Primary Renal Synovial Sarcoma Presenting as Haemorrhagic Shock: A Rare Presentation.

Primary synovial sarcoma (PSS) of the kidney is considered the rarest type of all renal sarcomas with specific chromosomal translocation t (X; 18) (p11.2; q11.2). We report the case of a 65-year-old man with no medical conditions who presented to the emergency department with sudden severe right flank pain associated with haemodynamic instability and haemorrhagic shock. Computed tomography (CT) of the abdomen and pelvis revealed a right renal mass. A right open radical nephrectomy was performed. Histopathology revealed a monophasic synovial sarcoma. The patient received six cycles of docetaxel and gemcitabine as adjuvant chemotherapy. No sign of recurrence was seen on a follow-up CT urogram. This rare tumour often presents atypically, and clear guidelines regarding appropriate treatment are lacking. Our case showed that treatment with docetaxel/gemcitabine after an open radical nephrectomy is promising.

Multiple Primary Malignancies of the Colon, Stomach, and Kidney in a Patient with Bowel Obstruction Requiring Emergency Surgery: A Case Report.

BACKGROUND Multiple primary malignancy (MPM) is defined as 2 or more primary malignancies diagnosed in the same patient. Even though MPMs are rare, various associated tumors have been reported in the literature. We report the first case of triple synchronous primary malignancies: gastrointestinal stromal tumor, colon adenocarcinoma, and renal cell carcinoma. CASE REPORT A 63-year-old man presented to our emergency department with a 7-day history of diffuse abdominal pain and constipation. Examination revealed a distended abdomen and diffuse tenderness. Enhanced computed tomography showed a high-grade large bowel obstruction with the transitional zone seen at the splenic flexure, which was suspicious for primary colon cancer, and a hypodense lesion on the left mid-pole of the kidney. An emergency exploratory laparotomy revealed a splenic flexure mass, which was resected, and a left renal mass, which was excised. A stomach mass at the greater curvature was an incidental intraoperative finding; a wedge resection was performed for it. The pathology for each of the masses showed a primary malignancy. CONCLUSIONS Multiple primary cancers are rare and a multidisciplinary team approach is essential for management of these patients, be it preoperative, intraoperative, postoperative, or long-term surveillance.

Granulomatous Interstitial Nephritis Due to Renal-limited Sarcoidosis.

Sarcoidosis can present as acute kidney injury (AKI) due to granulomatous interstitial nephritis (GIN). AKI caused by sarcoid GIN without extra-renal manifestations is extremely rare. We report a case of a 42-year-old man with a history of unexplained weight loss admitted with progressively worsening kidney function. Physical examination did not show any abnormality. Laboratory investigations were normal except for high calcium level with no evidence of organ involvement of sarcoidosis. A renal biopsy showed GIN with non-caseating granulomata. Prednisolone was initiated and renal function improved. This is a case of an extremely rare AKI caused by sarcoid GIN without extra-renal manifestations which responded to prednisolone.

Epidemiology profile of renal cell carcinoma: A 10-year patients' experience at King Abdulaziz Medical City, National Guard Health Affairs, Saudi Arabia.

PURPOSE: The purpose of this study is to describe the epidemiological profile, histopathological features, and outcomes of patients diagnosed with renal cell carcinoma (RCC) in a tertiary referral center over 10 years. METHODOLOGY: This is a retrospective cohort of 219 Saudi patients diagnosed with RCC between June 2003 and May 2013. The variables collected included the sociodemographic details and clinical presentation. The histopathological features investigated include the tumors histological subtype, pathologic staging tumor, node, and metastasis descriptors, and lymph-vascular invasion. Patients were followed until May 2013. Bivariable analysis was calculated using Chi-square test, with level of significance set at P < 0.05. Kaplan-Meier estimate was used to calculate the survival rate. RESULTS: The mean age of patients was 57.18 (±14.68 standard deviation). The trend of patients diagnosed with RCC over the past 10 years was higher among males than females (60.27% vs. 39.73%). Noticeably, more than half (57.58%) were diagnosed incidentally. The most common histological subtype was clear cell (conventional) RCC (70.44%). Patients were usually diagnosed at the pT1 stage (48.1%).The histopathological features associated with worse patient outcome were the stage of the primary tumor (P = 0.01) and lymph-vascular invasion (P = 0.003). The overall mean survival rate was 2.03 years. CONCLUSION: In the past 10 years, there are more patients diagnosed incidentally with RCC, which is in line with the global trend. Patients were more likely to be male and middle aged. We recommend further population-based studies in this area to establish a national epidemiological data for this common type of cancer.

Alport's syndrome with focal segmental glomerulosclerosis lesion - Pattern to recognize.

The association between Alport's syndrome (AS) and focal segmental glomerulosclerosis (FSGS) in the same patient is complex and rarely reported. We report a case of a 42-year-old male presenting with proteinuria, microscopic hematuria, elevated serum creatinine and hypertension with unremarkable physical examination apart from obesity. The renal biopsy showed well-established FSGS pattern of injury with mild interstitial fibrosis and tubular atrophy, while the electron microscopic examination demonstrated glomerular basement membranes (GBM) changes compatible with AS. AS can be complicated by segmental glomerular scarring, which can mimic primary FSGS, while familial FSGS can result from mutations in collagen IV network of the GBM. This overlap can complicate histopathological interpretation of renal biopsy, which should be accompanied by mutational analysis for accurate diagnosis and proper therapeutic intervention.

Atypical antiglomerular basement membranes disease with nephrotic-range proteinuria, mesangial proliferation, and membranoproliferative glomerulonephritis pattern of injury.

Antiglomerular basement membrane (anti-GBM) disease is an uncommon autoimmune disease characterized by the presence of IgG autoantibodies targeting the alpha-3 chain of type IV collagen. Some of the atypical forms of the disease have been described. Herein, we describe a case of atypical anti-GBM in a 27-year-old Saudi male who presented with lower limb edema, gross hematuria, elevated serum creatinine concentration, and nephrotic-range proteinuria. All serology tests were negative, except for anti-GBM which was weakly positive. Renal biopsy showed proliferative glomerulonephritis (GN) with nodular transformation of the glomerular tufts, mesangial hypercellularity (mesangial cell proliferation), segmental endocapillary hypercellularity and three incomplete cellular crescents, and recapitulating membranoproliferative GN pattern of glomerular injury. Direct immunofluorescence microscopy demonstrated diffuse, intense linear positivity for IgG and Kappa and Lambda light chains, and compatible with anti-GBM disease. The patient was treated with cyclophosphamide and corticosteroids in addition to therapeutic plasma exchange which resulted in mild improvement in renal function over a period of six weeks. We emphasize the importance of recognition of atypical pathological and serological patterns of anti-GBM disease, which is crucial for proper and early diagnosis and possibly improved clinical outcome and we highlight the importance of clinicopathological correlation in cases with atypical clinical and pathological presentations.

Rare genetic variant in the CFB gene presenting as atypical hemolytic uremic syndrome and immune complex diffuse membranoproliferative glomerulonephritis, with crescents, successfully treated with eculizumab.

BACKGROUND: Complement factor B gene (CFB) is an important component of the alternate pathway of complement activation that provides an active subunit that associates with C3b to form the C3 convertase, which is an essential element in complement activation. Among the complement-associated disorders, mutations and pathogenic variants in the CFB gene are relatively rare phenomena. Moreover, mutated CFB affiliation with immune-complex diffuse membranoproliferative glomerulonephritis (IC-MPGN) and atypical hemolytic uremic syndrome (aHUS) are considered a highly rare occurrence. CASE PRESENTATION: We describe the clinical presentation, course, and pathological findings in a 7-year-old boy who has confirmed CFB heterozygous variants with pathological features compatible with IC-MPGN. Mutational analysis revealed a heterozygous variant p.Glu566Arg in exon 13 of the CFB gene. The patient did not respond to steroids and mycophenolate mofetil (MMF) therapy but responded clinically and biochemically to eculizumab treatment. This is the first case report of CFB alteration associated with IC-MPGN and aHUS that was successfully treated with eculizumab. CONCLUSIONS: Heterozygous variants in the CFB gene can be pathogenic and associated with IC-MPGN and aHUS. Early diagnosis and prompt management can be essential in preventing end-stage renal disease. Eculizumab may provide an effective modality of treatment.

Herb-induced acute bone marrow intoxication and interstitial nephritis superimposing glomerular C1q deposition in a patient with paroxysmal nocturnal hemoglobinuria.

Paroxysmal nocturnal hemoglobinuria is a rare disease of the red blood cell membrane that renders it lyzable by the complement system, leading to chronic intravascular hemolysis. Renal hemosiderosis is a well-known complication of intravascular hemolytic anemia and can lead to acute kidney injury and renal failure. The use of herbal medicine is common worldwide. The nephrotoxicity of herbal remedies can take several forms, which include acute kidney injury and acute and chronic interstitial nephritis. In addition, the use of herbal remedies can result in bone marrow toxicity and suppression. C1q nephropathy is an uncommon form of glomerular disease characterized by dominant or co-dominant glomerular immunofluorescence positivity for C1q in the absence of clinical and serological evidence of systemic lupus erythematosus, and has various clinical presentations and outcome. Here, we report a patient of undiagnosed paroxysmal nocturnal hemoglobinuria who consumed herbal medicine of unknown constituents and clinically presented with anemia and acute kidney injury. The pathological findings of bone marrow and renal biopsies that include bone marrow intoxication, severe renal hemosiderosis and acute interstitial nephritis and kidney injury, as well as co-dominant glomerular deposition of C1q, are discussed. In addition, we discuss and hypothesize the possible pathogenesis of glomerular C1q deposition in the setting of paroxysmal nocturnal hemoglobulinuria.

Acute diffuse proliferative post-infectious glomerulonephritis in renal allograft--a case report and literature review.

PVN is a well-known cause of renal allograft dysfunction and failure. The diagnosis is established by examination of tissue from the renal graft, and confirmed by immunohistochemical or in situ hybridization techniques. Electron microscopy can be utilized as an ancillary modality to identify the viral particles ultrastructurally. The tubular epithelial cells are the primary target of PV cytopathic effect; however, PV-associated glomerular changes have also been described. Immune-type electron-dense deposits in the TBMs have been described in the setting of PVN, and rarely, likewise have glomerular subepithelial hump-like deposits. Diffuse immune-mediated proliferative glomerulonephritis in the setting of PVN has not been reported before. In this report, we describe an 11-yr-old kidney transplant recipient boy who developed immune-mediated glomerulonephritis with light microscopic, immunofluorescence, and ultrastructural features compatible with acute PIGN superimposing chronic PVN, discuss this unusual association and the possible mechanisms of antigen clearance in PVN and present a literature review.