Unusual eosinophilic variant of chromophobe cell renal carcinoma--an ultrastructural diagnosis

A case of eosinophilic variant of chromophobe cell renal carcinoma (EVCCRC), an uncommon variety of renal cell carcinoma, occurred in a 72 year old male. The most problematic differential diagnosis was renal oncocytoma, as the two entities share overlapping features on histology, yet differ completely in biological behavior. EVCCRC is a potentially malignant neoplasm whereas renal oncocytoma is totally benign. Staining with Hale's Colloidal Iron using modified Mowry's technique showed granular cytoplasmic positivity. The diagnosis was confirmed by ultrastructural examination of the tumor which revealed unique features of EVCCRC like presence of numerous cytoplasmic microvesicles along with mitochondria displaying tubulo-vesicular cristae. This case delineates the role of electron microscopic examination as the sole means to differentiate EVCCRC from renal oncocytomas.     

Membranous nephropathy with light chain restricted deposits

The literature on membranous nephropathy (MN) with monoclonal deposits on immunofluorescence (IF) and their outcome is very scarce. We report our experience of managing five patients with this clinical entity. The mean age of the patients was 33.2 ± 6.55 years. The mean proteinuria, serum albumin and serum creatinine was 5.73 ± 2.17 g/day, 2.86 ± 0.51 g/dL and 1.34 ± 1.19 mg/dL, respectively. None of the patients had a lymphoproliferative disorder. Only one patient had an elevated free light chain ratio. Four (80%) patients were M‐type phospholipase A2 receptor (PLA2R) negative (tissue and serum), and one (20%) was PLA2R related. Three (60%) cases had monoclonal IgG3/k, one IgG3/λ, whereas one patient with PLA2R positivity had an IgG3/IgG4k subtype. Two (67%) patients treated with cyclical cyclophosphamide and steroids (cCYC/GC) achieved complete remission and one patient (33%) with elevated baseline creatinine had a reduction in serum creatinine with persistent proteinuria at the end of the 12th month of follow‐up. One patient with PLA2R positive MN was treated with Rituximab and is in complete remission. The patient with an elevated free light chain at baseline was treated with Bortezomib/Thalidomide/Dexamethasone, had complete remission at 12 months, however, had a progressive rise in creatinine over the next 40 months of follow‐up. The current series, though limited by numbers, documents the efficacy of conventional therapies in non‐malignant associated MN with monoclonal deposits on IF.     

Leiomyomatosis of the Esophagus: Experience over a Decade

Purpose  To assess the clinical, radiological findings, and treatment strategies in patients with esophageal leiomyomatosis. Background  Esophageal leiomyomatosis is a rare hamartomatous disorder with varied presentation. It is described mostly in children and is associated with Alport’s syndrome. Methods  A retrospective analysis of three cases managed in the Department of General Surgery at Chandigarh over a period of 10 years. Results  The study involves three female patients of different generations within the same family with age range of 10–58 years. All presented with dysphagia of 2–7 years duration. Barium swallow revealed a long-segment stricture in two patients. Computed tomography (CT) demonstrated a circumferential mass lesion in the lower esophagus in all the patients. Esophageal resection was carried out in all the patients. All patients made an uneventful recovery. Conclusions  Esophageal leiomyomatosis should be suspected in patients with long-standing dysphagia. Barium findings are suggestive but can mimic achalasia. CT scan shows a circumferential esophageal wall thickening. Surgical resection and reconstruction of the digestive passage is the optimal treatment.     

Experience with similar biologic rituximab in 77 patients of granulomatosis with polyangiitis—a real-life experience

Clinical Rheumatology - To present single centre experience on the efficacy and safety of similar biologic of rituximab in patients with granulomatosis with polyangiitis (GPA). This was a...     

Membranoproliferative glomerulonephritis in a carcinoma with unknown primary: an autopsy study

Kidney disease frequently complicates malignancy and its treatment. Although many solid and hematologic cancers may involve the renal parenchyma, clinical sequelae are usually not prominent. Published reports cite membranous nephropathy as the most common malignancy-associated glomerulopathy, occurring with many carcinomas and occasionally with leukemia and lymphoma followed by minimal change disease. Rarely membranoproliferative glomerulonephritis (MPGN) has been reported in patients with malignancy. The mechanism by which malignancy induces disease remains unproved, but may involve deposition of tumor antigen in the subepithelial space with in situ immune complex formation and subsequent complement activation. Treatment of the underlying malignancy may lead to resolution of nephrotic syndrome, lending indirect support to this theory. We report a rare autopsy case of a patient with metastatic carcinoma (with unknown primary) associated with MPGN. The association between MPGN and metastatic carcinoma with unknown primary is uncommon and has not been previously reported in the literature.