Prognostic significance of Klotho expression in patients with renal cell carcinoma.

INTRODUCTION: Various molecular markers have been investigated in renal cell carcinoma (RCC) without significant reliability. We analyzed Klotho (tumor suppressive protein) expression in RCC to investigate its association with tumor-stage, grade, disease-free-survival (DFS) and overall-survival (OS). METHODS: Data of histologically confirmed patients of RCC with complete clinical follow-up were retrieved from Medical-Record-Library. Tissue sections of tumor and normal parenchyma were prepared from the blocks. Immunohistochemical studies for Klotho were done with commercially available kit (EPR6856, Ab181373; Abcam, Cambridge MA, USA). Klotho expression was scored between 0-3 and grouped into weak/absent (0, 1) and moderate/strong (2, 3). Tumors stages and grades were grouped into low stage (I and II) and high stage (III and IV) and into low grade (grade 1 and 2) and high grade (grade 3 and 4) according to WHO/ISUP grading. The histopathologists were blinded as to the clinical and follow-up data. Various prognostic factors were analyzed with respect to Klotho expression. Kaplan-Meier curves were created for DFS and OS. RESULTS: Fifty-four patients of mean age 55.15 ± 13.34 years and M:F ratio of 1.8:1 were included. Normal renal tissue had strong expression of Klotho in all. In tumor tissue 20 (37%) had negative, 7 (13%) had weak, 14 (25.9%) had moderate and 13 (24.1%) had strong Klotho expression. Significantly more patients had absent/weak Klotho expression with higher grade (16/24 (66.7%) vs 7/25 (28%); p = 0.007), higher stage (22/33 (66%) vs 5/21 (23.8%); p = 0.002), LVI (12/14 (85.7%) vs 2/14 (14.3%); p = 0.002), sinus-fat-invasion (16/21 (76.2%) vs 5/21 (23.8%); p = 0.002), renal-vein-involvement (14/18 (77.8%) vs 4/18 (22.2%); p = 0.004), necrosis (17/26 (65.3%) vs 9/26 (34.6%); p = 0.029) and metastasis (8/9 (88.9%) vs 1/9 (11.1%); p = 0.01). Median DFS and OS were significantly lower in patients with weak/absent Klotho expression (12 vs 23 months, p = 0.023 and 15 vs 33 months, p = 0.006 respectively). Kaplan-Meier curves showed lower estimated DFS and OS in patients with weak/absent expression. CONCLUSIONS: We conclude that Klotho expression in renal tumor could be a good prognostic marker in patients with RCC.

Toxic epidermal necrolysis-like presentation of toxic erythema of chemotherapy.

Toxic erythema of chemotherapy is an umbrella term encompassing a range of reactions characterized by symmetric erythematous to dusky patches, which can develop oedema, desquamation and/or purpura. We describe an elderly patient with prostate cancer who developed this complication while receiving docetaxel chemotherapy, presenting with prominent mucosal and periorificial involvement, along with epidermal necrosis, closely mimicking toxic epidermal necrolysis.

Large spontaneous steinstrasse: Our experience and management issues in tertiary care centre.

BACKGROUND: Steinstrasse, is described as array of stone pieces in the ureter following extracorporeal shock wave lithotripsy (SWL). It is well-recognized, transient event. Steinstrasse clears spontaneously, however about 6% require intervention. Spontaneous steinstrasse without prior history of SWL is a rare occurrence and only few case reports are published in literature. Objective of the study was to assess the aetiology and management issues of large spontaneous steinstrasse in our centre. METHOD: From February 2017 to March 2019, 684 patients underwent SWL for renal or ureteric stones. Twenty-eight patients presented with steinstrasse of which nine patients had no prior history of SWL. Detailed clinical profile and management issues have been discussed. RESULT: Among the nine patients of spontaneous steinstrasse, there were six males and three females. Mean age of the patients was 39 years (±13 years SD). Bilateral large spontaneous steinstrasse was seen in one out of nine patients. Seven patients had associated renal stones. Five patients presented with obstructive uropathy and three out of them had urosepsis as initial presentation. Ureterolithotomy and percutaneous nephrolithotomy were commonly performed procedures for stone clearance. Metabolic work was done in all cases in follow up period. Three patients had hypercalciuria and hypocitraturia suggestive of renal tubular acidosis. CONCLUSION: Large spontaneous steinstrasse is uncommon case scenario. It can be subtle in presentation and yet have significant patient consequence in terms of renal function and infection. Prompt management is essential to preclude permanent loss of renal function. Metabolic evaluation is indispensable in such cases.

Increased gene expression of B cell-activating factor of tumor necrosis factor family, in remitting antineutrophil cytoplasmic antibody-associated vasculitis patients.

AIM: To study the expression of B cell-activating factor of tumor necrosis factor family (BAFF) and A proliferation-inducing ligand (APRIL) genes in active and remitting patients with anti-neutrophil cytoplasmic antibody-associated vasculitis (AAV) and healthy controls and their correlation with disease activity. METHODS: This was a prospective case-control study. Gene expressions of BAFF and APRIL were studied in 32 patients with AAV (16 each with active disease and in remission) and 30 healthy age and sex matched controls by real-time polymerase chain reaction. RESULTS: Out of 32 AAV patients, 26 had granulomatosis with polyangiitis (GPA) and 6 had microscopic polyangiitis (MPA). Mean ages of patients in active (12 GPA and 4 MPA) and remission (14 GPA and 2 MPA) groups were 39.4 ± 17.2 and 44.6 ± 16.1 years, respectively. BAFF gene expression was significantly higher in both the active AAV group and remission AAV group compared to controls (P < .01). The BAFF expression was significantly higher in AAV patients in remission compared to active AAV patients (P = .003). In contrast, APRIL expression did not differ between AAV patients and controls (P = .829). However, APRIL had significantly higher expression in remission as compared to active patients (P = .048). There was no significant correlation of both BAFF and APRIL expression with disease activity markers (erythrocyte sedimentation rate, C-reactive protein, platelets and Birmingham Vasculitis Activity Score version 3). CONCLUSION: BAFF gene is significantly expressed in patients with AAV. Among AAV patients, there is a significantly higher expression of BAFF and APRIL in remitting state of the disease as compared to active state. There is no significant change in APRIL gene expression in patients with AAV as compared to controls. This makes a case for anti-BAFF therapy in future for AAV patients in northern India.

Laparoscopic Nephroureterectomy for Nonfunctioning Ectopic Pelvic Kidney with Ectopic Ureter and Lower Ureteral Stricture Opening in the Vestibule of the Vagina But No Incontinence: Challenges in Diagnosis and Surgical Dissection.

Failure of mature kidney to reach its natural location in renal fossa is termed as renal ectopia. Ectopic kidney can be found in pelvic, iliac, abdominal, and thoracic location. Pelvic ectopia has been estimated to occur in 1 of 2100 to 3000 autopsies. In contrast, ectopic ureters are commonly associated with complete renal duplication. Commonest presentation in females in continuous urinary incontinence with normal voiding habits as ectopic ureter open below the bladder neck in urethra or vagina. An ectopic kidney with ectopic ureter is extremely rare congenital anomaly. We report a 36-year-old woman presenting with left lower abdomen pain with no history of fever, dysuria, or urinary incontinence. On evaluation, she was found to have left nonfunctioning ectopic pelvic kidney with ectopic ureter opening in the vestibule of the vagina, which was managed with laparoscopic nephroureterectomy. One should suspect an ectopic ureter in a female presenting with continuous urinary incontinence since birth. However, diagnosis is challenging when clinical presentation is unusual with no urinary incontinence as seen in the index case. Detailed local examination in correlation with imaging is key for diagnosis and rule out other congenital anomalies. Laparoscopic approach in such clinical scenario is a safe and feasible option.

Ewing's Sarcoma of the Adrenal Gland.

Ewing's sarcoma (ES) or primitive neuro-ectodermal tumor (PNET) typically occurs in long or flat bones, the chest wall, extra-skeletal soft tissue, and rarely in solid organs. Incidence of adrenal Ewing's sarcoma is very rare. Here we report a case of Ewing's sarcoma of the right adrenal gland in an 8-year-old girl who presented with an abdominal mass. The huge tumor was managed by preoperative neo-adjuvant chemotherapy followed by surgical resection. She died due to metastasis after five months of surgery.