significantly improve the results of treatment of such patients. The article describes the description of 3 clinical observations of a widespread cancer of the kidneys treated in a somatic mode in the neoadjuvant mode. Range of therapeutic effects from progressive diseases to stabilization. 

2017. Vol. 13, issue1