Objective:To explore the clinical characteristics and prognosis of primary renal dedifferentiated liposarcoma.Methods:A retrospective analysis was conducted on the clinical data of 10 patients diagnosed with renal dedifferentiated liposarcoma in the First Affiliated Hospital of Zhengzhou University from January 2017 to December 2023. The cohort consisted of 8 males and 2 females, with a mean age of (59.0±6.8) years. Tumors were located in the left kidney in 8 cases and in the right kidney in 2 cases. Presentations included flank or abdominal masses in 4 patients, back pain in 3 patients, and asymptomatic in 3 patients. Imaging studies revealed solitary lesions in 9 cases and multiple lesions in 1 case. The maximum tumor diameter ranged from 95 to 178 mm, with a median of 119.5 mm. CT showed tumors within the renal parenchyma with unclear boundaries and displayed a "slow in, slow out" pattern of gradual enhancement. Clinical staging revealed T 2N 0M 0 in 3 cases, T 3N 0M 0 in 4 cases, T 4N 0M 0 in 3 cases, and T 2N 1M 0 in 1 case. Nine patients underwent radical nephrectomy, including 4 laparoscopic surgeries, 4 open surgeries, and 1 robotic-assisted with thrombectomy. One patient, presenting with multiple lymph node metastases confirmed by PET-CT, underwent a diagnostic biopsy. Postoperative pathological features, follow-up and prognosis were analyzed. Results:Pathological specimens appeared grayish-red, grayish-yellow or grayish-white cut surfaces with soft to moderate texture. Microscopically, tumor cells were ovoid or short spindle-shaped with significant atypia and cytoplasmic vacuoles, with visible pathological mitoses. Six cases showed only dedifferentiated components with tumor giant cells and multinucleated giant cells resembling pleomorphic undifferentiated sarcoma, with focal tumor necrosis. Fluorescence in situ hybridization showed MDM2 gene amplification in all cases. All cases were diagnosed as primary renal dedifferentiated liposarcoma. Pathological staging showed 4 cases as pT 2N 0M 0, 3 cases as pT 3N 0M 0, 2 cases as pT 4N 0M 0, and 1 case lacked pathological staging due to biopsy only. Five patients received postoperative adjuvant therapy, including two pT 2N 0M 0 cases who received immunotherapy and apatinib treatment respectively, with no recurrence. One pT 3N 0M 0 case received anlotinib treatment, with local recurrence after 12 months, followed by radiofrequency ablation combined with chemotherapy. Two pT 4N 0M 0 cases received ifosfamide combined with epirubicin and pirarubicin combined with lobaplatin respectively, with one case showing no progression at 11 months follow-up, and another case developing splenic metastasis 3 months post-surgery, followed by chemotherapy combined with targeted therapy, surviving for 20 months. Among the 4 cases without adjuvant therapy, two pT 2N 0M 0 cases developed multiple metastases within 1 month post-surgery and received immunotherapy combined with targeted therapy and/or chemotherapy, surviving 4-5 months.One of the two pT 3N 0M 0 cases developed local recurrence 2 months post-surgery and received chemotherapy, surviving 6 months, and another pT 3N 0M 0 case developed gluteal subcutaneous metastasis 1 month post-surgery and received immunotherapy combined with targeted therapy, surviving 8 months.One non-surgical pT 2N 1M 0 patient received chemotherapy and survived for 15 months. All patients were followed up for 4-52 months, with a median follow-up time of 11 months. At the last follow-up, 6 patients died and 4 survived. Conclusions:Primary renal dedifferentiated liposarcoma is clinically rare, with atypical symptoms and difficult preoperative diagnosis, relying on pathology for confirmation. Radical nephrectomy is the main treatment method, but surgery alone has poor prognosis with high recurrence and metastasis rates. Adjuvant therapy based on surgery may improve patient prognosis.Larger sample studies are needed for confirmation.

Objective:To explore the clinical characteristics and prognosis of primary renal dedifferentiated liposarcoma.Methods:A retrospective analysis was conducted on the clinical data of 10 patients diagnosed with renal dedifferentiated liposarcoma in the First Affiliated Hospital of Zhengzhou University from January 2017 to December 2023. The cohort consisted of 8 males and 2 females, with a mean age of (59.0±6.8) years. Tumors were located in the left kidney in 8 cases and in the right kidney in 2 cases. Presentations included flank or abdominal masses in 4 patients, back pain in 3 patients, and asymptomatic in 3 patients. Imaging studies revealed solitary lesions in 9 cases and multiple lesions in 1 case. The maximum tumor diameter ranged from 95 to 178 mm, with a median of 119.5 mm. CT showed tumors within the renal parenchyma with unclear boundaries and displayed a "slow in, slow out" pattern of gradual enhancement. Clinical staging revealed T 2N 0M 0 in 3 cases, T 3N 0M 0 in 4 cases, T 4N 0M 0 in 3 cases, and T 2N 1M 0 in 1 case. Nine patients underwent radical nephrectomy, including 4 laparoscopic surgeries, 4 open surgeries, and 1 robotic-assisted with thrombectomy. One patient, presenting with multiple lymph node metastases confirmed by PET-CT, underwent a diagnostic biopsy. Postoperative pathological features, follow-up and prognosis were analyzed. Results:Pathological specimens appeared grayish-red, grayish-yellow or grayish-white cut surfaces with soft to moderate texture. Microscopically, tumor cells were ovoid or short spindle-shaped with significant atypia and cytoplasmic vacuoles, with visible pathological mitoses. Six cases showed only dedifferentiated components with tumor giant cells and multinucleated giant cells resembling pleomorphic undifferentiated sarcoma, with focal tumor necrosis. Fluorescence in situ hybridization showed MDM2 gene amplification in all cases. All cases were diagnosed as primary renal dedifferentiated liposarcoma. Pathological staging showed 4 cases as pT 2N 0M 0, 3 cases as pT 3N 0M 0, 2 cases as pT 4N 0M 0, and 1 case lacked pathological staging due to biopsy only. Five patients received postoperative adjuvant therapy, including two pT 2N 0M 0 cases who received immunotherapy and apatinib treatment respectively, with no recurrence. One pT 3N 0M 0 case received anlotinib treatment, with local recurrence after 12 months, followed by radiofrequency ablation combined with chemotherapy. Two pT 4N 0M 0 cases received ifosfamide combined with epirubicin and pirarubicin combined with lobaplatin respectively, with one case showing no progression at 11 months follow-up, and another case developing splenic metastasis 3 months post-surgery, followed by chemotherapy combined with targeted therapy, surviving for 20 months. Among the 4 cases without adjuvant therapy, two pT 2N 0M 0 cases developed multiple metastases within 1 month post-surgery and received immunotherapy combined with targeted therapy and/or chemotherapy, surviving 4-5 months.One of the two pT 3N 0M 0 cases developed local recurrence 2 months post-surgery and received chemotherapy, surviving 6 months, and another pT 3N 0M 0 case developed gluteal subcutaneous metastasis 1 month post-surgery and received immunotherapy combined with targeted therapy, surviving 8 months.One non-surgical pT 2N 1M 0 patient received chemotherapy and survived for 15 months. All patients were followed up for 4-52 months, with a median follow-up time of 11 months. At the last follow-up, 6 patients died and 4 survived. Conclusions:Primary renal dedifferentiated liposarcoma is clinically rare, with atypical symptoms and difficult preoperative diagnosis, relying on pathology for confirmation. Radical nephrectomy is the main treatment method, but surgery alone has poor prognosis with high recurrence and metastasis rates. Adjuvant therapy based on surgery may improve patient prognosis.Larger sample studies are needed for confirmation.

Malacoplakia is a rare granuloma disease mainly occurred in the urinary system, it is even rarer for renal malacoplakia invading the descending colon complicated with bladder malacoplakia. In this study, one such case was reported. Imaging examination suggested that the left kidney was a large patchy mixed density shadow, and enhancement scan lesion was uneven enhancement. CT guided renal puncture biopsy was performed, and postoperative pathology suggested renal malacoplakia. Transurethral cystoscopy was performed, and postoperative pathology confirmed that it was malacoplakia of the bladder. The effect of conservative antibiotic treatment was not good. The patient underwent radical nephrectomy + left hemicolectomy under general anesthesia, and postoperative pathology confirmed the diagnosis of renal malacoplakia, which involved the mucosa of the intestinal tube and the entire muscular layer. The patient was followed up for 6 months after surgery, and no recurrence was seen on CT.

Objective:To explore the clinicopathological features and prognosis of renal mucinous tubular spindle cell carcinoma (MTSCC).Methods:The clinical data of 16 patients with MTSCC admitted to the First Affiliated Hospital of Zhengzhou University from July 2013 to July 2022 were retrospectively analyzed. There were six male cases and ten female cases. The mean age was (56.4±11.4) years old. Among them, 10 cases were asymptomatic, two complained of hematuria, three complained of lumbar pain, and one complained of lower limb pain. Twelve cases underwent preoperative enhanced CT examination, 6 cases of ultrasound examination, 3 cases of MRI examination, and 1 case of bone scan. Imaging manifestations showed that the masses were round or round-like with clear borders. Two cases combined with hemorrhage and three cases combined with calcification. Five cases showed exophytic growth, 10 cases partially exophytic, and 1 case completely endophytic. The maximum diameter of the tumor was (65.7±27.4) mm. The tumors were located in the left kidney in 11 cases and in the right kidney in 5 cases. The tumors were mildly delayed-enhancing under enhanced CT, long/short T1 signal mixed with long/short T2 signal under MRI, and diffusion-limited high signal under DWI. The tumors were hypoechoic masses without obvious blood flow signals under ultrasound. Twelve cases were diagnosed as renal occupying neoplasms, 2 cases were suggested as lack of blood supply renal tumor, and one was considered renal tumor rupture and bleeding. In one case, a bone scan suggested metastasis to the thoracic spine and pelvis. The metastatic renal tumor was diagnosed, and a renal puncture was performed to clarify the pathology. Eleven patients underwent laparoscopic radical nephrectomy, and 4 patients underwent partial nephrectomy. One case was metastasized without surgery and treated with apatinib mesylate and zoledronic acid.Results:The postoperative pathological specimens showed grayish, grayish-yellow, or grayish-red masses with a soft or medium texture. No perinephric, ureteral, or adrenal invasion was seen in all tumors. Microscopically, the tumor cells were round and ovoid. The tumor cells were arranged in tubular and striated shapes, and mucus pools were locally visible. No sarcomatous component was seen in all tumors. There were 9 patients with pT 1N 0M 0, 6 patients with pT 2N 0M 0, and 1 patient with pT 1N 0M 1. After operation, 2 patients with pT 2N 0M 0, who underwent laparoscopic radical nephrectomy were treated with pazopanib and sunitinib, respectively. All patients were followed up for a median of 50.7(25.8, 75.0)months, 15 patients were free of recurrent metastases, and 1 patient with pre-puncture metastasis died due to tumor progression of multiple pulmonary and bone metastases, with a survival of 16.9 months. Conclusions:Renal MTSCC is rare, mostly found on physical examination, with female patients predominantly, and imaging shows a lack of blood supply tumor. Surgery is the primary treatment method. Partial nephrectomy or radical nephrectomy could be chosen according to the tumor stage, kidney function, and patient's underlying condition, and patients have a good prognosis.

Objective:To discuss the treatment and prognosis of children with torsion of inguinal cryptorchidism.Methods:Clinical data of 15 cases of inguinal cryptorchidism with testicular torsion admitted to Beijing Children's Hospital from October 2015 to February 2019 were retrospectively analyzed. The average age of onset was 5.1 years (4 months-17 years). The clinical manifestations included inguinal swelling and pain in 13 cases, abdominal pain in 1 case and abdominal pain with vomiting in 1 case. The average duration of symptoms (up to the time of surgery) was 45.9 (5-170) h. Physical examination showed scrotal voided on affected side, groin swelling with tenderness. Lesions were found in 12 cases on the left and 3 cases on the right. Groin color Doppler ultrasound examination showed testis enlargement, uneven echo, decrease or disappearance of blood flow signal, suspected testicular torsion. Clinical diagnosis was inguinal cryptorchidism testicular torsion. Inguinal canal testicular exploration under general anesthesia. During the operation, the testicle was dark and twisted with an average torsion of 600°(180°-1 080°). Testicular activity was observed after exposure and reduction and torsion. After reduction, testicular color was immediately restored in 5 cases. After cutting open the white membrane of the testicular, there was blood outflow. In 2 cases, the testis was found to be inactive during the operation, but the parents requested to retain the testis and performed orchiopexy. 8 cases underwent orchiectomy due to necrosis of testis. The mean symptom duration (up to the time of operation) of the orchiectomy group and the orchiectomy group was 24.3 (5-73) h and 64.8 (7-170) h, respectively. The average torsion degree was 514° and 675°, respectively.Results:The average operative time of 15 patients was 56.7 min (40-85 min). Intraoperative blood loss averaged 1.5 (1-2) ml. There were no complications during and after operation. Postoperative follow-up averaged 27 (8-47) months. Among the 7 patients in the orchiopexy group, 3 patients had atrophy of testis 7.3 months after operation, and the remaining 4 patients had normal testis. In the orchiectomy group, 8 cases had normal contralateral testis without torsion.Conclusions:Inguinal cryptorchidism testicular torsion is a relatively rare disease in children. Color Doppler ultrasonography and inguinal canal exploration can help diagnosis and treatment. According to the condition of testis, orchiopexy or orchiectomy can be performed. Early diagnosis of this disease is difficult, easy to lead to testicular loss or postoperative atrophy.

Objective To investigate the timing and scheme of surgical treatment for the concomitant ureteropelvic junction obstruction(UPJO) and congenital abnormalities of the kidney.Methods The clinical data of 155 patients with concomitant UPJO and congenital abnormalities of the kidney from January 2006 to January 2016 was retrospectively analyzed.There were 107 males and 48 females,who aged 6 months to 16 years and 6 months.The average time was 5 years and 9 months old when they received operation.There were 8 cases less than 1 year old.There were 93 cases of UPJO on the left side,54 cases on the right side,and 8 cases on both sides.There were 33 cases with duplication of kidney,19 cases with solitary kidney,and 6 cases with renal dysplasia,6 cases with renal ectopia,12 cases with polycystic kidney disease,and 41 cases with dysplasia;2 cases with renal malrotation.There were 100 cases with symptoms such as fever,abdominal pain,vomiting.5 cases had received Anderson-Hynes pyeloplasty in other hospitals,2 cases received nephrectomy with symptoms did not relieve.4 cases were treated with nephrostomy in other hospital.Children with the repeated clinical symptoms,or renal function decreased significantly,or hydronephrosis progressive to the anteroposterior diameter of more than 30 mm received surgical treatment.Results There were 140 cases received Anderson-Hynes pyeloplasty,and 8 cases received nephrectomy with 5 cases were UPJO side and 3 cases were only abnormalities of the kidney without UPJO.All patients received IVP or ultrasonography postoperative 3-6 months,which showed hydronephrosis improved or no obvious change,and 4 cases were improved obviously.The IVP results showed that 5 patients with renal dysplasia together with UPJO had the renal function improved.There were 128 cases followed up for 12 to 106 months,with an average of 64.5 months.All patients had no clinical symptoms.83 cases were reexamined by IVP or ultrasonography,and hydronephrosis was getting better or no change.Conclusions The patients with concomitant UPJO and congenital abnormalities of the kidney don't need surgery in advance.The best choice for those patients is Anderson-Hynes pyeloplasty.The indication of nephrectomy should be considered carefully.

Objective: To analyze the clinical pathologic characteristics of cases with fluorescence in situ hybridization (FISH) positive of exfoliated urothelial cells, so as to evaluate the clinical utility of FISH in the diagnosis of urothelial carcinoma (UC).Methods: A total of 271 cases of FISH positive in Department of Urology of Peking University First Hospital from Apr.2012 to Sep.2015 were recruited in this study.Retrospective analysis was made on their clinical data.For FISH analysis, labeled probes specific for chromosomes 3, 7, 17, and the p16 (9p21) gene were used to assess chromosomal abnormalities indicative of malignancy.The positive predict values (PPV) of all the techniques were analyzed.Results: Of the 271 patients, 207 cases were UC, 7 cases were non-UC, and 57 cases were benign diseases.The PPV of FISH in detecting UC was 76.4%, while the 95% confidence interval (CI) 71.3% to 81.5%.In the cohort of FISH positive, this value was similar to that of urinary cytology (PPV 86.8%, 95% CI: 78.5%-95.0%).The PPV of FISH was lower than that of cystoscopy and ureteroscopy (PPV 96.1%, 95% CI: 91.7%-100.0%).There were significant differences between this study and the PPV of FISH reported abroad (PPV 53.9%, χ2=33.048, P<0.001).Of all the UC with FISH positive, bladder cancer showed an earlier pathological stage versus renal pelvic carcinoma and ureteral carcinoma, with significance (χ2=5.894, P=0.015, and χ2=13.601, P<0.001, respectively).However, no difference was found in the size, pathological stage and pathological grade of tumors between the urinary cytology positive group and the urinary cytology negative group.The rate of high-grade UC in ureteral carcinoma of FISH positive was 92.3%, much higher than that of ureteral carcinoma reported domestically.Conclusion: The PPV of FISH in detecting UC is higher relatively, with a better clinic value for Chinese patients.The ureteral carcinoma with FISH positive obtains a higher pathological grade, which is of great guiding significance for UC.

Birt–Hogg–Dubé (BHD) syndrome, a hereditary renal cancer syndrome caused by mutations in the folliculin (FLCN) gene, is characterized by the presence of fibrofolliculomas, pulmonary cysts, spontaneous pneumothorax, and renal cell carcinoma (RCC). Few BHD syndrome cases have been reported in Asian countries, and cutaneous presenta-tions are relatively rare in Asian patients. Asian BHD patients may be misdiagnosed due to their atypical manifesta-tions. Here, we report two Chinese BHD patients with novel FLCN mutations (c.946-947delAG in exon 9 and c.770-772delCCT in exon 7). Both of them had RCC and spontaneous pneumothorax without fibrofolliculomas. In patients with RCC and pulmonary cysts but without cutaneous lesions, screening for mutations in the FLCN gene should be performed, especially for those with a family history of RCC or pulmonary cysts (pneumothorax).

Objective To compare the treatment efficacy of severe hypospadias between two-stage urethral plate reconstruction procedure and two-stage tubularized transverse preputial island flap procedure. Methods From 2010 December to 2014 December, we retrospectively analyzed 82 cases with severe hypospadias, using two-stage urethral plate reconstruction and two-stage tubularized transverse preputial island flap urethroplasty, respectively.Their mean age in the first operation was 2.4 years, ranged from 2 to 3 years.Hypospadias were penoscrotal type in 32 cases, perineal type in 50 cases.In group A, 42 cases, including penoscrotal type in 15 cases, perineal type in 27 cases, with urethral plate reconstruction procedure underwent two-stage urethroplasty.The transected ventral penile urethral plate and to strip the ventral fibrous tissue were needed in correction of chordee of penis.The patients in group A were underwent the second urethroplasty with urethral plate reconstruction procedure 6-12 months later.In group B, 40 cases,including penoscrotal type in 17 cases, perineal type in 23 cases, who underwent one stage operation in the correction of chordee of penis, only with tubularized preputial island flap forming the part of the urethra.The patients in group B were underwent the second urethroplasty 6 ~12 months later.Results Patients were followed up for 1 to 5 years, mean 3.5 years.No recurrence of penis chordee was noticed.In group A, 6 cases (14.3%) had postoperative fistula.The second fistula repairing was perform 6 to 12 months after the second stage operation.However, still 3 cases were noticed the fistula recurrence, which was cured after the fourth operation.In group B, 3 cases (7.5%) developed fistula, which appeared in the second urethroplasty (P<0.01).They were all treated after the second repairing procedure.In group A, 4 cases were noticed urethral stricture, the urethral stricture rate was 9.5%.The stricture occurred 1 to 3 months after the second operation.After dilation and indwelling catheter, 3 cases resolved the problem. Another case still found the recurrence after 2 months, which the one stage urethral plication and second urethral reconstruction were considered to treat this patient.In group B, 1 cases of urethral stricture, the urethral stricture rate was 2.5%(P<0.01).After dilation and indwelling catheter, this case resolved the problem.In group A, 3 cases had urethral diverticulum, which occurred at a rate of 7.1%, whereas without urethral diverticulum occurred in group B (P<0.01).Those patient with urethral diverticulum accepted the diverticulum removing procedure 3 to 6 months after the second stage procedure.One case was found the urethral fistula, which was treated one year later.The success rate of operation was 69.0%in group A, the success rate of the operation was 90.0%in group B.The difference of the success rate between two groups was statistically significant ( P <0.01 ) . Conclusions With respect to two-stage urethral plate reconstruction procedure in treatment of severe hypospadias, there were the low complication rates of postoperative urinary fistula, urethral stricture and diverticulum in two-stage tubularized transverse preputial island flap procedure.Urethral meatus could be done at the glans of the penis.In addition, the two-stage tubularized transverse preputial island flap procedure provides a good opportunity to practice to master complex hypospadias operation.

AIM:To analyze the relationship between epicardial adipose tissue (EAT) thickness and plasma N-terminal pro-B-type natriuretic peptide ( NT-proBNP ) level in the patients with stable coronary artery disease . METHODS:The patients with chest pain ( n=115) admitted to our hospital underwent coronary artery computer tomo-graphy and further underwent coronary angiography for confirming whether they had coronary artery disease .EAT thickness was evaluated at the right ventricular free wall imaged by coronary artery computer tomography .Plasma NT-proBNP level was examined by an automatic biochemistry analyzer .RESULTS:Eighty-one patients were confirmed to have stable coro-nary artery disease and thirty-four patients were excluded to have coronary artery disease .Left ventricular ejection fraction of these patients of 2 groups were all normal.The natural logarithm of plasma NT-proBNP level [ln(NT-proBNP)] of the patients with stable coronary artery disease was significantly higher than that of the patients without coronary artery disease (P<0.05).EAT thickness of the patients with stable coronary artery disease was also higher than that of the patients with -out coronary artery disease(P<0.05).EAT thickness was related to ln(NT-proBNP) positively (P<0.05).After adjust-ment of related impact factors , EAT thickness was still related to ln (NT-proBNP) positively (P<0.05).Multiple-factor regression analysis showed that EAT thickness was the independent influence factor on LnNT -proBNP (P<0.05).CON-CLUSION:EAT thickness and plasma NT-proBNP level are both increased significantly and is related to each other in the patients with stable coronary artery disease .