Squamous Cell Carcinoma of the Kidney: A Large Case Series.

OBJECTIVES: We present our experience with and data about a very rare neoplasm of the kidney, squamous cell carcinoma (SCC). METHODS: A total of 14 patients with a diagnosis of SCC were identified on the basis of a retrospective analysis of medical records of patients who underwent surgery for renal cancers between 2015 and 2021 at the Sindh Institute of Urology and Transplantation. IBM SPSS v25 was used to record and analyze data. RESULTS: Most patients found to have SCC of the kidney were male (71.4%). The mean (SD) patient age was 56 (13.7) years. Flank pain was the most common presenting symptom (n = 11; 78.6%) followed by fever (n = 6; 42.9%). Only 4 (28.5%) of the 14 patients had a preoperatively established diagnosis of SCC; the remaining 10 (71.4%) had an incidental finding of SCC on their histopathology specimen. The mean (SD) overall survival was 5 (4.5) months. CONCLUSIONS: SCC of the kidney is a rare upper urinary tract neoplasm reported in the literature. The gradual onset of vague symptoms, lack of pathognomonic signs, and inconclusive radiological features make the disease unsuspected in most cases, therefore delaying diagnosis and treatment. It usually presents at an advanced stage, and the prognosis is often poor. A high index of suspicion is warranted in patients with chronic kidney stone disease.

Mixed epithelial and stromal tumour of the kidney masquerading as hydatid cyst: a rare case report from the developing world.

Mixed epithelial and stromal tumour (MEST) is a rare entity with an incidence of 0.2% among renal cancers. It has strong predilection towards females with a 1:6 male to female ratio, the tumour is cystic with some solid component with biphasic proliferation of stromal and epithelial cells. The case of a 37-years-old female is presented with right lumbar pain since 3 months. The family history was unremarkable. The routine workup revealed mild neutrophilia and borderline Echinococcus antibody titres. Ultrasound revealed a complex cystic lesion with a solid component in the right kidney. CT scan with contrast confirmed a multiloculated mixed density lesion with daughter cysts arising from the middle lobe of the right kidney. Initial diagnosis of renal hydatid cyst was established and she underwent partial nephrectomy with excision of the cystic mass. Surprisingly the histopathology revealed mixed epithelial and stromal tumour.

Inflammatory myofibroblastic tumour of genitourinary system.

Two cases of inflammatory myofibroblastic tumour (IMT) involving the genitourinary system are presented. One patient with mass of urinary bladder came in with lower abdominal pain whereas the second patient complained of right flank pain and investigations showed a mass involving the right kidney. At present, no specific guidelines exist for the management of inflammatory myofibroblastic tumours. In this case report, we will discuss the work-up and the management stratégies adopted for each case.

Percutaneous Nephrolithotomy in Anomalous Kidney.

OBJECTIVE: To assess the stone-free rate (SFR) subsequent to percutaneous nephrolithotomy (PCNL) in patients with anatomically anomalous kidneys. STUDY DESIGN: Cross-sectional study. Place and Duration of the Study: Department of Urology, Sindh Institute of Urology and Transplantation Karachi, from 23 July 2020 till 30 October 2021. METHODOLOGY: Sixty-five patients of renal stone disease with abnormal kidneys, aged 18-60 years of both genders were enrolled in this cross-sectional study. Demographic information like age, gender, stone size, duration of disease, and type of abnormality were noted. After PCNL, stone-free status was determined after 2 weeks of the procedure by performing ultrasound KUB. RESULTS: A total of 65 patients were included in this study with mean age of 36.37 ± 12.86 years [Range: 18-60]. There were 76.9% of males and 23.1% of females. Regarding anatomical malformation, 46.2% were malrotated kidneys, 16.9% were horseshoe kidneys, 16.9% were partial Duplex system, 9.2% had bifid pelvis. Median duration of the disease was 12 (IQR=10). Forty-one patients (63.1%) had single and 24 (36.9%) had multiple number of stones with average size of 3.26 ± 1.14 cm. SFR after PCNL in patients with abnormal kidneys was 70.77% (46/65) while 29.23% (19/65) were observed with residual fragments. Out of 19 cases with residual fragments, 10 (53.2%) had stone size <1 cm and 9 (47.4%) had stone of 1 to 3 cm. Sixteen out of 19 patients with residual stones were treated with ESWL (most required: one session), and re-do PCNL was performed in three cases. CONCLUSION: PCNL is an effective and safe operation in anatomically anomalous kidneys. For satisfactory outcomes, it requires extreme care and exceptional surgical skill. KEY WORDS: Malrotated kidney, Duplex system, Kidney anomaly, Horseshoe kidney, PCNL, Extracorporeal shockwave lithotripsy (ESWL).

Outcomes of Nephron-sparing Surgery in T1 vs T2 Renal Tumours.

OBJECTIVE:  To compare the drop in eGFR after nephron-sparing surgery in T1 and T2 renal tumours. STUDY DESIGN:  Descriptive study. PLACE AND DURATION OF STUDY:  Department of Uro-oncology, Sindh Institute of Urology, and Transplantation Karachi, from March 2020 to March 2021. METHODOLOGY:  Retrospective data were collected for all patients who underwent nephron-sparing surgery between 2014 to 2019. Eighty-seven patients were divided into two groups based on the T stage of renal tumours (T1 ≤7 cm and T2 >7 cm). The outcomes of the two groups were compared such as eGFR, blood transfusion, hospital stay and complications. RESULTS:  There was a higher drop in eGFR in T2 tumours when compared to T1 tumours at 1 year of follow-up. There were more perioperative complications, higher blood transfusions and longer hospital stays for T2 tumours. CONCLUSION:  Nephron sparing surgery for T2 renal tumours carries lower eGFR preservation, higher blood transfusions and complications when compared to T1 tumours. The indication for such extensive surgery should be individualized to specific contexts only. KEY WORDS: Adenocarcinoma kidney, Nephrectomy, Glomerular filtration rate, Length of hospital stay, Blood transfusion.

Excision of Postchemotherapy Residual Retroperitoneal Mass in Testicular Cancer.

In this study, patients, who underwent excision of retroperitoneal mass following chemotherapy for testicular cancers from 2006 to 2016, were studied and followed till 2021. The clinical and oncological outcomes were measured. Among 338 patients, who were treated for TC during the entire study period, 38 (11.2%) underwent excision of the residual retroperitoneal mass. The mean age of these patients was 26.9±6.3 years. The majority were stage 3 testicular cancer. Complete resection with negative microscopic margins was achieved in the majority of cases i.e. 31 (81.6%). Damage to the collateral structures was seen in 12 (31.6%) cases. On histopathological examination, teratoma was seen in 18 (47.4%) of the cases followed by necrosis in 10 (26.3%). Complications were recorded in 12 (31.5%) cases, most were low grades. At five years of follow-up, 19 (50%) were disease-free, 25 (65.8%) were alive, and 9 (23.6%) were lost to follow-up. Five-year survival rates are lower than standard template-based retroperitoneal dissection, however, complications rates are comparable. Such complex surgeries should only be performed in high-volume centres. Key Words: Testicular neoplasms, Lymph node excision, Seminoma, Germ cell and embryonal.

Management of stone disease: 17 years experience of a stone clinic in a developing country.

OBJECTIVE: To review 17 years experience of the stone clinic with incorporating the changes in practice over the years and to report the benefits of stone clinic in a developing country. METHODS: The SIUT Stone clinic was established in 1990 with installation of HM4 Lithotriptor. This clinic is run jointly by a Urologist, Dietitian, Nephrologist, Biochemist and Radiologist. From 1990 - 2007, about 38,749 stone patients received treatment with ESWL (55%), PCNL (6.0%), URS (15.5%), litholopaxy 4.0% and open surgery 19.7%. These patients after treatment were followed in the stone clinic with stone analysis and 24 hours urine metabolic studies where indicated. Dietary and oral hydration programme combined with medical therapy was also instituted. Recurrence rate was noted in those patients who were advised diet modification, oral hydration and medical treatment. Complications of stone disease were documented during the follow-up period. RESULTS: In ESWL group 8226 patients were followed in the stone clinic for 5 years. In this group, 185 (2.2%) had recurrence of renal calculi. In PCNL group 1306 patients were followed, and 16 (1.1%) had recurrence. In the open surgery group of 1294 patients, 17 (1.3%) had recurrence of renal calculi. The complications of stone disease noted were renal failure and infections in 162 (1.5%) patients, during the follow-up period. CONCLUSION: This study shows the beneficial effect of a stone clinic in a developing country and positive effect on the reduction of recurrence and complications of stone disease. Our experience promotes the need to open more specialized stone clinics in areas where stone disease is highly prevalent.

Adrenal myelolipoma: a report of three cases and review of literature.

Adrenal myelolipoma is a rare and benign tumour composed of mature adipose tissue and haematopoietic elements that resemble bone marrow. It is mostly discovered incidentally on imaging of abdomen done for non adrenal related reasons or at autopsy. Usually asymptomatic, but has been reported to present with symptoms such as flank pain resulting from tumour bulk, necrosis or spontaneous retroperitoneal haemorrhage. Symptomatic tumours, growing tumours or tumours larger than 10 cm should be excised surgically. We report cases of 3 male patients presenting with flank pain and upper pole renal masses. All three were treated surgically with adrenalectomy. Myelolipoma was confirmed in all three on histology.

Comparison of holmium: Yag laser and pneumatic lithoclast in percutaneous nephrolithotomy.

OBJECTIVE: To compare holmium Yag laser with lithoclast in patients with renal stones undergoing percutaneous nephrolithotomy (PCNL). METHODS: A comparative cross sectional study was performed on 60 patients undergoing PCNL. Patients with a 2.5 cm stone in renal pelvis, having preoperative negative urine culture, no coagulopathy and fit for general anaesthesia were included, pregnant females were excluded. They were randomly divided into 2 groups of 30 cases each depending on the energy source used. Group A included cases of PCNL subjected to laser treatment and group B had patients undergoing PCNL with pneumatic lithoclast. For each group duration of procedure, any per operative or postoperative complication, residual stone, duration of hospital stay and cost of each procedure were recorded. RESULTS: The average operative time was 125.7 +/- 31.1 minutes in group A and 98.5 +/- 18.7 minutes in group B (P = 0.0001). The overall complication rate was similar in both groups being 13.3% in group A and 23.3% in group B. Residual stone was observed in 17% cases in group A and 13% cases in group B (P = 0.5). The mean postoperative hospital stay was 3.17 +/- 1.6 days in group A and 4 +/- 2.3 days in group B (P = 0.4). Cost analysis showed that the initial capital cost of equipment was 40,000 Euro in laser and 24,000 Euro in pneumatic lithoclast. However because of reuse of fiber the per procedure cost was 60 Euro in laser and 50 Euro in lithoclast group. CONCLUSION: In our experience we found that Holmium: YAG laser and pneumatic lithoclast are both effective and safe lithotriptors for percutaneous stone removal. More operating time was required in laser, more complications encountered with pneumatic lithoclast and a high initial cost of laser. However with increasing experience with laser, more promising results are expected with this new technology. The cost can be compensated by using it in other procedures especially at a public sector hospital.

Treatment of ureteric calculi--use of Holmium: YAG laser lithotripsy versus pneumatic lithoclast.

OBJECTIVE: To compare the efficacy of Holmium: YAG laser and pneumatic lithoclast in treating ureteric calculi. METHODS: The study included total of 100 patients divided into two equal groups of laser lithotripsy (LL) and pneumatic lithoclast (PL). Study was conducted between September 2006 and February 2007. Inclusion criteria were patients with a ureteric stone of size 1-2 cm and negative urine culture. An x-ray KUB was mandatory. IVU and CT pyelogram were also done when required. Procedures were done under general anaesthesia after a single dose of pre-operative antibiotic. A 7.5 Fr semi rigid ureteroscope was used for ureteroscopy in all cases. Holmium: YAG laser with 365 microm wide probe was employed in laser group and frequency was set between 5 and 10 Hz at a power of 10 to 15 W. Swiss lithoclast with single or multiple fire technique was used accordingly in PL group. Postoperatively patients underwent radiography and helical CT as required at 4th week of follow up to asses stone clearance. RESULTS: The mean patient age in LL and PL group was 38 +/- 10 and 40 +/- 10 years respectively. The male to female ratio and stone size were similar between the groups. Stone migration up in pelvicalyceal system occurred in two patients of LL group while in eight patients of PL group. JJ Stent was placed in 5(10%) patients in laser group where as 13 (26%) patients required it in pneumatic lithoclast group. Stone free rate at 4 weeks was 92% in laser group as compared to 82% in pneumatic lithoclast group. Hospital stay was more than 24 hours in 2 patients of laser group as compared to 5 patients of pneumatic lithoclast group. Complication rate was 4% in LL group whereas it was 14% in PL group. CONCLUSION: Holmium: YAG laser lithotripsy is a superior technology compared to pneumatic lithoclast in terms of rate of stone clearance and complications, especially in upper ureteric stones.

Angiomyolipoma of the scrotum: report of a rarely seen case and review of the literature.

Angiomyolipoma (AML) is a benign, histologically complex mesenchymal tumor arising mainly from the kidney and liver. The majority (80%) of these tumors arise as sporadic tumors, while 20% of them are associated with tuberous sclerosis. Extra-renal sites of AML, though rare, have been reported in literature. In this report, we describe a case of AML arising from the scrotal skin, and presenting as a scrotal mass. Although skin is the most commonly reported site after kidney and liver, scrotal skin AML presents as an intriguing mass in a region known for germ cell tumors which has been reported only once before. A 35-year-old male presented with scrotal swelling. His physical examination, laboratory investigations and imaging studies were non-specific. Excision of the lesion with subsequent histopathological examination revealed the true nature of the lesion. This lesion should be included in the differential diagnosis of scrotal masses.

Analysis of the glutathione S-transferase genes polymorphisms in the risk and prognosis of renal cell carcinomas. Case-control and meta-analysis.

BACKGROUND: The Glutathione S-transferases (GSTs) genes deletion polymorphisms have been associated with the progression of several cancers. The association studies between the 2 GSTs (GSTM1 and GSTT1) null polymorphisms with the susceptibility to renal cell carcinoma (RCC) have been inconclusive. Therefore, with the inclusion of our own data, we performed a comprehensive meta-analysis to assess the association between these 2 polymorphisms and the risk of RCC. METHODS: A systematic literature search was carried out for studies published in the PubMed, EMBASE, Cochrane library, and Google Scholar from 1997 to December 2014. Results were stated as pooled odds ratios (ORs) for nonparametric data after heterogeneity analysis with 95% CI using fixed effect or random effect model. RESULTS: We systematically selected 13 relevant studies after thorough searches from the databases. Data showed no association between the GSTM1 and the GSTT1 null genotypes and the risk of RCC (OR = 1.01; CI: 0.92-1.11; P = 0.89 for GSTM1 and OR = 1.14; CI: 0.91-1.42; P = 0.25 for GSTT1). No association was found when the data were stratified according to the geographical/ethnic basis, source of control, and the risk factor evaluation. Subgroup analysis of occupational exposure to pesticides showed an inverse association of the active genotypes of both GSTM1 and GSTT1 polymorphisms with the exposed group of RCC (P<0.00001 and P<0.00001, respectively). The combined null genotype of the GSTM1/GSTT1 significantly increased the susceptibility to RCC by 1.4-fold (P = 0.001). This association remained significant for the Asian populations in subgroup analysis (OR = 1.8; CI: 1.30-2.49; P = 0.0004). CONCLUSION: In conclusion, this meta-analysis suggests that the 2 GSTs deletion polymorphisms independently have no association with the risk of RCC. However, combination of both deletions increases the risk of developing the RCC.

Unique molecular alteration patterns in von Hippel-Lindau (VHL) gene in a cohort of sporadic renal cell carcinoma patients from Pakistan.

BACKGROUND: Renal cell carcinoma (RCC) is the most frequent form of kidney cancer in adults. Somatic mutations that inactivate the von Hippel-Lindau (VHL) gene are the most common cause of RCC. The frequencies of molecular changes in the VHL gene in RCCs vary among different populations. So far, a single chromosomal-based study has been reported from a South Asian population. This report presents, for the first time, the somatic changes and promoter hypermethylation in VHL in a cohort of 300 RCC patients from Pakistan. METHODS: To identify mutations in the VHL gene, direct DNA sequencing was carried out. Epigenetic silencing was investigated by using methylation-specific polymerase chain reaction. RESULTS: Our data showed molecular alterations in the VHL gene in 163 (54%) renal cell carcinoma patients. Somatic mutations were found in 87 (29%) patients and 35 novel mutations were identified. VHL promoter hyper-methylation analysis showed epigenetic changes in 106 (35%) out of 300 patients. Patients who had no evidence of molecular alterations in the VHL gene were significantly younger than patients who carried some molecular change. Molecular alterations in the VHL gene were not restricted to clear-cell RCCs (ccRCCs). CONCLUSIONS: This is the first report that identifies molecular aberrations in the VHL gene from a South Asian population. The frequency of somatic mutation is lower and that of promoter hypermethylation is higher when compared with data from other parts of the world. The data has important implications in the population-specific application of tailored preventive and therapeutic regimens in non-familial RCCs.

Association of a single-nucleotide polymorphism in the promoter region of the VEGF gene with the risk of renal cell carcinoma.

AIMS: Vascular endothelial growth factor (VEGF) protein plays an important role in tumor development and progression. Polymorphisms in the VEGF gene may lead to over- or underexpression of the protein and may be associated with either risk or progression of malignancy. The aim of this case-control study is to identify and quantify the correlation between VEGF polymorphisms and renal cell carcinoma (RCC). RESULTS: Restriction fragment length polymorphism methods were used for the analysis of VEGF polymorphisms at -2578 and +936 positions in the promoter and 3'-untranslated regions, respectively. The VEGF -2578 A-allele was associated with an increased risk of RCC (odds ratio: 1.6; 95% CI: 1.2-2.3) and A-carrier genotypes were strongly correlated (odds ratio: 2.7; 95% CI: 1.5-4.7) with higher risk. Comparison of VEGF +936 C/T polymorphism between patient and control groups revealed no association with renal carcinoma. Both VEGF -2578 C/A and VEGF +936 C/T polymorphisms showed no significant association with the histopathological parameters of RCC. CONCLUSIONS: This study shows that VEGF -2578 A-allele and A-carrier genotypes are associated with an increased risk of RCC. In groups with higher incidence of RCC, a screening test for this polymorphism may be recommended in conjunction with other established markers.