The lecture presents the currently available data on the prevalence of upper urinary tract tumors, their etiology and epidemiology, and anatomical pathology. The clinical manifestations of the disease are stated. The bulk of the lecture is dedicated to the diagnosis of tumors of the renal pelvis and ureter. Treatment options, such as open and endoscopic surgery, radio- and chemotherapy, are discussed.

Transurethral removal of tumor with following intravesical chemotherapy with Gemicitabine were performed to 14 patients with superficial bladder cancer relapses after TUR with adjuvant intravesical BCG – therapy. Adjuvant treatment was initiated 2 – 3 weeks after TUR: 1000 mg of gemicitabine was solved in 100 ml of physiologic solution and instillated into the bladder 2 times a week for 3 week. After 1 week break the second course of chemotherapy was carried out.Treatment was well tolerated by all patients. The follow-up period ranged from 1,5 to 22 months (mean 10,6±7,0 months). After mean period of 11,3 months 10 patients were disease free. Recurrence developed in 4 patients with multifocal bladder relapses. No disease progression was registered. Adjuvand intravesical chemotherapy with Gemicitabine is effective in patients with recurrences of superficial bladder cancer and is well tolerated.

About 20—25 % of all rhabdomyosarcomas in children are located in the urogenital zone. Due to introduction of new drug treatment protocols and the use of radiation therapy, most patients are cured by conservative methods. Only about 20 % of patients undergo surgical treatment, significant number of which are underwent organ-preserving operations. In those cases when the tumor has resistance to conservative treatment, when there is a residual tumor or relapse of the disease develops, radical organ-resecting operations are considered. Among the methods of urine derivation after cystectomy, preference is given to options that allow patients to carry out urine excretion voluntary: the formation of heterotopic reservoirs connected to the urethra or opening on the skin surfaces, which are constructed from different parts of the intestine. A clinical example of late postoperative complication is presented — a calculi formation within the pouch 7 years after radical cystprostatectomy with continent urinary diversion in a patient operated for urogenital rhabdomyosarcoma.

Objective : to compare the results of partial (PN) and radical nephrectomy (RN) in patients with renal parenchymal tumors with nephrometric PADUA index ≥8 and/or RENAL index ≥7 and functioning contralateral kidney. Materials and methods . Medical data of 114 consecutive patients with renal cell carcinoma (RCC) cT1–3aN0M0 and functioning contralateral kidney were included into the study: 57 (50.0 %) persons undergone PN for tumors with nephrometric PADUA index ≥8 and/or RENAL index ≥7 (the main group), and 57 (50.0 %) patients undergone RN (the control group). The groups were comparable with regards to demographic characteristics, nephrometric parameters, morphological tumor features, and baseline glomerular filtration rate (р >0.05 for all). Median follow-up – 52.0 ± 23.6 (9.1–138.5) months. Results . Technically complicated PNs were associated with an increase of median surgery time (by 39 min, р = 0.06), blood loss (by 319 ml, p within the groups. Chronic kidney disease (CKD) progression rate was significantly higher in RN than in PN group (40 % vs 31.6 % respectively, р = 0.050), including more frequent development of CKD stages III–IV (31.6 % vs 26.3 % respectively; р = 0.034). There was no difference of long-term survival between PN and RN groups, and that included survival results stratified according to gender, age, baseline CKD stage, PADUA and RENAL indexes. Five-year recurrence-free survival was 94.1 % vs 92.2 % (р = 0.223), cancer-specific survival – 92.3 % vs 90.8 % (р = 0.443), cardio-specific survival – 91.6 % vs 77.9 % (р = 0.549), overall survival – 89.8 % vs 70.7 % respectively (р = 0.858). Conclusion . PN is effective and safe method of treatment in patients with renal parenchymal tumors with PADUA index ≥8 and/or RENAL index ≥7 and functioning contralateral kidney, providing significant functional benefit without survival compromising when compared with RN.

Objective: to develop an effective and safe surgical technique for the treatment of patients with renal cell carcinoma with invasive tumor venous thrombosis of the inferior vena cava (IVC). Materials and methods. The study included 75 patients underwent surgical treatment at the N.N. Blokhin Russian Cancer Research Center between 1995 and 2017. The median age of patients was 57 years (range: 32–72 years). All patients were diagnosed with RCC with invasive tumor venous thrombosis levels II–IV; of them, 55 patients (73.3 %) had complete IVC obstruction and mature venous collaterals. Twenty- seven patients (26.0 %) were diagnosed with regional, 37 (49.3 %) – with distant metastases. Prior nephrectomy was performed in 5 (6.7 %) cases. Surgical treatment included nephrectomy (n = 70; 93.3 %), thrombectomy with IVC resection (n = 75; 100 %), and metastasectomy in solitary distant lesions (n = 11; 14.7 %). Partial IVC resection was demanded in 18 patients (24.0 %): with infrarenal IVC plication – 14 (18.7 %), with reconstruction of IVC with synthetic patch – 4 (5.3 %). Fifty-seven patients (76.0 %) underwent circular IVC resection (with left renal vein (LRV) ligation – 35 (46.7 %)). The IVC was replaced with ePTFE grafts in 4 (5.3 %) patients, IVC reconstruction was not required in 53 (70.7 %) patients. IVC grafting was considered to be justified in patients without mature venous collaterals. Twenty-two patients (29.3 %) received systemic antitumor therapy. Median follow-up was 32.3 months (range: 1–226 months). Results. Median operative time was 237.5 min (range: 135–580 min); median blood loss – 7000 mL (range: 1200–27 000 mL). The post- operative complications rate was 52.1 % (grades III–V – 31.5 %). Hospital mortality was 13.3 % (10 of 75 patients). Thirty-two months overall, cancer-specific, and recurrence-free survival were 42.4 %, 49.5 %, and 61.2 % respectively. At 19 months all prosthesis were patent. None of the patients had glomerular filtration rate <60 ml/min/1.73 m2 after LRV ligation. No patients developed disabling chronic venous insufficiency of the lower limbs after IVC ligation/resection without grafting. Conclusion. Nephrectomy, thrombectomy, and IVC resection is the only effective method of treatment for RCC with invasive tumor venous thrombosis. The development of IVC and LRV venous collaterals allows performing circular IVC resection with LRV ligation without graft replacement.

Kidney transplantation is the most frequently performed organ transplant procedure in the world. The occurrence of malignant tumors is one of the well-known late complications of organ transplantation, which is induced by immunosuppressive therapy. In the vast majority of patients, kidney cancer occurs in the native organs; however, in a small percentage of cases, malignancies are found in the graft organ. The article describes a rare clinical case of a patient with synchronous cancer in the native and graft kidneys.

Objective: to assess the early and late functional results of partial nephrectomy in patients with solitary functioning kidney tumors.Materials and methods. Medical data of 131 consecutive patients with solitary kidney parenchymal tumor, who had undergone partial nephrectomy at the N. N. Blokhin Russian Cancer Research Center, were analyzed. The median age was 57 (26–75) years. All the patients were diagnosed with solitary kidney tumor (median RENAL score was 7.0 ± 2.4 and median PADUA score was 9.0 ± 2.2). The median baseline glomerular filtration rate (GFR) was 74 (33–159) ml/min/1.73 m2 (30 (22.9 %) – stage III chronic kidney disease (CKD) and 0 (0 %) – stages IV–V CKD). All the patients underwent partial nephrectomy (with ischemia in 98 (74.8 %) patients, including cold ischemia in 59 (45.1 %)). The median time of ischemia was 24 (7–80) min. The median blood loss was 800 (20–4500) ml.Results. Acute renal injury was recorded in 69 (52.7 %) cases; 6 patients (4.6 %) had indications for acute dialysis. The independent risk factors of acute renal injury were sinus invasion (hazard ratio (HR) 0.08; 95 % confidence interval (CI) 0.03–0.22; p = 0.051), baseline GFR <80 ml/min/1.73 m2 (HR 0.45; 95 % CI 0.22–0.92; p = 0.021), parenchymal ischemia (HR 0.13; 95 % CI 0.05–0.33; p = 0.032), and >500-ml blood loss (HR 0.24; 95 % CI 0.12–0.51; p = 0.005). Progression of previously diagnosed CKD was recorded in 58 (44.6 %) patients; chronical dialysis was required in 2 (1.5 %) patients. The independent risk factors of CKD progression were sinus invasion (HR 0.38; 95 % CI 0.18–0.81; p = 0.002), medial location of the tumor (HR 0.19; 95 % CI 0.09–0.41; p = 0.001), baseline GFR, <60 ml/min/1.73 m2 (HR 0.24; 95 % CI 0.10–0.56; p <0.0001), warm ischemia (HR 0.41; 95 % CI 0.17–1.00; p = 0.052).Conclusion. Solitary kidney resection is associated with a low risk for renal function loss. To achieve optimal functional results, it is desirable to follow the balance between the indications for renal vessel ligation and the need to avoid >500-ml blood loss and cold ischemia.

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

Paraganglioma is hormonally active tumor originating from chromaffin tissue of sympathoadrenal system that secrete large amounts of catecholamines. Paraganglioma of the urinary bladder is a rare neoplasm, constituting approximately 0.06 % of all tumors of this localization. It is believed that this disease arises from embryonic remnants of chromaffin cells in the sympathetic plexus of the detrusor. Up to 10 % of these tumors have malignant origin. Diagnosis and tactics of treatment of patients with malignant paragangliomas of the bladder presents certain difficulties. The article describes 2 clinical observations of patients with this bladder pathology.

The paper describes a rare case of synchronous bilateral tumor involvement of the urinary tract.

Цель исследования – изучение целесообразности и безопасности спасительной цистэктомии после органосохраняющего лечения у больных мышечно-инвазивным раком мочевого пузыря. Материалы и методы. В исследование ретроспективно отобраны данные 130 больных переходно-клеточным раком мочевого пузыря, получавших лечение в РОНЦ им. Н.Н. Блохина в период с 1981 по 2016 г. В основную группу вошли 66 пациентов, подвергнутых спасительной цистэктомии после безуспешного органосохраняющего лечения, основанного на лучевой терапии. Лимфодиссекцию произвели 42 (63,6 %) пациентам. С целью отведения мочи выполнялись: операция Бриккера (42 (63,6 %)), операция Штудера (7 (10,6 %)), другое (17 (25,8 %)). Морфологически рак мочевого пузыря верифицирован в 62 (93,9 %) препаратах (категория Р1 – 6 (9,1 %), Р2 – 21 (31,8 %), Р3 – 25 (37,9 %), Р4 – 18 (27,2 %)); метастазы в лимфатические узлы выявлены в 11 (16,6 %) случаях. Степень анаплазии расценена как G3 в 35 (56,5 %) из 62 препаратов, содержавших опухоль. В группу сравнения включены 64 пациента, которым была выполнена радикальная цистэктомия без предшествующего лечения. Частота категорий Т3a–4b и степени анаплазии G3 была достоверно выше для всех пациентов основной группы (р < 0,0001). Результаты. Частота интраоперационных осложнений спасительных цистэктомий составила 10,6 %, послеоперационных – 42,7 % (28 из 65) (I–II степени тяжести – 27,3 % (18 из 28), III–V степени тяжести – 15,4 % (10 из 28)). Пятилетняя общая, специфическая и безрецидивная выживаемость пациентов основной группы составила 43,7; 58,6 и 54,7 % соответственно. Независимыми факторами благоприятного прогноза выживаемости явились степень анаплазии G1–2, гидронефроз и лимфодиссекция. Рецидивы после спасительных операций развивались реже, чем после цистэктомий, выполненных без предшествующего лечения (19 (30,6 %) и 31 (48,4 %) соответственно, р = 0,031). Других статистически значимых различий результатов спасительной и радикальной цистэктомий не выявлено. Заключение. Спасительная цистэктомия после безуспешного органосохраняющего лечения у больных мышечно-инвазивным раком мочевого пузыря ассоциирована с приемлемым операционным риском и обеспечивает удовлетворительные отдаленные результаты, сопоставимые с радикальной цистэктомией.

Objective. Evaluation of utility and safety of salvage cystectomy after organ preservation treatment in patients with muscle-invasive bladder cancer.Materials and methods. A retrospective study included data on 130 patients with transitional cell carcinoma treated at the N.N. Blokhin Russian Cancer Research Center in 1981-2016. The main group included 66 patients who underwent salvage cystectomy after unsuccessful organ preservation treatment based on beam radiation therapy. Lymph node dissection was performed in 42 (63.6 %) patients. For the purposes of urinary diversion 42 (63.6 %) patients received ileal conduit (Bricker procedure), 7 (10.6 %) - Studer deal neobladder, 17 (25.8 %) - other treatment. Bladder cancer was morphologically confirmed in 62 (93.9 %) samples (P1 stage - 6 (9.1 %), P2 - 21 (31.8 %), P3 - 25 (37.9 %), P4 - 18 (27.2 %)); lymph node metastases were discovered in 11 (16.6 %) cases. Anaplasia grade was G(3) in 35 (56.5 %) of the 62 samples containing tumor. Control group included 64 patients who underwent radical cystectomy without previous treatment. Rates of T3a-4b categories and G3 anaplasia grade were significantly higher in the main group (p < 0.0001).Results. Rate of intraoperative complications of salvage cystectomies was 10.6 %, postoperative - 42.7 % (28 of 65) (severity grade I-II in 27.3 % (18 of 28) of cases, severity grade III-V in 15.4 % (10 of 28) of cases). Five-year total, specific and relapse-free survival in the main group was 43.7, 58.6 and 54.7 %, respectively. Independent factors of favorable prognosis for survival were anaplasia grade G(1-2), hydronephrosis and lymph node dissection. Recurrences after salvage surgeries were less frequent than after cystectomies performed without previous treatment (19 (30.6 %) and 31 (48.4 %), respectively, p = 0.031). No other statistically significant differences between salvage and radical cystectomies were observed.Conclusion. Salvage cystectomy after unsuccessful organ preservation treatment in patients with muscle-invasive bladder cancer is associated with acceptable surgical risk and provides satisfactory long-term results comparable with radical cystectomy.

Objective: to assess the results of nephrectomy, thrombectomy in RCC patients with level III–IV tumor venous thrombosis with and without cardiopulmonary bypass.Materials and methods. Medical data of 167 consecutive RCC patients with level III–IV tumor venous thrombosis underwent nephrectomy thrombectomy in N.N. Blokhin Russian Cancer Research Center between 1998 and 2012 were collected. Right side tumor was in 122 (73.1 %), left side – in 42 (25.1 %), bilateral – in 3 (1.8 %) cases. The extent of thrombus was defined as intrahepatic in 82 (49.1 %), supradiaphragmatic – in 85 (50.9 %) (intrapericardial – in 44 (26.3 %), intraatrial – in 39 (23.4 %), intraventricular – in 2 (1.2 %)) cases. Nephrectomy, thrombectomy with cardiopulmonary bypass was used in 9 (5.4 %), 158 (94.6 %) patients underwent radical nephrectomy with thrombectomy without CPBP and sternotomy. Intrapericardial IVC and right atrium were exposed through transdiaphragmatic approach and providing vascular control over infradiaphragmatic IVC and renal veins.Results. Median blood loss was 6000 (600–27 000) ml. Complications rate was 62.8 %, 90-day mortality – 13.2 %. Intraoperative complications were registered in 80 (47.9 %), postoperative – in 66 (40.5 %) (grade II – 16 (9.8 %), grade IIIb – 1 (0.6 %), grade IVа – 28 (17.2 %), grade IVb – 3 (1.8 %), grade V – 18 (11.1 %)) patients. Modified thrombectomy technique insignificantly decreased blood loss compared to thrombectomy with CPB, did nоt increase complications rate including pulmonary vein thromboembolism, or mortality. Five-year overall, cancer-specific and recurrence-free survival was 46.2, 58.3 and 47.1 %, respectively. Thrombectomy technique did nоt affect survival.Conclusion. In selected patients with mobile thrombi transdiaphragmatic approach allows to avoid the use of CPBP and decrease surgical morbidity without survival compromising.

Paraganglioma is hormonally active tumor originating from chromaffin tissue of sympathoadrenal system that secrete large amounts of catecholamines. Paraganglioma of the urinary bladder is a rare neoplasm, constituting approximately 0.06 % of all tumors of this localization. It is believed that this disease arises from embryonic remnants of chromaffin cells in the sympathetic plexus of the detrusor. Up to 10 % of these tumors have malignant origin. Diagnosis and tactics of treatment of patients with malignant paragangliomas of the bladder presents certain difficulties. The article describes 2 clinical observations of patients with this bladder pathology.

To compare 30 day outcomes and costs of fenestrated and branched stent grafts (f/b EVAR) and open surgery (OSR) for the treatment of complex abdominal aortic aneurysms (AAA) and thoraco-abdominal aortic aneurysms (TAAA).The multicenter prospective registry WINDOW was set up to evaluate f/b EVAR in high risk patients with para/juxtarenal AAA, and infradiaphragmatic and supradiaphragmatic TAAA. A control group of patients treated by OSR was extracted from the national hospital discharge database. The primary endpoint was 30 day mortality. Secondary endpoints included severe complications, length of stay, and costs. Mortality was assessed by survival analysis and uni/multivariate Cox regression analyses using pre- and post-operative characteristics. Bootstrap methods were used to estimate the cost-effectiveness of f/b EVAR versus OSR.Two hundred and sixty eight cases and 1,678 controls were included. There was no difference in 30 day mortality (6.7% vs. 5.4%, p = 0.40), but costs were higher with f/b EVAR (€38,212 vs. €16,497, p < .001). After group stratification, mortality was similar with both treatments for para/juxtarenal AAA (4.3% vs. 5.8%, p = .26) and supradiaphragmatic TAAA (11.9% vs. 19.7%, p = .70), and higher with f/b EVAR for infradiaphragmatic TAAA (11.9% vs. 4.0%, p = .010). Costs were higher with f/b EVAR for para/juxtarenal AAA (€34,425 vs. €14,907, p < .0001) and infradiaphragmatic TAAA (€37,927 vs. €17,530, p < .0001), but not different for supradiaphragmatic TAAA (€54,710 vs. €44,163, p = .18).f/b EVAR does not appear justified for patients with para/juxtarenal AAA and infradiaphragmatic TAAA fit for OSR but may be an attractive option for patients with para/juxtarenal AAA not eligible for surgery and patients with supradiaphragmatic TAAA. Clinical Trial Registration: http://www.clinicaltrials.gov/ct2/show/NCT01168037; identifier: NCT01168037 (WINDOW registry).

Seminal vesicle tumors are very rare malignancies which are not diagnosed in daily clinical oncology practice. Primary malignant tumors in seminal vesicle are difficult to define due to the lack of specific symptoms in the early stages of the disease. Another obstacle of proper diagnosis is the frequent invasion of tumors of the surrounding organs, especially the prostate, rectum and bladder which is difficult to differentiate. Very often seminal vesicle tumors are difficult to detect. Digital rectal examination as well as transrectal ultrasound scan (US) could reveal a bulky mass in the retrovesical space. Computed tomography and magnetic resonance imaging (MRI) are the main diagnostic methods which could help to reveal pathologic masses in the region of seminal vesicles. Levels of prostate-specific antigen, carcinoembryonic antigen and tumor markers specific for colorectal cancer are negative in seminal vesicle tumors.The world experience of treating seminal vesicle tumors is very limited. There is paucity of data regarding appropriate choice of surgical approach and further treatment strategy and most of the time the treatment is individualized and based on very scarce information. At the same time surgical approach may vary significantly from vesiculectomy to pelvic exenteration. Possibility of using any regimens of adjuvant radiation therapy, chemotherapy or hormone therapy is highly debatable. However, aggressive surgical approach with radical tumor removal followed by extended lymphodissection shows the most favorable results in survival of patients suffering from seminal vesicle cancer.Squamous cell carcinoma of the seminal vesicles is presumed to be an extremely rare disease as there are only 3 reports of it in the world literature. We report a case of patient B. suffering from squamous cell carcinoma of the right seminal vesicle whom we conducted an aggressive surgical approach – prostatovesiculectomy followed by resection of the posterior bladder wall. There was no adjuvant chemotherapy after surgery. In the next 22 months such diagnostic methods as US and MRI revealed no metastases or symptoms of the disease recurrence.