Clinicopathological features and prognosis of fumarate hydratase deficient renal cell carcinoma / 北京大学学报(医学版)

OBJECTIVE@#To investigate the clinicopathological features and prognosis of fumarate hydratase deficient renal cell carcinoma (FH-RCC).@*METHODS@#Immunohistochemical (IHC) staining was used to detect the expression of fumarate hydratase (FH) in tumor tissues of 109 different types of renal cell carcinoma (RCC) patients aged 60 years and younger from the Department of Urology of Peking University First Hospital from January 2013 to December 2019. The clinicopathological data and prognosis of FH-RCC were collected and analyzed.@*RESULTS@#There were eleven patients with FH-negative expression. Seven were males and four females. The age of onset ranged 16-53 years (mean age: 36.7 years), and four female patients all had a history of uterine leiomyoma. Only one first-degree relative of one patient had renal cancer, and none of the patients had a history or family history of cutaneous leiomyomas. The diameter of the tumor was 2.1-12.0 cm (mean: 8.83 cm). Renal sinus or perirenal fat invasion was seen in nine cases, tumor thrombus in renal vein or inferior vena cava in six cases, lymph node metastasis in seven cases, adrenal gland invasion in four cases and splenic capsule invasion in one case. The cases were initially diagnosed as type Ⅱ papillary RCC (7/49, 14.3%), collecting duct carcinoma (2/9, 22.2%) and unclassified RCC (2/51, 3.9%). Tumor histopathology mostly showed a mixture of different structures, such as papillary, tubular cystic, solid, and so on. The most common histological structures were papillary (9/11, 81.8%) and tubular (8/11, 72.7%). Three cases had sarcomatoid areas. At least focal eosinophilic nucleolus (WHO/grades Ⅲ-Ⅳ) and perinuclear halo could be seen in all cases. Immunohistochemical (IHC) stains of most tumors were negative for CA9, CD10 and CK7. The results of fluorescence in situ hybridization (FISH) showed that there was no translocation or amplification of TFE3 gene in two cases with TFE3 IHC expression. All the patients were followed up for 11-82 months. Mean survival was 24 months. Five cases died of distant metastasis 9-31 months after operation (mean: 19 months), and five of the six patients alive had became metastatic.@*CONCLUSION@#Morphologically, FH-RCC overlaps with many types cell RCC. A mixture of papillary and tubular cystic arrangement is the most common growth pattern of FH-RCC. At least focally large and obvious eosinophilic nucleoli are an important histological feature of this tumor. The negative expression of FH can help to confirm the diagnosis. Young female RCC patients with uterine leiomyomas should be suspected of FH-RCC. Some FH-RCC cases lack clinical evidence. The suspicion raised by pathologists based on histological characteristics is often the key step to further genetic testing and the final diagnosis of the tumor.

Three dimensional nephrometry system for partial nephrectomy: Our initial exploration / 北京大学学报(医学版)

OBJECTIVE@#To construct a preoperative evaluation system for partial nephrectomy using CT three-dimensional visualization technology and to explore its practical value.@*METHODS@#The clinical data of the patients who underwent partial nephrectomy for renal tumors in Department of Urology, Peking University First Hospital were collected retrospectively. At the same time, the homogenized standard data of patients who underwent partial nephrectomy for renal tumors were collected in 16 clinical centers in China. The CT three-dimensional visualization system was applied (IPS system, Yorktal) to evaluate tumor anatomy, blood supply, perirenal fat and other information. The parameters were summarized to build a three-dimensional nephrometry system, on the basis of which virtual surgery design and intraoperative navigation were completed.@*RESULTS@#A three-dimensional visualization image was established based on the enhanced CT urography. The nephrometry system included the longest diameter and volume of the tumor, proportion volume of tumor invading the parenchyma, maximum depth of the tumor invading the parenchyma, contact surface area, flatness of the tumor surface, renal segment where the tumor was located, vascular variation, and perirenal fat. The average two-dimensional diameter of the tumor was (2.78±1.43) cm, the average three-dimensional maximum diameter was (3.09±1.35) cm, and the average postoperative pathological size was (3.01±1.38) cm. The maximum tumor diameter in the three-dimensional image was significantly related to the prolonged renal artery clamping time and intra-operative blood loss (r=0.502, P=0.020; r=0.403, P=0.046). The three-dimensional and pathological tumor volume were (25.7±48.4) cm3 and (33.0±36.4) cm3, respectively (P=0.229). The tumor volume was significantly related to the intraoperative blood loss (r=0.660, P < 0.001). The proportion volume of the tumor invading into renal parenchyma was significantly related to the prolongation of renal artery clamping and the occurrence of postoperative complications (r=0.410, P=0.041; r=0.587, P=0.005). The tumor contact surface area and the presence of vascular variation did not show correlation with the perioperative data and postoperative complications. While the preoperative evaluation was completed, the reconstructed three-dimensional image could be zoomed, rotated, combined display, color adjustment, transparency, and simulated cutting on the Touch Viewer system. The process generally consisted of showing or hiding the tissue, adjusting the transparency of the interested area, rotating and zooming the image to match the position of the surgical patient. Together, these functions met the requirements of preoperative virtual surgery plan and intraoperative auxiliary navigation.@*CONCLUSION@#Three-dimensional images can provide a more intuitive anatomical structure. The CT three-dimensional visua-lization system clearly displays tumor anatomical parameters, blood supply and perirenal fat. The three-dimensional nephrometry system for renal tumors can help predict the difficulty of partial nephrectomy and perioperative complications. Importing the reconstructed three-dimensional visualization image into the specified program or robot operating system can complete virtual surgery and intraoperative navigation, helping the surgeon to better grasp the surgical process. The indexes included in the nephrometry system and the score weights of each index need to be confirmed and perfected by multi-center study with large samples.

Advance in re-do pyeloplasty for the management of recurrent ureteropelvic junction obstruction after surgery / 北京大学学报(医学版)

Ureteropelvic junction obstruction (UPJO) is characterized by decreased flow of urine down the ureter and increased fluid pressure inside the kidney. Open pyeloplasty had been regarded as the standard management of UPJO for a long time. Laparoscopic pyeloplasty reports high success rates, for both retroperitoneal and transperitoneal approaches, which are comparable to those of open pyeloplasty. However, open and laparoscopic pyeloplasty have yielded disappointing failure rates of 2.5%-10%. The main causes for recurrent UPJO are severe peripelvic and periureteric fibrosis due to urinary extravasation, ureteral ischemia, and inadequate hemostasis. In addition, failing to diagnose lower pole crossing vessels before or during the primary procedure is also responsible for recurrent UPJO. In addition, poor preoperative split renal function, hydronephrosis, presence of renal stones, patient age, diabetes, prior endopyelotomy history, and retrograde pyelography history were considered as predictors of pyeloplasty failure. The failure is usually defined by persistent pain, persistent radiographic obstruction (infection or stones), continued decline in split renal function, or a combination of the above. And the failure of pye-loplasty often occurs in the first 2 years after the surgery. The available options for managing recurrent UPJO with a salvageable renal unit include endopyelotomy, re-do pyeloplasty, stent implantation, percutaneous nephrostomy, ureterocalicostomy, and nephrectomy. Re-do pyeloplasty has such merits as high successful rates and rare complications, compared with endopyelotomy or ureterocalicostomy. And some investigators think that re-do pyeloplasty should be regarded as the gold standard for secondary therapy if feasible. Open pyeloplasty can enlarge the operating field, facilitate the exposure of the ureteropelvic junction, reduce the difficulty of operation, and thus reduce the occurrence of complications. There are no significant differences among the success rates of re-do pyeloplasty under open approach, traditional laparoscopy and robot-assisted laparoscopy, according to previous reports. However, traditional laparoscopic and robot-assisted pyeloplasty give advantages of cosmetology, small trauma, less postoperative pain, speedy recovery and shorter hospitalization, fewer complications and lower recurrent rates. If the primary pyeloplasty is an open operation in retroperitoneal approach, the traditional laparoscopic and robotic operation with retroperitoneal approach should be considered for secondary repair. The cause of recurrent UPJO should be evaluated before surgery and identified intraoperatively to minimize the possibility of recurrence.

Robot-assisted laparoscopic upper urinary tract reconstruction surgery: A review of 108 cases by a single surgeon / 北京大学学报(医学版)

OBJECTIVE@#To summarize the experiences and outcomes of 108 robot-assisted laparoscopic upper urinary tract reconstruction surgeries conducted by a single surgeon.@*METHODS@#We consecutively and retrospectively reviewed 108 patients who underwent robot-assisted laparoscopic upper urinary tract reconstruction surgeries by a single surgeon from November 2018 to January 2020. The patient demographics, perioperative variables, postoperative complications and follow-up data were recorded. Fifty-three modified dismembered pyeloplasties (MDP), 11 spiral flap pyeloplasties (SFP), 11 ure-teroureterostomies (UUT), 4 lingual mucosal onlay graft ureteroplasties (LMU), 5 appendiceal onlay flap ureteroplasties (AU), 11 ureteral reimplantations (UR), 6 Boari flap-Psoas hitch surgeries (BPS) and 7 ileal ureter replacements (IUR) were enrolled finally. The success was defined as the improvement in subjective pain levels, and the improvement in the degree of hydronephrosis at ultrasound.@*RESULTS@#All the surgeries were successfully completed without open or laparoscopic conversion. The median operative time was 141 min (range: 74-368 min), median blood loss was 20 mL (range: 10-350 mL) and median hospital stay was 4 d (range: 3-19 d) in MDP group, with the success rate of 94.3%. The median operative time was 159 min (range: 110-222 min), median blood loss was 50 mL (range: 20-150 mL) and median hospital stay was 5 d (range: 3-8 d) in SFP group, with the success rate of 100%. The median operative time was 126 min (range: 76-160 d), median blood loss was 20 mL (range: 10-50 mL) and median hospital stay was 5 d (range: 4-9 d) in UUT group, with the success rate of 100%. The median operative time was 204 min (range: 154-250 min), median blood loss was 30 mL (range: 10-100 mL) and median hospital stay was 6 d (range: 4-7 d) in LMU group, with the success rate of 100%. The median operative time was 164 min (range: 135-211 min), median blood loss was 75 mL (range: 50-200 mL) and median hospital stay was 8.5 d (range: 6-12 d) in AU group, with the success rate of 100%. The median operative time was 149 min (range: 100-218 min), median blood loss was 20 mL (range: 10-50 mL) and median hospital stay was 7 d (range: 5-10 d) in UR group, with the success rate of 90.9%. The median operative time was 166 min (range: 137-205 min), median blood loss was 45 mL (range: 20-100 mL) and median hospital stay was 5 d (range: 4-41 d) in BPS group, with the success rate of 83.3%. The median operative time was 270 min (range: 227-335 min), median blood loss was 100 mL (range: 10-100 mL) and median hospital stay was 7 d (range: 5-26 d) in IUR group, with the success rate of 85.7%.@*CONCLUSIONS@#The surgeon performed and modified numerous complicated upper urinary tract reconstruction surgeries by the robotic platform, which facilitated the development of the standardized upper urinary tract reconstruction surgical technique.

Advances in surgical repair of ureteral injury / 北京大学学报(医学版)

Ureteral injury can be classified as iatrogenic or traumatic, which represents a rare but challenging field of reconstructive urology. Due to their close proximity to vital abdominal and pelvic organs, the ureters are highly susceptible to iatrogenic injury, while ureteral injury caused by external trauma is relatively rare. The signs of ureteric injury are difficult to identify initially and often present after a delay. The treatment of ureteral injury, which is depended on the type, location, and degree of injury, the time of diagnosis and the patient's overall clinical condition, ranges from simple endoscopic management to complex surgical reconstruction. And long defect of the ureter presents much greater challenges to urologists. Ureterotomy under endoscopy using laser or cold-knife is available for the treatment of 2-3 cm benign ureteral injuries or strictures. Pyeloplasty is an effective treatment for ureteropelvic junction obstruction and some improved methods showed the possibility of repairing long-segment (10-15 cm) stenosis. Proximal and mid-ureteral injuries or strictures of 2-3 cm long can often be managed by primary ureteroureterostomy. When not feasible due to ureteral defects of longer segment, mobilization of the kidney should be considered, and transureteroureterostomy is alternative if the proximal ureter is of sufficient length. And autotransplantation or nephrectomy is regarded as the last resorts. Most of the injuries or strictures are observed in the distal ureter, below the pelvic brim, and are usually treated with ureteroneocystostomy. A non-refluxing technique together with a ureteral nipple or submucosal tunnel method, is preferable as it minimizes vesico-ureteral reflux and the risk of infection. In order to cover a longer distance, ureteroneocystostomy in combination with a psoas hitch (covering 6-10 cm of defect) or a Boari flap (covering 12-15 cm) is often adopted. Among various ureteral replacement procedures, only intestinal ureteral substitution, which includes ileal ureter, appendiceal interposition and reconfigured colon substitution, has gained wide acceptance when urothelial tissue is insufficient. Ileal ureter can be used to replace the ureter of >15 cm defect and even to replace the entire unbilateral ureter or bilateral ureter. Laparoscopic and robotic-assisted techniques are increasingly being employed for ureteral reconstruction and adopted with encouraging results.